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1 AARTS clinics, as it takes a short time and is easy to complete.

Forced use applied to the affected upper limb


of children with cerebral palsy
P B M AARTS, J VAN DER BURG, R A B OOSTENDORP
Sint Mmrtenskliniek, Nljmegen, the Netherlands 3 AHMAD
Violence against females in Pakistan
Objective: Following on from a new theory of motor skill R G AHMAD, MUHAMMAD
development, called the dynamic system theory (DST), we AMAL (youth Empowerment Skills), Pakistan
attempted to formulate an occupational therapy framework.
A specific treatment method, the Forced Use approach, was Introduction: Women form half of the world’s population
applied to children with cerebral palsy (CP). Asymmetry of yet they are still subject to inequality and injustice on a global
the upper extremities was evaluated. scale, far more than their male counterparts.
Methods: In this prospective randomized clinical trial, the This state of affairs is mirrored in Pakistan which has a total
research group consisted of 11 children with CP aged 4 to 10 population of 142 million, of which 48.15% are female.
years. The primary outcome measure was the Melbourne Pakistan is among the very few countries in the world where
Assessment of Unilateral Upper Limb Function, and the sec- life expectancy of women (62 years) is less then that of men
ondary outcome measure was the Pediatric Evaluation of (65 years). The reasons cited for this situation are: illiteracy
Disability Inventory (PEDI) using the domain self-care from amongwomen (71%);a high rate of incidence of domestic vio-
the functional skills scale; the frequency and duration of lence (reported in 80%of households); poor nutrition; abject
spontaneous use of the upper extremity during play and poverty; absence of proper health care facilities; child mar-
ADL-activities with video observations, were recorded, and riages; and excessivechild bearing (average of6 to 8 children).
ratings were made by parents of the experimental group in Four women are murdered each day throughout
the forced use intervention. Pakistan. Many women are victims of ‘honour killings’, phys-
Results:The Melbourne assessment and PEDI showed no sta- ical violence, and thousands more suffer from psychological
tistically significant difference. The video observations result- torture, much ofwhich is hidden from public view.
ed in a statisticallysignificant difference for the activity ‘taking Objectives: The study is aimed at identifying the causes of
shoes off’.The ratings given by parents of two of the six chil- domestic violence, its manifestations, consequences, and
dren of the experimental group were above 7 (scale l to 10). intervention effects, for the evaluation of policy and to docu-
Conclusions: Based on the primary outcome measurement, ment the current situation.
the Melbourne assessment, that represents the functional Methods: The study was undertaken in Quetta, Pakistan,
effect of the forced use intervention, results showed no which hosts thousands ofAfghan refugees. Data were collect-
improvement o n standard treatment. Forced use was shown ed from monthly and yearly reports, a baseline survey in
to enhance spontaneous use of the more affected arm in the refugee settlements, focus group discussions, in-depth inter-
activity ‘taking shoes off’ to a greater extent than the stan- views with key informants, and participant observation.
dard treatment. Findings: Key factors in violence against women are disabili-
poverty, early marriage, forced marriage, infidelity and
polygamy Violence is expressed through physical abuse and
2 ACEK psychological torture. The latter includes verbal insults and
Hand function and activities in cerebral palsy neglect of family obligations. The most common conse-
E,
S BILGUTAY, G BUMIN, M UYANK, H KAYHAN quence of these is family separation, which has resulted in an
Hacettepe University, l%e School of Physical Therapy and increased number of single parents and female-headed house-
Rehabilitation, Ankara, Turkey holds, and suicide. Power imbalances compound the prob-
lem as a large percentage of women rely on their spouses for
Objective:To investigate the role of hand function testing in social and economic support. Social perceptions of single
evaluating activity performance inside and outside the home women are also biased, making it difficult forwomen to leave
in children with cerebral palsy (CP). abusive spouses, thereby perpetuating the violence.
Methods: Eighty two children (37 female and 45 male) with Conclusion: Despite ongoing intervention, violence against
CP whose mean age was 83.3239.6 months participated in females continues, particularly against females with disabili-
the study. TheJebsen Hand Function test was used for assess- ties, and females, in the study area. Emphasis should be on
ing hand function; the Physical Ability Test was used to evalu- addressing the causes through continual community partic-
ate gross and fine grip, and hand dexterity according to their ipation in psychosocial help workshops, empowerment of
development. PEDI and WeeFIM assessments were also com- females, and counselling at individual family and group lev-
pleted by observing the children and asking their families. els. Alcohol and drug abuse needs to be addressed socially
Results: There was acorrelation between theJebsen Hand Function and legally in the area, as this is also a contributing factor.
Test and the PEDI (p<0.005),WeeFIM (p<0.005), and the Physical
Ability Test (p<0.005). The correlation between the PEDI and
WeeFlM was significant (p<0.005). Also, the WeeFIM and the
PhysicalAbility Test showed a strong correlation (p<0.005).
Conclusion:The Jebsen Hand Function Test can be used to
assess daily activities of children with CP The PEDI pro-
vides more detailed information about the abilities of chil-
dren, but the WeeFIM can be used as an evaluation tool in

3
4 ALMOND The child had received weekly physiotherapy including
Design and validation of a nutrition screening stretching for knee flexion contracture on both lower limbs,
tool for children with physical disabilities and but showed no improvement.
learning difficulties Outcomes: The knee flexion contracture, which was not cor-
SARAH ALMOND rected with passive stretching, improved with casting from
ChaileyHeritage Clinical Services, East Sussex, UK -40" knee extension to - 5 " . Knee extension degree was mea-
sured by standard Goniometer over a period of 4 weeks.
Aim: To design and validate a nutrition screening tool that Careful measurements were taken to ensure skin integrity.
will accurately identrfy children aged 3 to 19 years with neu- After one year the child continues to ambulate independent-
rodisabilitiesfor referral to dietetic services . ly with the help of walking aids.
Methods: A nutrition screening tool has been designed Discussion: The outcomes indicate that serial casting fol-
which contains the clinical characteristics associated with lowed up with bracing may be useful for the enhancement of
malnutrition in children with neurodisability. walking ability for a young child with spina bifida and knee
School nurses complete the tool and separately assign flexion contractures. Further investigation of serial casting as
children into one of three categories: (1) do not need to be well as investigation of using serial casting with other inter-
referred to the dietitian; (2) should be monitored; and (3) ventions is warranted.
need to be referred to the dietitian. For interrater reliability
three school nurses must complete the tool for each child so
when the tool is used by several raters variability can be
assumed to come from the child rather than nurse bias. 6 ARVIO
On obtaining 100 results, an item analysis will be carried out Topiramate treatment: follow-up of cognitive
discarding the questions which do not correlate highly with skills in patients with learning disability and
true nutritional status; a scoring system will be applied. True epilepsy
nutritional status is ascertained by full dietetic assessment. OIL1 SAUNA-AHO, MARIA ARVIO
Validity is measured to assess how well the tool identifies Key Foundation Lahti, Pdhjarvi Inter-Municipal
at-risk cases and whether the tool is able to discriminate Association, Lammi, Finland
between good and poor nutritional status.
Receiver operating Characteristic (ROC) analysis will pro- Objective: To ascertain cognitive adverse effects of topira-
vide the number of correct and incorrect classifications for mate (TPM) in patients with epilepsy and learning disability
every cut-offscore. A score will be chosen which has best bal-
ance between: sensitivity and specificity. This will take into Method: A clinical neuropsychologist (0s) assessed the
account the relative costs of making a mistake, as it would be reasoning, attention, memory, visual perception, visuomo-
more clinically sound to be too sensitive than too specific, tor and verbal abilities, speech production, and naming of
i.e. to have the occasional child referred who is not at nutri- 12 patients with LD and epilepsy before and, at 3 , 6 , and 9
tional risk. months, after TPM administration. Tests were selected
Logistic regression may be used to ascertain the weight according to the developmental stage of the patient (e.g.
each question should bear to provide optimum results. WISC-111, WPPSI-R, Raven, LIPS, Boston Naming Test, and
Results:At 2 years the project is still in its early stages. Nepsy) .The parents and/or caregivers evaluated the behav-
Multicentre consent has been received for all entering the iour of patients with the Nisonger Child Behaviour Rating
reliability study and item analysis stages, and data collection Form according to the same schedule. Aetiology of LD was
has commenced. heterogeneous and the degree of the LD varied from pro-
found to mild.
Results: In 1 patient the medication was stopped because of
rage outbursts, thus the number of follow-up patients was
5 AL- ORAIBI 11. The naming of 2 patients with mild LD became slower.
Serial casting to correct knee flexion Performance of 10 patients remained stable; 1 patient with
contracture in children with spina biflda autism showed poorer contact. Four patients displayed an
SALEH AL- ORAlBl improved mood, 2 better behaviour, the speech of 1 patient
AL-Hashmite University,Jordan became clearer, and 1 showed better memory.
Conclusion: The cognitive adverse effects of TPM in
Bac&groundand aims: Severe knee contracture that devel- patients with learning disabilities and epilepsy seem to be
ops early following muscle imbalance may not improve with slight and the performance of the patients appears to
stretching exercise and splinting. An alternative treatment is remain stable.
serial casting which has been used to promote increased
range of motion. It has also been used to facilitate patient
compliance with positioning and to prevent complications
or adverse effects associated with surgical intervention. This
case report describes, for the first time, the use of serial cast-
ing to correct knee flexion contracture for a young child with
spina bifida.
Case description: The child was 6 years old with L3-L4 spina
bifida level lesion and quadriceps muscle strength grade 3 . +

4 EACDAbstractsZOO5
7 ARVIO Conclusions: Patients with some DS manifest specific psy-
Head may shrink - evaluation in patients with chological profiles of cognitive and behavioural characteris-
aspartylglucosaminuria tics according to genotype and the age. They may be
P ARVIQ~, K H U R M E R I N T A ~ s , A R V I O ~ ,M SILLANPAP successfully used both in support of diagnostic process and
, PIRINEN~M
S a m m i - l i d o s Health Care Centre; bHelsinkiUniversity as a basis for adequate medical and psychological interven-
Hospital; CHelsinkiUniversity;dP&jarvi Centre; ‘7iuku tion and counselling of parents.
University,Finland
Objective: Aspartylglucosaminuria (AGLJ) is a recessively
inherited lysosomal storage disorder caused by deficiency of
aspartylglucosaminidase(AGA), its main symptom being pro-
gressive mental retardation. Patients have a consistent dys- 9 AUBERT
morphic gestalt including hypertelorism, a short and broad Siblings of a child with brain injury:
nose, simple ears, thick lips, and a square face. The aim of this a clinical study
study was to evaluate cranial anatomy of patients with AGU. SOPHIE AUBERT~, RECINE S C E L L E S ~ADELINE
, ZAEPFELL, SYLVIE
Methods: Head circumference of 78 patients (aged 3 to 68) VIGNAULT‘, ANNE LAURENT-VANNIER~
was measured and compared to Finnish standards. The aServicede reeducation des pathologies neurologiques
heads of 40 middle-aged patients were re-measured 10 years acquises de l’enfant,Hbpital National de Saint Maurice;
after onset. Lateral cephalometric radiographs of 47 patients bUniversiteFranGoisRabelais, Tours, France
and 47 age- and sex-matched controls were evaluated using
15 landmarks. Introduction: The aim of this psychological study was to
Results: Of 29 patients under 15 years of age, 9 (31%) had observe the personal experiences and investigate the feel-
macrocephaly (head circumference >2SDs); of 7 patients ings expressed by brothers and/or sisters of brain-injured
over 45 years of age, 4 had microcephaly. Longitudinal mea- children about 6 months after the injury. The findings of this
surements of 40 adult patients showed reduction in head work could be used to minimize the mental suffering of sib-
size in 25 (63%) from 1 to 4.5cm (mean 1.7).Radiographs lings of children with brain injuries. Development of a pic-
showed that young patients had longer skulls than the con- ture book was proposed to promote family communication
trol and middle-aged patients. Compared with controls, the about brain injury and to try to minimize the psychological
patients’ crania, both at glabella and at opisthocranium, consequences.
were thick (p~0.001).The cranial base appeared normal. Methods: The study was performed in a rehabilitation
Sphenoidal as well as frontal sinuses appeared flat and short department for children with acquired brain injuries. Six
(p<O.OOl). Frontal sinuses were absent in 30% and sphe- children from 4 families were investigated several months
noidal sinuses absent in 6%of patients. after the brain injury. Tivo complementary types of assess-
Conclusions: Children with AGU show macrocephaly. ment were used: a semi-directed interview and the projec-
Cerebral atrophy and reduction in brain volume after puber- tive drawing of the family.
ty is evident in patients with AGU and is reflected in a Resultsldiscussion: Elements of a psycho-traumatic syn-
decreased head size. drome were observed in most children. The children used
psychic resources to cope with the real and subjective conse-
quences of the brain injury. Sibling relationships of each
8 ASSENOVA SHOPOVA member of the family changed over the time. The brain
Cognitive and behavioural phenotypes of injury event was considered a step in the family’s life with
children with dysmorphic syndromes and their before and after periods. Recurrent topics were covered in
transformation throughout adulthood the picture book which allows children and adults to talk
S ASSENOVA SHOPOVA, E SIMEONOV, 1 KALEV about the brain injury and its consequences. It provides an
UniversityHospital, Alexandrovska ’Pediatric Clinic, Sofia opportunity for children to ask questions, to share their sub-
jective experiences and emotions with family members, and
Testing cognitive capacity is a modern diagnostic approach for parents to better understand their children’s concerns.
used for patients with genetic diseases which present diag- Conclusion: The picture book allows discussion about the
nostic problems due to their variety. That renders the precise brain injury and its main aspects. It might be useful in pre-
estimation of the cognitive and behavioural phenotype venting or minimizing the psychological consequences of
important. brain injury in the family and, most particularly, in siblings.
Aim: To study cognitive and behavioural characteristics of
patients with dysmorphic syndromes (DS): Down, FRAX-A,
Prader-Willi, Williams-Beuren, Noonan, and Cornelia de
Lange syndromes, and to define the specific cognitive and
behavioural phenotypes.
Results and discussion: These syndromes manifest cogni-
tive capacity deviations of differing severity. IQ level as in
an individual patient changes with age. Patients with DS
show the most maladaptive behaviour. Autism and atten-
tion-deficit-hyperactivity disorder are frequently observed
in DS. Intellectual functioning and adaptive skills have an
inverse relationship.

5
10 BAKKER ninemia per year, and psychomotor development between 3
Functional consequences of progressive and 6 years of age using the Ruth Griffiths Mental Develop
scoliosis and operative intervention of mental Scale or the Denver I1 test.
children and adults with cerebral palsy: Result: 'henty one children had normal developmental
a literature review assessments and onlytwo had GQ scores below 80 in the Ruth
W F BAKKER, J W GORTER, J E PRUIjS, M KETELAAR, E LINDEMAN Griffiths scale with an homogeneous profile. Median age of
Rehabilitation Centel;De Hoogstraat, Utrecht;Partner of beginning of diet was 20 days except for the two children
NetChild Networkfor ChildhoodDisability Research in the with abnormal development who started the diet later than
Netherlands, the Netherlands the others (at 47 and 51 days).
Eleven children had average PHE levels between 6 and 10
Objective: The reported prevalence of scoliosis in children mg/dL in the first year of life.
and adults with cerebral palsy (CP) varies between 15%and Conclusion:Although almost all of the children in our sam-
72% related to the severity of CP in the described group. ple had average PHE levels between 6 and 10 mg/dL in the
Progression of the curve extends into adulthood, and there- first year of life; psychomotor development was normal in
fore requires long-term follow-up. When scheduled for an almost all. The two children with abnormal scores began the
operation the patient and family need to be informed about diet late. Early start of low PHE diet seems to be important for
the functional outcome based on scientificevidence. a good outcome.
The aim of the study was to learn more about the function-
al consequences and care load related to progressive scoliosis
in a patient with CI? We conducted a systematicreview on (1)
functional deterioration as a result of scoliosis and CP and (2) 12 BASKENT
functional consequences followingspinal fusion in CI? Effect of hippotherapy on children with
Methods: We searched PubMed and Cinahl databases on sco- spastic diparesis
liosis and CP (all languages published u p to January 2005, no G BASKENP, M OZMEN~
other limits). According to a predetermined audit proce- aMetin Sabanc, Spastic Children Centet;Istanbul; bDivision
dure, all articles were independently selected and reviewed of Child Neurology, Department of Pediatrics, Istanbul
by two team members (WB, JWG). Medical Faculty,Istanbul University, Turkey
Results: In total, 21 1references were found in the electronic
databases, ofwhich 137 were about CP and scoliosis. Objective: Neurodevelopmental treatment (NDT),known as
Only four articles were appropriate for describing the Bobath therapy, is the most common intervention pro-
course of functional abilities and care load without surgical gramme used in the treatment of children with cerebral palsy
intervention. More information was available from 16 studies (CP). Hippotherapy intervention is a therapy model applied
about the functional consequences of spinal fusion (Sackett's on the horse. The aim of this study is to evaluate the effect of
Level IV or V). The most frequent items investigated were hippotherapy on the muscle tone, gross motor function
gross motor activities (sitting, self-mobility) and caregiver sat- measure (GMFM), range of motion (ROM), and gait pattern
isfaction. A questionnaire designed by the Pediatric of children with spastic diparesis.
Orthopedic Society of North America was the only validated Methods: Fifty participants with spastic diparesis (20 hip-
instrument used. potherapygroup, 30 control group) between the age of 7-14
Conclusion: Limited evidence is available on the functional years were included in the study. Participants were selected
consequences of progressive scoliosis, and operative inter- who had the ability to communicate, to sit alone or with min-
vention of children and adults with CP Prospective con- imal support, no visual or hearing problems, and those with
trolled studies using validated instruments are needed to passive hip abduction of at least 20'. NDT was applied as a
outweigh the risks and benefits of spinal stabilization, and to home programme for each participant and a hippotherapy
inform the patients and their families. programme was undertaken twice a week in the study group.
ROM was evaluated with goniometric measurement, muscle
tone using thehhworth Scale, motor functions with GMFM,
and gait analysis was carried out before and after treatment.
11 BAPTISTA Results: Tests and measurements were repeated after six
Developmental assessment of children with months of treatment. For both groups there were statistically
phenylketonuria significant differences in ROM and muscle functions after
ARTUR SOUSA, BRIGIDA ROBALO, MANUELA BAPTISTA, FERNANDA treatment. In the hippotherapy group the increase in ROM
TORGAL, FILOMENA EUSEBIO, ANA GASPAR, ROSA GOUVElA and decrease in spasticity were higher than in the control
Child Development Department, Hospital Santa Maria, group. GMFM values were significantly improved in both
Universiv of Lisbon, Portugal groups. The increase in percentages in the standing and
walking-running parts of GMFM were higher in the study
Objective:The objective ofthis study was to evaluate psycho- group than in the control group. The results of the gait analy-
motor development between 3 and 6 years of age in children sis in the hippotherapy group revealed significant differ-
with phenylketonuria (PKU) at the Child Development ences in stance time, swing time, cadence, double support
Department of Hospital Santa Maria. time, step length, and mean velocity but not in step width
Method: Clinical records of 23 children with PKU were retro- @<0.05). In the control group there was not any significant
spectively reviewed for demographic data, age at beginning difference in any ofthe gait analysis parameters @>0.05).
of low-phenylalanine (PHE) diet, average levels of phenylala- Conclusions: According to o u r findings, we suggest that

6 EACDAbstracts 2005
hippotherapy might enhance the effects of physiotherapy natural histories of neuromuscular diseases, assist in select-
and increase the motivation of patients. More studies are ing patients for therapeutic trials, and help to quantify out-
needed to establish hippotherapy as an effective and wide- comes of therapeutic measures.
spread form of treatment. Objective:Validation of the Motor Function Measure: a c h i -
cal scale designed for neuromuscular diseases of children
and adults.
Material and method: The validation study took place in 19
13 BECKUNG units and included 303 patients aged 6 to 62 years. Seventy-
Gross and fine motor function and two patients had Duchenne muscular dystrophy (DMD), 32
accompanying impairments in cerebral palsy: Becker muscular dystrophy, 30 limb-girdle muscular dystro-
a population-based study of children of 4 to 8 phy, 39 facio-scapulo-humeraldystrophy, 29 myotonic dys-
years of age trophy, 21 congenital myopathy, 10 congenital muscular
K HIMMELMA", E BECKUNG , G HAGBERG, P UVEBRANT dystrophy, 35 spinal muscular atrophy, and 35 hereditary
TbeQueen Silvia Children's Hospitallsahlgrenska neuropathy. The scale comprised 32 items with ratings rang-
UniversityHospital, Goteborg,Sweden ing from O=no movement to 3=complete movement, test-
ing a variety of movements (in standing and sitting position).
Aims: To describe and analyze gross and fine motor function The sensitivityto change was tested in 152 patients.
and accompanying neuroimpairments in children with cere- Results:A factor analysis confirmed the structure of the scale
bral palsy (CP) born between 1991 and 1998 in western with three dimensions: standing position and transfers, axial
Sweden. and proximal motor function, and distal motor function.
Method: A population-based study comprising 367 children Agreement coefficientsfor intemter reliabilitywere excellent
with a diagnosis of CP verified at four to eight years of age. for 9 items, good for 20 items, and moderate for 3 items. High
Gross Motor Function Classification System (GMFCS) levels correlations were found between the total score and \rignos
were documented in all children, Bimanual Fine Motor (r=0.91) and Brooke (r=0.85) grades, Functional Indepen-
Function (BFMF) classification levels in 345, and learning dence Measure (r=0.91), the global severityof disabilityeval-
disability, epilepsy, visual and hearing impairments, and uated with visual analogue scales by physicians (r=O.88)and
hydrocephalus in 353. physiotherapists (r=0.91).Asubsample ofpatientswasevalu-
Results: GMFCS was distributed at level I in 32%, level I1 in ated one year later. Patients with DMD (n=41) showed the
29%,level I11 in 8%,level IV in 1596, and level V in 16%.The highest decrease: 5.5 6.1 (p<O.OOOl).The effect size in this
corresponding percentages for BFMF were 31%, 32%, 12%, groupwas 0.95.
12%,and 14%.Learning disability was present in 40%,epilep- Conclusion: The Motor Function Measure is reliable, does
sy in 33%,and severe visual impairment in 19%of the chil- not require any special equipment, and is well accepted by
dren. More severe GMFCS levels correlated with larger patients. Its sensitivityto change enables use in clinical trials
percentages of accompanying impairments. GMFCS level I of neuromuscular diseases, especiallyin patients with DMD.
correlated positivelywith increasing gestational age. Children
born before 28 weeks of gestation had the highest percentage Acknowledgements: This work was supported by the Associadon
of accompanying impairments. Children born at term with CP FranCaise contre les Myopathies.
of perilneonatal aetiology had more severe motor impairment
and more frequent accompanying impairments than those
with a prenatal or unclassifiable aetiology.A new classification
of spastic CP was applied. 15 BLANK
Conclusion: The classification of CP should be based on CP Reliability of a screening-questionnsre
type and motor function, as the two combine to produce an for assessment of activities of daily living in 3-
indicator of total impairment load. The aetiology of CP influ- to 7-year-old children with cerebral palsy
ences the severity of disability. The concept of uni- and bilat- R BLANK, S HENGVOSS S, H VON VOSS
eral spastic CP adds structure and comprehensibility to the Institute of Social Paediatrics and Adolescent Medicine,
CP classification. Ludwig-Mmimilians-University, Miinchen, Germany

Objective: The measurement of activities of daily living is


14 BERARD important for the management and evaluation of therapeu-
A motor function measure scale for tic interventions in children with disabilities.
neuromuscular diseases: validation study When developing the present questionnaire, the objec-
c BBRARD", M FOURNIERB MEHOUAS~, c PAYAND, J FERMANIAN tive was to create a simple, reliable, and valid screening
M D&
~ M F M COLLABORATIVE STUDY GROUP instrument that would be useful for everyday use, and which
aDepartmentof PaediatricRehabilitation CHU, Lyon-Sud; could be used for the quality management of treatment
bDepartmentof PhysicalMedicine,Hijpital de I'Arcbet, methods in children with disabilities.
Nice; Institute of Myology, HijpitalPitie-SaIp&i&re, Metbods: In two samples (n=48 and n=28) 3- to 7-year-old
Paris;dLaboratoirede Biostatistiques,Hdpital Necker- children with cerebral palsy, covering the full spectrum of
Enfants Malades, Paris,France severity (GMFCS I to V), were examined by a parental ques-
tionnaire (The Munich Screening for Activities of Dady Living
Backgmund: Precise tools are required to determine motor [M-ADL])on activities of daily living. The scales cover eating/
disabilities of patients. Such tools improve knowledge of the drinking, self-careat home, toileting, hand skills - summarized

7
in a score ‘self-careand independence’ - and mobility (scales 17 BLANK
on communication and interactional skills data not shown). Hand motor function and activities of daily
Each category was examined by a global rating (0-10) to test living in children with cerebral palsy
the subjective bias, and there was a rating based on defined R BLANK, H VON VOSS
scales. Interrater reliability was estimated by comparing par- Institute of Social Pediatrics and Adolescent Medicine,
ent and therapist ratings. The test-retest reliability was tested Child CentreMunich,Miinchen, Germany
within a 4-month interval.
Results: Interrater reliabilitywas very high (Spearman’s rho Objectfve:In the past, several kinetic and kinematic measures
mobility: 0.86 to 0.89, self-care: 0.84 to 0.85, subscales: have been examined in children with cerebral palsy (CP).This
0.73 to 0.86). The retest reliabilitywas excellent (Spearman’s studies summarizes a number of target parameters and tries to
rho mobility: 0.97 to 0.91, self-care:0.92 to 0.93, subscales: find out how these correlate with activities of daily living.
0.79 to 0.97). Method: Forty 3- to 7-year-old children with cerebral palsy
Dksnrssion: M-ADL is quick (for the examinerabout 5 minutes) were examined by kinetic and kinematic analysis of different
and easy to apply; it also provides reliable information. hand functions (grip object 200g with 3D-accelerometer,uni-
axial force sensor, pinch grip object 20g, digitizer tablet, ultra-
sound-based movement analysis). Target parameters were
maximum grip force, fastest isometric grip force, changes lift-
16 BLANK ing an object,grip force modulation while moving an object up
Validity of the Munich Screening for Activities and down, drawing lines and circles as fast as possible in two
of Daily Living in children with cerebral palsy sizes, finger tapping and hand tapping, and frequency aiming
p BLANK, S HENGVOSS S, H VON VOSS movements.The target variableswere summarized in two cate-
Institute of Social Paediatrics and Adolescent Medicine, gories: (1) elementary hand function - maximum grip force,
Ludwig-M~milianUniversity,Miinchen, Gemzany fastest grip force, changes finger and hand tapping frequen-
cies, and fastest drawing of simple lines, (2) coordinative hand
Objective: According to the International Classification of function - grip lift synergy, grip-load force, adaptation while
Functioning, measurement of activities and participation is moving an object, aiming (fluency), and drawing circles.These
crucial, and it becomes increasingly important for the man- two dimensions were also confirmed by factor analysis.
agement and evaluation of therapeutic interventions in chil- The Munich ADLquestionnaire was used for activities of daily
dren with disabilities. living.
In contrast to previous measures, the questionnaire quick Results: Almost all children revealed deficits in elementary
to administer and does not require specific training. hand function, but only two-thirds showed pathological
Methods: In two samples of 3- to 7-year-old children with coordinative hand function. However, elementary hand
cerebral palsy covering the full spectrum of severity (GMFCS function correlated at 0.5 (non-dominant hand) resp. 0.6
I toy n=33, n=63) the concurrent and discriminant validity (dominant hand) with activities of daily living,whereas coor-
of the The Munich Screening for Activities of Daily Living (M- dinative hand function correlated at 0.6, resp. 0.7. The veloc-
ADL) was examined. The discriminant validity was tested for ity-dependent parameters correlated well with the severity of
each age group by the ROC-procedure which compared the neurological impairment.
study group with a normal sample of 2 17 children. The scales Conclusion:Children with CP, even if with apparent normal
cover eating/drinking,self care at home, toileting, hand skills clinical function, seem to have consistently pathological ele-
- summarized in a score ‘self care and independence’ - and mentary hand function. Coordinative function was better
mobility (scales on communication and interactional skills correlated with activities of daily living.
data not shown). Each categorywas examined by a global rat-
ing (0-10) to test the subjective bias, and there was a rating
based on defined scales. For concurrent validity, the M-ADL
was compared with the Pediatric Evaluation of Disability 18 BLAUW-HOSPERS
Inventory (PEDI). Opening the black box of neurodevelopmental
Results: The concurrent validity was very good (Spearman’s treatment of infants with developmental
rho [global estimation vs scaled scoring]: mobility 0.80 vs motor disorders
0.92; self-care, 0.78 vs 0.82). Sensitivity and specificity was C H BLAUW-HOSPERS, J F DIRKS, M HADDERS-ALGRA
>0.8 from the age of 4 years, and particularly high for the Department of Neurology, Developmental Neurology,
mobility domain. The ratings based on defined scales were UniversftyMedical Centre Groningen,Gronfngen,the
more valid and sensitive/specificthan the global estimation. Netherlands
Discussion:The M-ADL is avalid tool requiringlittle time and no
specific training. It is useful in achieving an oveMew of the activi- Objectfve:Knowledge about the effect of specific elements of
ties of daily living of children with disabilities,to plan treatment an intervention is crucial for determining its effectiveness.
strategies,and to evaluate therapeutic interventions. The aim of the present study was to develop a standardized
observation protocol for the assessment of specific tech-
niques used by paediatric physiotherapists when applying
neurodevelopmental treatment (NDT) in infants with devel-
opmental motor disorders.
Methods: The first step consisted of a systematic analysis of
the literature available on the application of NDT in infants

0 EACD Abstracts 2005


with developmental motor disorders. In the second phase 10 20 BOBBA
video recordings of infant NDT sessions were analyzed in Idiopathic central nervous system ischaemia
terms of overt and directly observable therapeutic actions. In in childhood: past and present outcomes
the final phase the therapeutic actions were classified into BIANCA BASSI, BARBARA FULVA BOBBA, ANNA SALWNO
specific categories of NDT activities. University of %in, Department of Pediatrics, Child
Results:The reiterative process ofvideo-observationand litera- Neuropsychiatry Unit, Turin,Italy
ture analysis resulted in an observation protocol by which the
actions of a therapist applying NDT can be quantified. The pro- Objective: Although stroke in childhood is certainly less fre-
tocol classifies actions into the following six categories: (1) quent than in adulthood, it is sociallyrelevant because of the
Facilitationhnhibitiontechniques, (2) Tapping techniques, (3) severity of outcome. The aims of the present study were two-
Sensory experience, (4) Passive motor experience, (5) Active fold: first, we wanted to evaluate the neurological and neu-
motor experience, and (6) Parental involvement. The protocol ropsychological outcome of a cohort of children with idio-
allowed translation into algorithms of the computer program pathic central nervous system (CNS) ischaemia after the
‘The Observer’, thereby offering the possibility of multilevel injury. Second, we wanted to compare the present outcome
quantitative analysis of actions during an NDT session. of the patients with those who had a CNS stroke in the past
ConcZusion:It is possible to develop a standardized observa- (from the 1980s to the 1990s) when no immediate secondary
tion protocol for the quantification of NDT actions. Thus treatment with heparin was carried out.
time has come to open the black box of the application of Methods: Since October 1998 we have studied 14 patients
NDT in infants with developmental motor disorders. (group A: 10 females, 4 males) whose age ranges between 7
months and 13 years. Patients showed idiopathic CNS
ischaemia occurring during pregnancy until the age of 13
years. All these patients were treated with heparin and pro-
19 BOBBA phylactic treatment with ASA, or warfarin is currently in use
Case report: cognitive and language since thrombotic risk factors were assessed.
dysfunctions in a child with cerebellar Ten children (group B) who had a CNS stroke in the 1980s
hypoplasia (when no urgent medical treatment was carried out) were
BIANCA BASSI, BARBARA FULVA BOBBA contacted to assess thrombophilia risk factors and neuropsy-
University of firin, Department of Pediatrics, Child chological outcome. We compared the results of the assess-
Neuropsycbiatry Unit, Turin,Italy ment in these two cohorts of patients.
Patients were assessed according to the Raven Matrix,
Objective: The cerebellum plays an important role not only Wechsler Scale, Rey figure, logopedical, and psychomotor
in motor control but also in cognitive processing, language, evaluation just one month after stroke and after 6 months’ fol-
thought modulation, emotion, and organization of activities low-up.
in a sequential manner. Results: IQ was normal in both the groups but there was a sta-
We describe the case of a 5-year-oldfemale with a diagno- tistically significant difference among the two groups
sis, made during pregnancy, of cerebellar hypoplasia involv- (p c 005).
ing the vermis and hemispheres. This child’s development Conclusions:The importance of a complete thrombophlilia
has been followed since her birth. screening protocol and an immediate therapywith an antico-
She lives in a very good environment and her family agulant drug is emphasized. We further stress the need for
accepted her disability.At 2 years of age she underwent a sur- careful neurological diagnosis and extensive follow-up for
gical intervention for the insertion of a ventriculo-cerebellar better evaluation of the disease and prevention of further
shunt. episodes.
On the Griffith Scale, assessed once a year, she shows a mild
delay, mostly in Hearing and Speech and Performance.
She had physiokinesic therapy in her first year of life
because of a mild motor delay and at present, because of lan- 21 BOIX
guage disabilities (which are her most significant delay), she Reversible cognitive deterioration associated
is undergoing logopedical treatment. with valproate
We demonstrate her cognitive development assessed by c BOIX, A SANS, A COLOME, A LOPEZ-SALA, F X SANMARTI,
the Grif!ith Scale and present avideo that shows her previous J CAMPISTOL
motor difficulties and her current growth. HospitalSantJoan de Deu, Barcelona, Spain
Conclusions: Despite the anatomical damage involving the
vermis, this patient has a good modulation of her emotions. Objective: Cognitive disorders in epilepsy are frequent and
Her cognitive and language delay are mild. We underline the have a multifactorial origin. It is well known that antiepilep
importance of a early diagnosis of cerebellar malformations, tic drugs (AEDs) have a negative influence on cognition.
good neuropsychiatric follow-up, and discuss how the envi- There is the possibility of severe cognitive decline as a result
ronment can play a role in improving sequele of congenital a individual susceptibility.
brain damage. Cases have been reported regarding cognitive deterioration
associated with valproate P A ) . We present two patients with
neuropsychologicalimpairment during WA treatment.
Method: We studied two patients at 13 and 14 years of age
with the diagnosis of partial epilepsy. At the beginning of

9
cognitive deterioration, the drug therapies used were VPA visuospatial and visuoconstructive functions.
and clobazam (Observation 1) and VPA and topiramate
Acknowledgements: This work was supported by Handicap
(Observation 2). In both patients, serum levels ofVPAwere in
International and Association pour I’EducationTherapeutiqueet la
the normal range and there was no analytic sign of toxicity. Wadaptation des enhnts IMC APETREIMC.
EEG activity was normal when neuropsychological deterio-
ration was evident.
Results: Results in both patients showed cognitive deteriora-
tion with diffuse cerebral impairment.Reduction ofWA dosage
and subsequent discontinuation improved neuropsychologi- 23 BOURELLE
cal profiles. However, attentional and executive dysfunction Computerized posturography in children with
persisted. It is difficult to know if these residual deficits were a cerebral palsy after treatment of equinus
sequele of intoxication or were premorbid features. deformity
Conclusion: The adverse side effects of AEDs are more fre- 5 BOURELLE, J COTTALORDA,M C D’ANJOU, c PALAYER,
quent with higher doses, especially when more than one V GAUTHERON
drug is used. CHU de Saint-Etienne,France
A drug-induced toxicity effect should be considered when
treating patients with epilepsy who manifest cognitive dete- Objective:In the cerebral palsy (CP) field, assessment of reha-
rioration while undergoing treatment. bilitation, medical (invasive and non-invasive), and surgical
treatment has become essential to define benefits for
patients. Gait analysis has been used extensively to assess
objectivelythe effects of intervention on lower limbs. The pre-
22 BOUDIA requisite for walking is, however, adequate postural control.
Hemiplegic cerebral palsy: a longitudinal Therefore, when a treatment has improved gait parameters, it
study of intellectual development of 32 should improve postural control. Evaluation of postural con-
children from infancy to adolescence trol has been made easier by using computerized dynamic
B BOUDY, S G O N W E Z , A RITZ, C BERARD posturography The Balance Master System (NeuroCom,
Department of Paediatrlc Rehabilitation, CHULyon-Sud, Clackamas, Oregon, USA) offers technology for objective
mance assessment and comprehensive documentation of postural
control. It is a computerized device made for real-time move-
To evaluate the intellectual development of children with ment analysis o n a force plate. By using this technology, the
right versus left congenital hemiplegic cerebral palsy, 32 chil- aim was to evaluate postural control in children with CP
dren were studied longitudinally over a mean period of 7 before and after treatment of equinus deformity.
years 5 months. Verbal (VIQ), Performance (PIQ), and Full- Methods: Seven children with diplegic CP (4 females, 3
scale (FIQ) scales were administered. males) participated in the study. Assessment was conducted
Objectives: (1) to explore the relative stability or instabilityof before and after medical or surgical treatment of equinus
cognitive performance over time using available longitudinal deformity. Three static tests (Weight Bearing Squat [WBS],
data, (2) to define cognitive profiles according to sex and the Unilateral Stance, modified Clinical Test for Sensory Inter-
hemispheric side of lesions. action on Balance [ mCTSIB]), and two dynamic balance tests
Material and method: Thirty-two children (13 females and (Limits Of Stability [LOS] and Rhythmic Weight Shift (RWS])
19 males) were included in the study. They were divided into were performed on the Balance Master.
two groups: one left-hemisphere-impaired (LHI) group with Results: After treatment, mean weight-bearing asymmetry
right hemiplegia (n=14), and one right-hemisphere-impaired measured by WBS was improved at any knee flexion degree,
@HI) group with left hemiplegia (n=l8). CT and/or MRI especially when treatment was unilateral. In the mCTSlB all
were obtained for 29 children to locate and define the type children were improved. In the Unilateral Stance, results
and extent of brain lesion. were not homogeneous. The LOS was very difficult to per-
Intellectual function was tested with the Wechsler form for almost all the children. In the RWS, mean direction-
Intelligence Scales from one to three times. VIQ was assessed al control was improved in all directions.
with 5 subtests of the verbal subscale, PIQ with the 5 subtests Conclusions:The Balance Master System offers the opportu-
of the performance subscale, and FIQ with all 10 subtests. nity for objective and easy assessment of postural control in
Results: The majority of children had normal intelligence children. We are encouraged by our first results to continue
and 11 children had low FIQ scores (FIQC70). Results balance testing on children with CP by posturography, espe-
revealed that (1) the female group was more impaired in VIQ cially after a treatment.
than the male group: however impairments were restricted
to the females’ LHI group who also exhibited low PIQ and
stabilityof intellectual performance over time, and (2) insta-
bility of cognitive functioning occurred over time for the RHI
females and LHI and RHI males in whom verbal function
remained normal over time; whereas non-verbal function
was more likely to decrease.
Conclusion: LHI in females may be more limited in cerebral
plasticity than RHI females and males, and that the func-
tional reorganization of language occurs at the expense of

10 EACDAbstracts 2005
24 BOURG bral palsy (CP). The aims of this multicentre study were to
A comparative study of quality of life in three assess ( 1 ) the feasibility and efficacy of this complex tech-
sets of children affected by different nique, and ( 2 ) to determine its long-term advantages and
disabilities side-effects.
v BOURG",
M C D ' A N J O U ~ ,s MANIFICAT' From 2000 to 2002,12 French centres treated 24 children
aCRFPaulDottin, Ramonville; bCentreL'Escale,Lyon Sud; (6-18 years old) with CP; of these, only 19 eventually
CHbpitalSaintJeande Dieu, Lyon, France received an IBI?Patients were followed-up at 3,6,12,18,and
24 months. The immediate efficacy of the IBP was assessed
Quality oflife (QoL) is a useful tool in rehabilitation medicine. over a period of 3 to 5 days using the Ashworth score (upper
The QoL Auto-Questionnaire (AUQUEI; for children) has and lower limbs), whereas the long-term effects were evalu-
already been validated in children aged between 4 and 1 1 for ated using the Gross Motor Function Measure (GMFM). Any
its ease of use. AUQUEI evaluates QoL subjectively using 36 associated symptoms, such as perception of pain or skeletal
items scored between 0 to 4 ; some of which have been adapt- deformities were noted and a subjective evaluation from the
ed with respect to age. To have a better understanding of the patient, their family, and the health team was obtained.
way that children and their families cope with disability, and At 24 months there was a significant decrease in the
to envisage the best therapeutic goals, we gave the AUQUEI to Ashworth score for a daily baclofen dosage of 1862172mg.
children as well as a QoL measure to parents. The parents Although the GMFM did not show a statistically signrficant
assessed themselves and their child, whereas the medical change, the Bleck Locomotion Index enabled us to detect an
team assessed children in their care. improvement in the functional status of 41% of patients. There
The study population included 54 children with diplegia was also an improvement in subjective qualitative evaluationin
who were able to walk, and 23 children with isolated urinary 40 to 75%of patients. Pain was dramaticallyimproved in 66%of
disability; all were attending regular school. We also evaluat- patients. Seven adverse events were reported, mainly within
ed 28 children with quadriplegia who were unable to walk the first 6 months. Of these, five required surgical re-opera-
and who attended school in dedicated structures. tions; however, all IBPs are still functioning.
Results were compared to those observed in a population We conclude that IBP is an efficient technique that can
of 233 children without disability. When compared with con- reduce spasticity in children with Ce and it can improve their
trol children, QoL scores obtained from children with disabili- quality of life. Somatic symptoms need to be defined by the
ties is lower especially in those who are quadriplegics. patient, their family, and the health team to ascertain those
However the score is not all negative i t . a mean of >2. The who will benefit most from this technique.
type of disabilitydoes not alter the global score but does affect
self-image. For walking diplegic children they awarded higher
scores to their functional capabilities with lower scores for
their satisfaction. Medical staff always awarded lower QoLs to 26 BUMIN
the children especiallywhen they are in dedicated structures. Anxiety, depression, and quality of life in
Parents of children with disabilitiesalso experienced impaired mothers of children with disabilities
QoL which was positively correlated with the physical depen- GONCA BUMIN, AYLA GUNAL, SERMIN TUKEL
dency of their child. They also were less confident about the Hacettepe University,School of Physical Xberapy and
future. Rehabilitation, Ankara, Turkty
Assessment of children's QoL by parents was always
ranked higher, especially if the child lived in normal sur- Objective: In the population of mothers of children with dis-
roundings -although their score often did not correlate with abilities, anxiety and depression are the most frequent emo-
that defined by the child. This study shows that QoL is scored tional disorders. The purpose of this study was to determine
for the same child differently if performed by the child, hisher the relationship between anxiety and depression, and quali-
parents, or by the health staff. There is often confusion ty of life in mothers of children with disabilities.
between QoL and health status. A child who has never known Methods:The studywas performed at three rehabilitation cen-
normal health status has a life perspective based on hisher tres in Ankara. One hundred and seven mothers of children
own references. Therefore, it is important to base assess- with disabilities were included in the study. The Beck
ments on child and adult projections. Depression Inventory (BDI), the State-TraitAnxiety Inventory
(STAI), and the Nottingham Health Profile-Part 1 were admin-
istered. Assessments were performed during children's treat-
ment at the rehabilitation centres. In this study, Pearson's
25 BOURG correlations were used to analyze the relationship between
Implantion of an intrathecal baclofen pump in BDI, STAI, and the Nottingham Health Profile.
children with cerebral palsy: the results from Results: There was a significant correlation between BDI and
a two-year follow-up of a multicentre study STAl (r=0.344,p<0.01)and all subscales ofNottingham Health
v BOURG", Y LAZORTHES~,B S A L L E R I N ~ Profile (emotional reaction r=0.622, energy level r=0.416,
Paul Dottin, Rarnonville; bNeurosurgeryDepartment, pain r=0.463, sleep r=0.429, physical abilities, r=0.422,
UniversityHospital, Toulouse, France social isolation r=0.587;p<O.Ol). Statistically significant
correlations were found between STAI and emotional reac-
Implantation of an intrathecal baclofen pump (IBP) is one of tion (r=0.271,p<0.01),energy level (r=0.206,p<0.05),and
the techniques used to treat lower-limb diffuse spasticity It sleep (r=0.252,p<O.Ol) of the Nottingham Health Profile.
has been used in Toulouse since 1990 for children with cere- There was a significant correlationbetween mothers' education

11
leveland STAI (r=-0).209,p<O.O5).Asign1licantcorrelationwas the effect on cardiac variability using a method previously
found between mothers’ education level and the Nottingham validated.
Health Profile pain scale (r=-0.240,p<0.05). There was no Participants: Bventy children with CP requiring high dose
correlationbetween STAI and all of Nottingham Health Profile treatment ages 4-14 years.
subscales. Method: Gradually increasing total doses were given at each
Conclusions:The findings of this study indicate that mothers treatment, 4 to 6 monthly, using a strict injection protocol.
of children with disabilities tend to have anxiety and depres- Questionnaires on side effects were completed at 2, 6, and
sion. Increased depression and anxiety levels had negative 24 weeks after treatment. Cardiac variability was measured
affects on mothers’ quality of life. Researchers should deter- for 6 hours overnight before, and 2 weeks after, each course
mine whether anxiety, depression, and quality of life affect of treatment. Changes were expressed as the autonomic
mothers’ abilities to provide rehabilitation and emotional ratio (AR) .
care for their children during and after rehabilitation. Results: Sixty seven treatments were monitored (range 13-50
unitshg) and all patients exceeded 30unitshg. Six episodes in
five participants were associated with systemic side effects at
total dosages 37, 39, 40, 43, 44, and 49unitshg. These were
mainly in the form of mild weakness in non-injected muscles
27 BUMIN lasting 3-30 days, and were not associated with an obvious
Comparison of somatosensory perception in change in AR measurements. Fifteen participants tolerated
children with preterm and term cerebral palsy high-dose BTA up to 50unitshg without side effects.
GONCA BUMIN, MINE U M N K , HULM KAYHAN Comparison between episodes with and without systemic
Hacettepe University,School ofPhysical Therapyand side effects showed higher total dose for the former (42 vs
Rehabilitation, Ankara, nCrkey 36unitskg) without reaching statisticalsignificance (p=0.06).
There was also no differencesin total number of injections (12
Objective: The aim of this study was to compare somatosen- vs 12.9)oraverage dose perkilogramofinjections ( 3 . 7 3.9).
~
sory perception of children with preterm and term cerebral However, a significant difference in AR was found (1.11 vs
Palsy (W. 0.87,p=0.038).
Methods: This study was carried out at the School of Physical Conclusions: Multilevel BTA treatment at dosages above
Therapy and Rehabilitation of Hacettepe University’s Occu- 30unitskg (Dysport) can be given safely using gradual incre-
pational Therapy Unit. Thirty-nine children diagnosed with ments. Some participants tolerated more than others.
diplegic CP were assessed. Children were randomly divided Cardiac variability did not reliably predict the development
into two groups. The study included 20 preterm (group 1) and of systemic side effects.
19 term children with CP (group 2). Mean age for group 1was
7 years 3 months (SD 1.2) and group 2 was 7 years 1month (SD
1.5). Somatosensory perception was assessed using Ayres’
Southern California Sensory Integration Somatosensory Sub- 29 CANITANO
tests (Double Tactile Stimuli Perception, Localisation of Tactile Tourette syndrome and autism spectrum
Stimuli,Graphestesia, Kinaesthesia, Finger Identification,Man- disorders
ual Form Perception). ROBERTO CANITANO, GlACOMO VIVANT1
Results: There was a significant difference between preterm Division of Child Neuropsychiatry, General University
and term CP (p<0.05). Children with term CP had higher Hospital of Siena, Sfena,ItaIy
scores in somatosensory perception.
Conclusion:The somatosensory perception of children with Autistic spectrum disorders (ASDs) are associated with tic
term CP was better than children who were preterm. Gest- disorders at a rate that is higher than expected by chance.
ational age appears to affect somatosensory perception in Variable rates of comorbidity have been reported and com-
children with CE mon genetic and neurobiological factors are likely to be
involved. The aim of this study was to determine the rate of
tic disorders in a clinical population of children and adoles-
cents with ASDs, and to describe the clinical characteristicsof
28 CAMPBELL the group with comorbidity. The overlapping between tics
High dose botulinum treatment in cerebral and other repetitive movements and behaviours in ASD was
palsy using cardiac variability to monitor carefully assessed. Among 105 individuals with ASD repre-
systemic side effects senting the study group, 22%of patients presented tic disor-
v CAMPBELL*, L WATSON~,R E MORT ON^,^ ders: 11% with Tourette Disorder (TD) and 11%with chronic
“QueensMedical Centre,Nott fngharn;bDerbyshire motor tics. All patients had various degrees of cognitive
Children’sHospital, Derby, UK impairment. A correlation between level of mental retarda-
tion and tic severity was found.
Introduction: The recommended maximal dose of botulinum
(BTA; Dysport) in cerebral palsy (CP) is 30unitshg, to avoid
systemic side effects, but sometimes more is needed. We
have treated children with higher doses on the basis that this
would be tolerated if split between more muscles for multi-
level treatment. Systemic spread of BTA was monitored by

12 EACD Abstracts2005
30 CANS All participants were evaluated by the neurologist and they
Origins of severe neuro-sensorial deficiencies fulfilled thehbury and Cornblath criterion. The information
in school-aged children was obtained from the clinical history of the patients.
CANS, P GUILLEM, C TRONC, G MAESTRI, P S JOUK Absolute numbers and percentages were used for statistical
RhTEOP ChildrenDisability Registet Grenoble,France analysis.
Result: W o peaks of incidence were found in 1992 and 1996.
Aims: Despite improvements in neonatal and obstetrical Males were much more affected by the disease. The highest
care, the prevalence rate of children with disabilities has not incidence was between the ages of 1 and 10 years old. Acute
decreased. The aim of this work was to describe the timing of respiratory infections and acute diarrhoea were the most fre-
the aetiological event responsible for the disability, and to quent precedent factors. Moderate axonal lesion was the
check if any change over time could be detected. most frequently electrophysiological pattern and was related
Methods: Data collected by a morbidity register on children to the most severe motor weakness.
with disabilities born between 1980 and 1995 were analyzed, Conclusion: GBS in children had a good prognosis indepen-
taking into account available information on aetiology for chil- dent of the therapeutics applied.
dren with severe motor, intellectual,or sensorial deficiency, i.e.
children with neuro-sensorial deficiencies (NSDef). The infor-
mation relied on the aetiological event of the main deficiency
when such an event was clearly identified, e.g. postneonatal 32 CHAIX
meningitis, prenatal hereditary deafness, or other prenatal dis- Intensive training in developmental dyslexia:
orders. Otherwise, the presence of perinatal risk factors non-specificity of effects
@reterm birth <32 wks, birthweight <15OOg, NCU hospital- Y C H A I X * , ~ ,M TRABANINO~,c BILLARD~,L SPRENGER-CHAROLLES~,
ization, hypoxic-ischemia encephalopathy,periventricularleu- JF DEMO NET^
comalacia) facilitatedthe ability to distinguishthose conditions aUnitbde NeurologiePediatrique, Hdpital des Enfants,
of probable perinatal origin from those of unknown origin. Toulouse;bINSERM U 455, H6pital de Purpan, Toulouse;
Results: There were 1370 children with severe NSDef, the Wnitede Neurologie Pediatrique, Hbpital Kremblin-
prevalence rate being 5.9per 1000 (CI 95% ,5.6-6.2). Bic&tre,Paris; dCNRS Facult6 deJussieu, Paris, France
Among them, 745 had severe motor deficiency (483 of
whom had cerebral palsy), 587 had severe mental retarda- Objective: In developmental dyslexia, phonological prob-
tion (IQC 50), and 344 had severe sensorial impairment. The lems are the most prominent difficulty. Some theories
origin of NSDef was prenatal in 46% of children (18% were emphasize the role of low-level perceptual deficits in
chromosomal abnormalities), postneonatal in 9%, probably impaired phonological processes. Intervention based on
perinatal in 20%, and remained unknown in 25%. According auditory temporal processing deficits have shown inconsis-
to the type of deficiency, the proportion of deficiencies with tent gains in reading skills, whereas improvement in word
unknown origin varied from 13 to 50%. The proportion of reading accuracy after intensive phonologically-based train-
probable perinatal origin was highest among severe motor ing has been regularly reported. The first aim of this work
deficiencies (31%). Prenatal origin was most frequently was to compare the effects of two types of phonological
observed (57%)in severe intellectual and visual deficiencies. training: non-modified speech and slowed-down rate of
N o significant difference over time could be observed for speech. The second aim was to confirm the specificity of
any origin except the proportion of severe motor deficien- phonological training by contrasting it with visual training.
cies of unknown origin which decreased significantly from Methods: lkenty three children with developmental dyslex-
26 to 14% (p=0.006). ia, aged 8 to 12 years from two language disorder centres
(Paris and Toulouse), were recruited. Children with pure
visual dyslexia and/or specific language impairment and/or
attentional deficit were excluded. Treatment consisted of two
31 CASAMAJOR CASTILLO intensive (daily) training periods of 8 weeks, a phonological
Some considerations about the and a visual intervention administered using a cross-over
Landry-Guillain-Barre syndrome in design. 'helve children out of 23 received phonological
childhood: twelve years of study training with slowed-down speech. Neuropsychological
MAWS AIME CASAMAIOR CASTILLO, LUIS RODRIGUEZ RODRIGUEZ assessments were conducted three times: before training,
SouthernPediatric UniversityHospital, Santiago de Cuba between, and at the end of the two training periods.
Results: The study showed a significant improvement after
Introduction: Landry-Guillain-BarrkSyndrome (GBS) is the two training sessions in reading, accuracy, fluency, and
most common demyelinating disease of the peripheral ner- metaphonological abilities. This result was found in the nor-
vous system. People of all ages are affected but the highest mal and the slowed-down speech groups: no interaction was
incidence is in persons between the ages of 50 and 70 years. found for speech rate. The second result was a significant
The syndrome may follow a mild viral illness or appear with- improvement in the same abilities without effect of the train-
out prodrome. Its prevalence is 0.4-2.4x 100 000 persons. A ing modality: phonological versus visual.
retrospective and transverse study was made of 35 children Conclusions: The absence of specificity of training effects
with GBS who were admitted to the Southern Pediatric observed may relate to the important inter-participant vari-
University Hospital of Santiago de Cuba from 1 January 1991 ability of performance that was found despite careful screen-
to 31 December 2002.Patients with GBS were characterized ing of participants. It might also be linked to some features of
by means of demographic, clinical, and laboratory variables. training that were given without explicit feedback to children.

13
Finally these negative results also underscore the impact of ferent points of view regarding how low the temperature has
‘non-specific’cognitive factors, such as attention, which may to be to achieve effective myorelaxation.
account for the general improvement observed regardless of Methods: Twenty four ambulatory children with spastic
the type of training. diplegia aged between 5 and 14years were studied. Cold ther-
apy included 10consecutive procedures with local application
over the triceps surae muscle of one leg. The first group was
33 CHARLES treated with a temperature of 0°C (with ice cubes) and the
Efficacy of intensive practice on upper second group was treated with a temperature of -10°C (with
extremity movement in children with a freezingdevice). Treated muscles were tested clinically (the
hemiplegia ankle’s passive range of motion, reflex excitability, and
J CHARLES, A M GORDON Ashworth scale) and with EMG (latency and H-reflex) before
Teachers College,Columbia University,New York,M: USA treatment and at the first, third, and tenth procedure during
cold applications.
Impaired hand function is one of the most disabling symp- Results: The second group treated within minus -10°C had
toms of hemiplegic cerebral palsy (CP). Intensive practice of better clinical results (increased passive range of motion and
the involved hand maybe beneficial. One method to elicit such decreased reflex excitation) and EMG changes (increased
practice is constraint-induced (CI) therapy which involves latency and decreased H-reflex)in treated triceps surae mus-
restraint of the non-involved hand and intensive practice cles. Ashworth scale scores were similar for the two groups.
with the involved hand. In its original form, CI therapy is not N o significant difference in clinical or EMG changes during
child-friendly. Furthermore, CI therapy focuses exclusively consecutive procedures was observed in the two groups, but
on the involved extremity, while increased functional abili- slightly more EMG changes were recorded on the 10th day
ties require use of both hands. by the second group.
Objectives: Results of a randomized control trial of CI thera- Conclusions:Cold therapy is more effective at treating spas-
py were presented. We then tested whether the same results ticity in children with CP if used at lower temperatures gener-
could be achieved with a new therapy: hand-arm bimanual ated from freezing devices.
intensive therapy (HABIT).
Methods: Rventy-two children (4 to 8 years of age) were ran-
domized to an intervention group (n= 11) or a control group
(n=11). Children wore a sling on their non-involved upper 35 CHEVIGNARD
extremity for six-hours per day, during 10 out of 12 consecu- Use of constraint-induced therapy for children
tive days. They were engaged in play and functional activities with hemiplegia due to acquired brain lesions
that provided structured practice using the involved upper V AZZI, M CHEVICNARD, D G BRUGEL, G ABADA, C LEMESLE,
extremity,An additional six children received HABIT without S BUR, H TOURE, A LAURENT-VANNIER
a sling. Blinded evaluations occurred once before the inter- Service de rbeducation despathologies neurologiques
vention and at one week, one month, and six months post- acquises de l’enfant,H6pital National de Saint Maurice,
intervention. Saint Maurice, Cedex, France
Results: Children in the CI therapy group demonstrated
improved scores on the Jebsen-Taylor Test and subtest 8 of Introduction: Constraint-induced movement therapy is
the Bruininks-OseretskyTest (p<0.05 in both cases). Initial effective in treating adult hemiplegia and has occasionally
severity of hand impairment and testing compliance account- been used in children.
ed for 73%of the variance in changes from pre- to post-inter- Objective: To study the feasibility and efficacy of this method
vention scores. Preliminary results suggest similar changes of treatment in children.
may be elicited without the restraint. Method;Three children (A, B, and C), aged 5 years 1month, 5
Conclusions:Results suggest that CI therapy modified to be years 2 months, and 5 years 6 months respectively,tookpart in
child friendly improves movement efficiency of the involved a constraint-induced therapy programme. The children had
hand in children with hemiplegic CP However, not all chil- right hemiplegia and predominant brachiofacial involvement,
dren may benefit. Coordination of the two hands may be Zancolli classification 1, 2A, and 2B due to stroke, head trau-
improved through intensive practice of bimanual tasks. ma, and brain tumour which occurred 1 year, 2 years, and 9
months earlier.
Baseline evaluation consisted of the ‘nine peg holes’ test,
box and blocks test, conekylinder displacement, test tower
34 CHAVDAROV of blocks, and a neglect assessment battery.
Influence of different low temperatures on The unaffected arm was then immobilized with a Mayo
spasticity of children with cerebral palsy: Clinic contention for 7 hours per day. Three hours of physi-
a comparative study cal therapy on the affected arm was given each day, 5 days a
IVAN CHAVDAROV, P MINEVA, E TODOROVA week for 2 weeks. Baseline tests were repeated at week4.
Specialized Hospitalfor Residential Reatment of Results: The protocol was carried out in its entirety, Perform-
Prolonged Therapyand Rehabilitation of Children with ances improved on timed tests. Qualitatively there was better
CerebralPalsy, Sojia, Bulgaria control and better quality of movements.
Children B and C, who also had severe visuospatial and
Objective:Cold therapy is used for childrenwith spastic cere- motor neglect in addition to right hemiplegia, showed a
bral palsy (CP) as a method of myorelaxation. There are dif- reduction in the number of right-sided omissions in the

14 EACDAbstractsZOO5
teddy bear cancellation test. Lack of experience of students with special needs in terms of
Discussion:Provided that the child is well informed and that environmental adaptation was shown to influence the results.
playful activities are proposed, constraint-induced therapy The most important source of information is the student.
can be beneficial. It was effective for improving motor perfor- The views of parents and teachers also need to be taken into
mance, with better voluntary control of movements (distal account.
and proximal), greater awareness of the affected side of the
body and its possibilities, and more symmetrical use of the
body. However, there was no improvement in USN task per-
formance although there was more involvement of the right 37 CIONI
hemispace. Clinical forms of hemiplegia in children:
Conclusion: This method can serve as an additional tool for relationship with time and timing of the lesion
rehabilitation of hemiplegia. G CIONI, S MONTI, P B PAOLICELLI, E PETACCHI, S LANCIANI
Department of Developmental Neuroscience, Stella Maris
Reference ScientificInstitute, Pfsa,Italy
1. Kannan N, MarylesJ, Baker RX.(2003) Constraint-induced
movement therapy for hemiplegic children with acquired brain
injuries.JHead 7kaumaRebabill8 259-267. Objective:Magnetic resonance imaging (MRI) of 142children
with hemiplegic cerebral palsy was analyzed with the aim of
categorizing their features into fairly homogeneous forms.
Methods: MRI findingswere classified into four groups accord-
36 CIBULE ing to the pathophysiologicalmechanisms of the lesion thought
Participation in school activities in to be responsible for the hemiplegia, and its probable tim-
adolescents with nhssical disabilities: ing. Form 1 comprises brain malformations, form 2 grouped
examination of students and environmental abnormalitiesof the periventricularwfiitematter, form 3 repre-
adaptation sented cortical-subcortical lesions, and form 4 grouped non-
L CIBUL@, A GREITANE~,I JEKABSONE", E AUZINA~ progressive postnatal brain injuries. Four main forms of
aRiga Stradins University;bChildren'sRehabilitation childhood hemiplegia were shown to have distinct clinical
Centq Mes esam lidzas,Riga, Latvia features.
Results: None of the children had normal MRI. A high inci-
Background: There were 10 055 children with movement dence of unilateral lesions were found in all except form 2.
and developmental disorders in Latvia in 2003. However, Left motor involvement was prevalent in the second and
only 1663were integrated into mainstream schools. fourth forms. Severity of impairment was mainly moderate in
Inclusion of children with special needs in mainstream forms 1 and 3, and mild in the others. The upper limb was
schools versus inclusion in special schools has become a new found to be more affected in all forms except the second one,
strategy for Latvia since the country's acceptance into the which presented a greater involvement of the lower limb.
European Union. Mental retardation occurred in about one-fifth of the sample,
It is important to identdy main factors influencing partici- mostly in children with forms 1 and 4. Seizures occurred in
pation in school life so that intervention strategies for families, about half of the children with forms 1 or 3, while the inci-
health care professionals, and community social workers can dence was lower in forms 4 and 2. A strong correlation was
be developed. observed between the presence of seizures and mental retar-
Objective: To determine the adjustment needs of students dation. A correlation between forms of hemiplegia and fea-
with physical disabilities in the school environment by using tures of sensorimotor reorganization indicated by fMRl and
different sources of information: students, parents, and TMS was also found.
teachers. Conclusions: This study confirms the importance of timing
Participants: Thirteen students with physical disabilities of brain lesions, as revealed by MRI, to indicate specificforms
(mean age 14y 6mo) from two mainstream schools, as well as of hemiplegia with different clinical features.
13 parents and 13 teachers took part in the study.
Instruments:The School Setting Interview and physical envi-
ronment observation checklist were used for assessment.
Results and conclusions: Results are based on interviews with 38 CIONI
students, parents, and classroom teachers, as well as evalua- Reach to grasp in children with spastic
tion of objects in the physical environment. Participation in hemiplegia: role of the head, trunk, and upper
the school environment was discussed in terms of students, limb
environmental adaptation, necessity for adjustments from M C O L U C C I N I ~E, S M A I N I ~c, MARTELLONPJ',
c SGANDURRA~,
different viewpoints, satisfaction with adjustments of envi- s LANCIANI~,G CIONI"
ronment, and ability to function in school setting. aDepartment of Developmental Neuroscience, Stella Maris
To analyze the relationship between the responses of dif- Scientijic Institute, Pisa; bScuolaSuperiore di Studi
ferent groups, the correlation was determined. The lowest Universitari e Perfezionamento Sant 'Anna,ptsa, Italy
correlation (0.4187) was between the answers of students
and teachers, and the highest was between the answers of Objective: The ability to reach and grasp objects is a funda-
parents and teachers (0.7086). Comparing the responses of mental aspect of many activitiesof daily living. The aim of this
students and parents resulted in a correlation of 0.5865. study was to investigate upper limb performance during a
AU answers revealed the need for individual adjustments. functional task (reach-to-graspand release) in children with

15
hemiplegia compared with controls. The relationship births). These year effects were independent of the variation
between motor impairment of the upper limb and presence in prevalence by centres. For those with birthweight <2500g
of compensatory movements of the head and the trunk was there appeared to be an increase over time, peaking in 1988
also studied. (prevalence 1.2/1000),but this was not sustained.
Methods: Patterns of movement of the head, trunk, and upper Adjusting for unrecorded data, 50%(201/402) of children
limb were studied during a reach-to-grasp and release task. had significantcognitive impairment with IQC50, and 57.8%
Ten children with spastic hemiplegia participated (mean age (278/481) were non-ambulant; 38.3%(148/386) had low IQ
9 y 5mo) as well as an age-matched group of 10 children with- and were non-ambulant and were thus severely impaired.
out disabilities (mean age 8y 10mo). Data acquisition was There was no significant change in the proportion of chil-
carried out using an optoelectronic motion analyzer with 8 dren severely impaired over the study period.
IR cameras (IOOHz).Clinical assessment was also performed Conclusions: Prevalence and severity of dyskinetic CP have
using the Melbourne Assessment of Unilateral Upper Limb remained stable over the period 1976-1996. Although the
Function. prevalence in the low birthweight group appeared to increase
Results: Preliminary results reinforce the evidence that chil- until 1998,it subsequently decreased. Overall, the proportion
dren with hemiplegia showed patterns of movement with of those with severe dyskinetic CP remained unchanged.
prolonged duration, decreased shoulder flexion, and
decreased elbow and wrist extension. In terms of the rela-
tionship between upper limb and compensatory move-
ments, children with hemiplegia generally used a strategy of 40 COLOME
increasing trunk anterior displacement and rotation to trans- Non-verbal learning disorder:
port the hand to the target. A correlation between clinical neuropsychological assessment of 20 children
assessment and quantitative data was found. R COLOME, A SANS, A LOPEZ-SAM, C BOIX, M MARISTANY,
Conclusions: Quantitative analysis confirms the clinical evi- R GASSIO, J CAMPISTOL
dence that children with hemiplegia perform upper limb Department of Neurology, SantJoan de Deu Hospital,
movements with a different level of impairment, even if each Barcelona, Spain
individual develops alternative compensatory strategies to
complete tasks. Current research aims are to increase the Background:Non-verballearning disorder (NVLD) is a neuro-
study of normal and pathologic populations and to develop a logical condition that is considered to be a developmental dis-
quantitative index for assessing the smoothness, repeatabili- ability It is characterized by a specific dysfunction in motor
ty, coordination, and the role of compensatory movement visuospatial-organizational and social skills in patients with
during task performance. normal IQ and development of language. Indications of the
condition are poor psychomotor coordination, mathematic
skills, and painting activities. Current evidence and theories
suggest that dysfunction of white matter in the right hemi-
39 COGHLAN sphere could be the cause of NVLD. Clinical characteristics
Dyskinetic cerebral palsy in Europe: trends in and diagnostic criteria have not yet been fully agreed.
prevalence and severity Objective: To describe the most relevant clinical manifesta-
DAVID COGHLAN O N BEHALF OF SCPE COLLABORATION tions and cognitive-behavioural profiles of 20 children with
(SURVEILLANCE O F CEREBRAL PALSY IN EUROPE) NVLD.
Department of Paediatrics, Mercy University Hospital, Patients and methods: The sample comprised 20 children,
GrenvillePlace, Cork,Ireland aged between 7 years and 14 years, diagnosed in our neu-
ropsychological unit. The main concern of parents attending
Objective: To study the prevalence rates and clinical severity in our unit was their children’s learning difficulties at school.
of children with dyskinetic cerebral palsy (CP), including Results: Neuropsychological assessment reveals visuospatial
dystonic and choreo-athetotic types, across 14 European and visuoconstructive difficulties, dyscalculia, dysgraphia,
centres participating in the SCPE collaboration. and attentional deficits. A majority also had affective prob-
Methods: All children with dyskinetic CP born in the years lems, such as depression and anxiety.
1976-1996 entered the study. Post-neonatal cases were Conclusions:NVLD is a developmental disabilitywhich often
excluded. Point prevalence was calculated using live birth goes undiagnosed. We emphasize the importance of an inter-
data. Severity was determined using intellectual impairment disciplinary team to arrive at a diagnosis. Motor clumsiness,
and walking ability as measures. Poisson regression analysis low visuoconstructive and visuospatial skills, and social diffi-
was used to study trends over time. culties may indicate a diagnosis of NVLD. Neuropsychological
Results: There were 502 children (6.8%of all CP cases) iden- examination reveals a specific profile for each child that is
tified with dyskinetic CP During the study period there were necessary to address and accommodate an educational
4 564 159 liveborn infants recorded, giving an average preva- programme.
lence of 0.11/1000 live births (CI 95%0 . 1 4 1 2 ) . Prevalence
varied over time but there was no evidence of a linear trend
over the study period (average annual rate ratio R R = l . O O
p=0.90). When each year was examined in relation to the
first year of the study, there was significant evidence that the
prevalence was higher in theyears 1984 (RR=2.11,pC0.05)
and 1988 (RR=2.13, p<0.05; prevalence 0.17/1000 live

16 E4CD Abstracts 2005


41 COLVER Adeli Suit). A dynamic axial-loading orthosis (DALO), con-
Development of an instrument to assess sisting of elastic tethers extending shoulders to feet, was
environmental factors across Europe relevant used with intensive therapy during this study. A volunteer
to children with cerebral palsy: validation and sample (n= 57) included children aged 3-12 years with spas-
preliminary data tic diplegia categorized by Gross Motor Functional Class-
ALLAN COLVER, KATHRYN PARKINSON, HEATHER DICKINSON, ification System (GMFCS I, n=6; 11, n320; 111, n= 17; n= 14;
ROBERT FORSYTH, ON BEHALF OF SPARCLE GROUP and\! n=O).All participants randomized into control (n=28)
UniversityofNewcastle upon p n e , Newcastle, UK and experimental groups (n=29) received physical, occupa-
tional, and speech therapy 3 times a week for 8 weeks, fol-
Objective:To develop an instrument to assess environmental lowed by a 4-week home programme. The experimental
factors believed to facilitate or hinder participation of chil- group were fitted with a DALO during weeks 4-8. The Gross
dren with cerebral palsy (CP). Motor Functional Measure (GMFM) was administered at 4-
Methods: Literature review, qualitative interviews in the UK, week intervals.
and focus group work with parents in six European coun- Results: Mean GMFM change scores were compared for each
tries generated a prototype instrument which was adminis- interval. Analysis of covariance (ANCOVA) was calculated
tered to 600 children in the UK with a variety of severe from baseline to week 4 to offset initial differences due to
impairments.A revised questionnaire was then administered prior ‘therapy effect’. Comparison demonstrates no statisti-
to 800 children with CP across Europe. cally significant difference in mean change scores between
Results: The qualitativework identified five main dimensions: groups (4-8 weeks p=0.964 and 8-12 weeks p=0.682)
transport, support to child and family, attitudes, individual across all GMFCS levels combined or within level.
mobility, and bureaucracy. Questions concerning these Conclusions: Despite lack of statistical significance in com-
dimensions were developed to determine how much the paring axial-loadingwith intensive therapy to therapy alone,
presence or absence of 60 environmental features facilitates clinicallyrelevant improvements were noted for both groups.
or hinders participation. Mean GMFM change scores of a sample population docu-
Principal Component Analysis on the prototype adminis- mented in the GMFM User’s Manual were used for compari-
tered to 600 children in the UK, generated five main compo- son. Children participating in intensive therapy withhvithout
nents accounting for 50% of the variance. The components DALO demonstrated greater mean change scores at 4,8, and
reflect support that the child and family receive from profes- 12 weeks than were documented for the sample population
sional services and family (Cronbach Alpha 0.9), physical over 6 or 1 2 months. This supports the belief that intensive
accessibility (0.85), educational support (0.7,transport therapy over a defined time period may result in clinically sig-
(0.6), and attitudes (0.4). There was a strong correlation nificant improvement in gross motor function.
between how much and how frequently the environment is
reported to exert its influence (Kendall’s Rank correlation Acknowledgements: This study has been funded by grants from the
coefficientp~0.001for all questions). Some questions, such United Cerebral Palsy Research & Education Foundation and the
Children’sResearch Center of Michigan.
as those concerning emotional support from the family, are
important and almost always answered as present.
The metric properties of the instrument and data on the
800 children with CP across Europe are presently being gath-
ered and will also be presented. 43 DALLMEIJER
Conclusions:This instrument appears to have robust metric Development of a mobility questionnaire in
properties. Such an instrument is essential for testing quanti- children with cerebral palsy
tative hypotheses and evaluating interventionswhich assume A I DALLMEllER , L D ROORDA, V A B SCHOLTES, J C BECHER
the model of the International Classificationof Functioning. Department ofRehabllitation Medicine, W University
Medical Centre,Amsterdam, the Netherlands
Acknowledgement: Supported by European Commission Research
Framework 5, Grant number QLG5-CT-2002-00636.
Objective:The purpose of this study was to develop a clinical
instrument for measuring limitations in mobility (MoVra) in
children with cerebral palsy (CP) using Rasch analysis.
Methods: The MoVra is a Dutch questionnaire that was devel-
42 DABROWSKI oped for measuring mobility in children with CP aged 2 to 12
Effect of axial-loading and intensive therapy years. The current version of the MoVra (1.3) measures diffi-
on gross motor function in cerebral palsy culty in performing 47 mobility tasks as rated by parents or
E DABROWSKI, G BACKER, R THOMAS, G PARKER, S SICONOLFI caregivers. Items are scored on a 9-point rating scale ranging
W a v eState University Children’sHospital of Michigan, from ‘no difficulty’ (score 1) to ‘not possible’ (score 9). The
Detroit, Michigan, USA responses of 325 parents of children with CP were analyzed
(Gross Motor Functional Classification System [GMFCS]:
Objective:To describe results of a randomized blinded study levelI,n=157;II,n=86;III,n=61; R n = 2 1 ) . Childrenwere
testing the hypothesis that ‘axial-loading’ in combination aged 7 years 1month (SD 2y 11mo) .
with therapy yields greater gains in gross motor skills of chil- To identify items that form a uni-dimensionalscale (i.e. fit to
dren with cerebral palsy than therapy alone. the Rasch model), the current 47 MoVra items were analyzed.
Methods: Axial-loading was derived from the work of the Items that did not fit the Rasch model were excluded. In addi-
Russian Space Agency and modified for clinical use (e.g. tion, invariance of item difficulties across GMFCS levels, sex,

17
and age groups was investigated for the remaining items. 45 DE GRAAF-PETERS
Results:Analysis showed that 35 out of the original 47 items, Specific postural support promotes variation
with answering categories reduced from 9 to 5 levels, in motor behaviour of infants with Minor
revealed a fitting model for children with GMFCS levels 1-111 Neurological Dysfunction
and aged 4-12 years. Results indicate that these 35 items VB DE GRAAF-PETERS, A H DE GROOT-HORNSTRA, J F DIRKS,
measure mobility on an unidimensional scale. Item difficul- M HADDERS-ALGRA
ties were found to be invariant in all 35 items for sex, in 33 Department of Neurology, Developmental Neurology,
items for GMFCS level, and in 3 1items for age group. Further UniversityMedical Centre Groningen,Groningen, the
analyses are required to identify whether subsets of items Netherlands
can be constructed for children with GMFCS N and lower
age ( ~ 4 y e a r s ) . Objective:Specificpostural support is said to improve motor
Conclusions: Present findings shows that 35 items of the behaviour of infants with a developmental motor disorder.
MoVra with 5-point rating categories reveal a uni-dimensional However this claim has never been studied systematically.
scale for measuring mobility in children with CI! Reliability and The present study aims to evaluate the effect of postural sup-
responsivenesswill be established in future studies. port on motor behaviour of infants with and without minor
neurological dysfunction (MND) . The study addressed sever-
al questions. Does application of supportive pillows affect the
time during which the infant exhibits general movements
44 DE CASTELNAU (GMs) or specific movements? Specific movements were
Dynamic and electrophysiological approach to defined as occurring in specific parts of the body in a specifi-
developmental coordination disorder cally recognizable way. Does application of pillows improve
P DE CASTELNAIJ~J’, ST J M ALBARET~,P G Z A N O N E ~ Y
, CHAIX~ the quality of GMs or repertoire of specific movements? Is a
aToulouse University;bToulouseHospital, France pillow effect affected by neurological condition?
Methods: Forty healthy full-term infants aged 1-5 months
Objectiw Childrenwith DevelopmentalCoordinationDisorder participated in the study. ’Itventy infants were neurologically
(DCD) have persistent difficulties in activities of daily living normal and twenty had MNE) (mildly abnormal GMs and/or
that require motor coordination. This disorder is specific, mild neurological dysfunction. Spontaneous motor behav-
and not due to a general medical condition, but its aetiology iour in supine position was video recorded for 180 seconds
is still unknown. A dynamic perspective on motor coordina- under four conditions applied in random order: support by a
tion proposes that behavioural stability stems from self-orga- pillow in (1) the shoulder region; (2) the pelvic region; (3)
nized synchronization of the subcomponents. The present shoulder and pelvic region; and (4) no pillow support.
study investigatesthe hypothesis that the lack of motor coor- ’Ikoindependent assessors evaluated the quality of GMs.
dination in DCD relates to a more general disorder of syn- The other movement parameters were assessed with a com-
chronization which is observable at behavioural and puter program. Duration of movements was determined and
electrophysiological levels. a variation-index consisting of the number of different specif-
Methods: We recorded EEG activity (Neuroscan system) with ic movements in a condition was calculated.
32 electrodes in 24 children with DCD (aged 8 to 13 years) Results: The presence of pillows did not affect the time spent
and 60 control children while performing a sensorimotor with GMs, specific movements, or GM quality. But it did
coordination task. Children had to synchronize and rhythmi- affect the variation-index. In infants without neurological
cally move their preferred hand with avisualperiodic stimulus: conditions the shoulder pillow, with or without a pelvic pil-
frequency was increased stepwise. They also underwent a low, resulted in an increase in the variation-index (p<O.Ol);
visual continuous performance test aimed to assess the puta- in infants with MND all pillow conditions resulted in a sub-
tive attentional contribution to the motor deficit. In parallel stantial increase of the movement repertoire (p<0.001).
to stability measures, in terms of the variability of perfor- Conclusion:Specific postural support promotes variation in
mance over time, we performed a movement-related brain motor behaviour of young infants, particularly of infants
macropotentials (MRBMs) EEG analysis. with MND.
Results: Behavioural measures showed that children with
DCD were less attentive than controls. Furthermore, they
were more variable in synchronization and syncopation, and,
unlike controls, their performance deteriorated significantly 46 DONKERVOORT
with repetition. MRBM results indicated that the DCD group Transition into adulthood and level of
had the same morphology of ‘Bereitschaftspotential’than participation in adolescents and young adults
controls but with a longer NlOO latency. These findings sug- with cerebral palsy
gest that for DCD children, the complex subcortical circuits of M DONKERVOORTA, D J H G WIEGERINK”.”, ME ROEBROECKAvC,
feedback loop supporting the rederent activation of muscles H J STAM MDA
and tendon is notably delayed. aErasmusMC, UniversityMedical CentreRotterdam,
Conclusions: These findings indicate a general disorder in Rotterdam; bRijndamRehabhilitation Centre,Rotterdam,
synchronization for children with DCD. the Netherlands; Ci”hansition
Research Group,
Rehabilitation Centres in south-west Netherlands, the
Acknowledgements:This work is supportedby Contract no 0220019 Netherlands
from the French National Science Fundation (ACI Ecole et sciences
cognitives) and was sponsored by the University Hospital of
Toulouse for regulatory and ethic submission. Objective: The literature suggests that development of

18 EACDAbstracts2005
young adults with cerebral palsy (CP) may be delayed in rela- nencdconstipation, paralytic ileum) well treated with osmot-
tion to several aspects of life. It is also suggested that they ic laxatives, polyethylene glycol, and enemas; heartburn and
encounter specific problems related to an independent epigastric pain caused by gastroesophageal reflux improved
adult lifestyle. The relationship between phases of transition by protein pump inhibitors and prokinetic agents.
from childhood to adulthood and level of participation of (3) Endocrinological: hyperandrogenism and oligomen-
adolescents and young adults with CP was studied. orrhea in polycystic ovary syndrome responding to antian-
Methods: In a CP transition study in south-west Netherlands, drogenic and estrogen-progesterone therapy; amenorrhea
adolescentsand young adults (16 to 20 years of age) diagnosed caused by iatrogenic hyperprolactinemia (prokinetic agents)
with CP without severe learning disabilities (n= 105) partici- which disappeared after suspending the treatment;
pated. Patient characteristics, functioning in daily activities, oligomenorrhea due to functional hyperprolactinemia treat-
and social participation (LIFE-H) were measured. The Trans- ed with low-dose D2-agonists; loss of libido and erectile
ition Profile categorizes participants into three phases of transi- deficit, probably caused by primary hypogonadism and
tion regarding studyhvork, housing, intimate relationships, hyperprolactinemia improved by D2-agonists, and low-dose
and finance:dependent (child),increasing independence, and transcutaneous/oral androgenic agents; demand for physical
independent lifestyle (adult). Correlations were determined sexuality and psychological management.
using Pearson's r. This study was performed as part of Pediatric (4) Nephro-urological: incontinence self-management,
Rehabilitation Research in the Netherlands (PERRIN). autocatheterism difficulties in obese patients, surgical app-
Results: In different domains of participation, 12 to 25% of roach stones, rare renaVvesical complications in patients fol-
participants reached an independent lifestyle. These per- lowed u p adequately at pediatric age.
centages are slightly lower than reference values of peers ( 5 ) Neurological: shunt malfunction (rare and underesti-
without disabilities. Age was related to transition phases mated) worsening of lesional level in myelolipomas sympto-
(range r=0.30 to r=0.62).Furthermore, GMFCSAimb distrib- matic underestimated Chiari malformation; tethered cord
ution was related to transition phases in studyhork requiring a neurosurgical approach.
(r=-0.25/-0.22), and level of education was related to transi- (6) Physiatrical: mobility preserved in normally-walking
tion in finance (r=0.21). Overall, participants who reached an participants, mixed mobility with crutches and wheelchair in
independent lifestyle encountered fewer problems in partici- partially-walking participants, progression of kyphosidscol-
pation than those who were still in transition (p=O.OO to iosis in non-walking participants back pain, and radicular
0.09). Fifty per cent of participants did not visit a pediatrician lesions osteoporosis (only post-traumatic fractures).
or adult department of rehabilitation, and 10%visited adult Conclusions: Only a thorough understanding of the com-
rehabilitation services in the past year. The latter group com- plexities of this disease in adulthood will enable appropriate
prised relatively more women, older patients, and patients and adequate management.
with a lower level of education and GMFCS.
Conclusions: Transition into adulthood of adolescents and
young adults with CP was delayed. During the transition peri-
od a large group of patients do not utilize rehabilitation care 48 DRIGO
that may contribute to reaching successful independence. Prenatal counselling and spina bifida
P DRIGO, E TRIPOLI, M FRANZOI
Dipartimentodi Pediatria, Universitd di Padova, Italy

47 DRIGO The incidence of neural tube defects (NTD) has progressive-


Spina bifida in adulthood: needs and problems ly decreased in recent years due to the preventive adminis-
p DRIGO, M FRANZOI, E TRIPOLI, G Z E C C O L I N I ~R, MIONI", tration of folic acid and prenatal diagnosis at ultrasound
G STURNIOLOA, A MARUCCO screening in the 20th gestational week (with an accuracy of
DIpartimento d i Pediatria; aUniverstta,Azienda 70%),though recourse to voluntary termination of pregnan-
Ospedaltera Aadova, Italy cy varies considerably in different parts of Europe (from 20%
in Austria to 100%in Germany; from 33% in Sicily to 93%in
Three years ago a multidisciplinary outpatients clinic in Tuscany).
Padua was established for adult patients with dysraphia to How the diagnosis is reported to parents plays a funda-
help them progress from the dependence of childhood to mental part in the clinical relationship which takes on a ther-
the independence of adulthood. Different specialists aimed apeutic aspect. It can help to deal with problems and establish
to develop a new relationship unmediated by parents some- balanced understanding in parents, as a prenatal diagnosis
times involving a partner. comes at a time of great emotional investment and is usual-
Forty-two patients visited the clinic (120 visits in total): 24 ly unexpected.
with spina bifida, 16 with myelolipoma, and 2 with caudal It is essential that prenatal counselling occurs when the
regression (22 females, 20 males; range 16to 38y). 'kenty-five diagnosis is reported and has to provide: (1) information: the
patients were already being followed up at our pediatric multi- clinical picture must be explained clearly and simply; ( 2 ) sup-
disciplinaryclinic,while 17were being looked after by special- port for the parents from the whole team, coordinated by a
ists at other hospitals. The problems that emerged were as principal reference figure, and (3) containment: using an app-
follows. ropriate communication style and setting, and an empathetic
(1) Generic: overweight/obesity,precordialgia, respirato- attitude.
ry insufficiency, bedsoreshfected ulcers. The following points need to be considered for the prena-
(2) Gastroenterological: bowel motility problems (inconti- tal diagnosis of NTD: (1) when ultrasound has aroused an

19
initial diagnostic suspicion, a specialist ultrasonographer must ly higher than that of PX( and PW showed higher scores than
be called in immediately; (2) if the suspicion is confirmed, NW (Spine BMD z-score: W=-0.74+ 1.29, PW=-1.42+ 1.37,
parents must be told as succinctly and accurately as possible; NW=-2.2121.65; femur BMD z-score: W=-1.34k1.42,
(3) an appointment for the parents with an expert in dys- PW=-2.54 k 1.11, NW=-1.65 k 1.49;total femur BMD z-score:
raphia should be arranged as soon as possible; (4) parents W=-1.29+1.2, PW=-1.9f1.14, NW=-O.722 1.71). Patients
should be given psychological support when the time comes with injury below S1 showed BMD values significantlyhigher
to decide whether to continue or terminate the pregnancy. than thosefoundintheothertwogroups(p<0.05).
Communicating the diagnosis adequately will not reduce At femoral level, P W patients showed BMD lower than that
the sorrow associatedwith a diagnosis,but it may help parents found in W and N W patients. QUS parameters, as well as lab-
to cope with it. It helps if parents are supported in their deci- oratory variables, did not differ between the groups.
sion-makingprocess by ensuring that they are well informed Conclusions:Our findings show that walking ability does not
and free to decide, and by helping them to work through their have a significant influence on BMD in patients with
often painful emotions. Diagnostic communication is a 'med- myelomeningocele. BMD is lower in the denervated sites,
ical action' that needs to be studied and prepared. suggesting an integral role of the nervous system in skeletal
trophism independent of motility. The lower BMD explains,
at least in part, the high number of fractures at the lower
limbs in these patients.
49 DRIGO
Osteoporosis and myelomeningocele
P DRIGO, V CAMOZZI", G DE ECCHER, E PERTOSSI, B DISARb,
A MARUCCO, G LUISETTO~ 50 EINSPIELER
Department of Pediatrics and aDepartrnentof Surgical Is the early development of females with Rett
and GastroenterologicalSciences, Bone Metabolism Unit, disorder really normal?
Unfversityofpadua, Italy m"
A M K,
E R R ~ ,H F R PRECHTL"
ahtitUteof Pbysiology, CentreforPbysiological Medicine,
Motor deficit in the lower limbs of patients with myelo- Medical University of Grax,Austria; bDepartment of
meningocele prevents the attainment of peak bone mass and Psychological Medicine,Academic Centre Gartnavel, Royal
the risk of fracture is increased due to limitations of walking Hospital, Glasgow, UK
and loading. Although fractures are the main clinical prob-
lem, there is little information about bone status and mineral An apparently normal early development was one of the ini-
metabolism in these patients. tial criteria for classical Rett syndrome. However, several
Aims: (1) To measure bone mineral density (BMD) in patients investigators considered Rett syndrome to be a developmen-
with myelomeningocele. (2) To evaluate the presence of a tal disorder manifesting soon after birth. Videos of 22
relationship between BMD and other variables, such as daily patients with Rett syndrome were carefully assessed for
calcium intake, number of fractures, level of injury, and movement, posture, and behaviour during the first six
degree of motor deficit. (3) To evaluate the diagnostic signifi- months of life. All signs which deviated from the normal stan-
cance of laboratory parameters and of two different methods dard were recorded meticulously. Special attention was paid
to evaluate bone status: dual-energy X-ray absorptiometry to the face, hand, and body movements. A detailed analysis
(DXA) and quantitative ultrasound technique (QUS). clearly demonstrated an abnormal quality of general move-
Method: Sixty one patients (27 females, 34 males) with mean ments (loo%), tongue protrusion (62%),postural stiffness
age 16.39 years k6.54 years were studied. None of them had (58%), asymmetric eye opening and closing (56%),abnor-
disease or took medication that could interfere with bone mal finger movements (52%), hand stereotypes (42%),
metabolism. bursts of abnormal facial expressions (42%), bizarre smile
Level of injury was above L3 in 19 patients, between L P S l (32%), tremor (28%), and stereotyped body movements
in 25, and below S1 in 17. Patients were divided into three (15%). Our study is the first to apply specific standardized
groups according to level of motor deficit: completely unable measures of early spontaneous movements to infants with
to walk (NW n=15, age 18.7y+6.18y),partially walking (pw; Rett syndrome, proving that the disorder is manifest within
n=17, age 18.7yk6.15y), and able to walk CJV; n=29, the first months of life. Although not necessarily specific, the
age= 16yk6.83~).Anthropometric variables, daily calcium signs observed will be of value in alerting clinicians to the
intake, laboratory parameters of mineral and bone metabo- possibility of the diagnosis at an early stage, when interven-
lism, DXA and QUS parameters were assessed in each patient. tion is likely to be most effective.
BMD was measured using a Hologic QDR 4500C densitometer
(Waltham Mass, USA) and values were expressed as BMD z-
score at lumbar spine and proximal epiphysisof the left femur.
QUS parameters were measured at the proximal phalanges of
the finger,2 to 5 ofboth hands, with a DBM Sonic Profile device
(Igea Carpi, Italy). Results are expressed as amplitudedepen-
dent speed of sound (AD-SOS) as well as ultrasound bone pro-
file index (UBPI) derived from mathematical analysis of the
ultrasound-wave graphic trace.
Results: AU DXA values were lower than normal reference val-
ues at spine and femur.W patients showed spine 2-score slight-

20 EACDAbstracts2005
51 ELIASSON 52 ELLIOTT
Development and reliability of a system to A randomized controlled trial of the effects of
classify hand function in children with lycra arm splints on movement substructures
cerebral palsy: Manual Ability Classification during functional tasks in children with
System cerebral palsy
A C ELIASSON, B ROSBLAD, E BECKUNG, M ARNER, A M OHRWALL, C M ELLIOTPJ’, P H A M E R ~ , B C E L L I O T T ~J, A ALDER SON^
PETER ROSENBAUM aUniversityof WesternAustralia; bWomenand Children’s
Department of Womanand Child Health, Karolinska Health Service, WesternAustralia; =NotreDame University,
Institute, Stockholm, Sweden;Department of Community WesternAustralia, Australia
Medicine and Rehabilitation Pbysiotherapy, University of
Umed,Sweden;Queen Silvia Children’sHospital, Goteborg, Objective: The purpose of this study was to measure the
Sweden;Hand UnitDepartment of Orthopedics,Lund changes in movement substructures (in addition to movement
UniversityHospital, Sweden; CanChild Centrefor time) and upper limb motor function following the wearing of
ChildhoodDisability, McMaster University,Hamilton, ON, a lycra splint by children with cerebral palsy (CP). The study
Canada also explored the efficacy of lycra splints in CP sub-populations
of spastic and dystonichypertonia.
Objective: To develop a system to classify how children Methods: Sixteen children with CP (hypertonia) aged
with cerebral palsy (CP) use their hands when handling between 8 years 11 months and 14 years 1 month with a
objects in daily activities, i.e. activities that are relevant and mean age of 11 years 6 months (SD 2y 3 mo) were recruited.
age appropriate. The classification is designed to reflect the A randomized cross-over research design was employed with
child’s typical manual performance, not the child’s maximal Group 1 wearing the lycra splint for three months. Then
capacity. It is the collaborative use of both hands together Group 2 wore the lycra splint for the same period of time.
that is classified. The Manual Ability Classification System Measures were made at baseline initial lycra splint applica-
(MACS) is not intended to explain the underlying reasons tion, 3 months after lycra splint wear, on immediate lycra
for limitations of performance or to classify types of CI! splint removal, and 3 months post lycra splint wear.
When defining the five levels of the MACS, our primary cri- Three-dimensional upper limb and trunk kinematic data
terion was that the distinctions in manual ability should be were recorded using a seven camera Vicon (Oxford Metrics
clinically meaningful. MACS is analogous to the Gross Ltd, Oxford, UK) motion analysis system. Movement sub-
Motor Function Classification System (GMFCS) and covers structures during tasks taken from the Melbourne Assessment
the same ages. were analyzed from calculations of the 3D movement of the
Design: An expert group of researchers with clinical experi- wrist joint centre. A full Melbourne Assessment of Unilateral
ence in CP created the classification.Aspects of validity and Upper Limb Function’ was also completed across all treat-
reliability were evaluated by an expert group of parents and ment conditions.
professionals. Results and conclusion: A significant difference was estab-
Method:The validation was based on the experience within an lished between baseline and 3 months after lycra splint wear
expert group representing different professionals, review of for the movement substructures: movement time, percent-
the literature, and thorough analysis of 15 children (8-12 age of time, and distance in primary movement, jerk index,
years) across a spectrum of function. Discussions continued normalized jerk, and percentage of jerk in primary and sec-
until consensus occurred, first about the constructs and then ondary movements. These substructures moved closer to
at each level until these were clear and easily understood. The the motor behaviour of children without CP at 3 months after
next step in the validation involved 25 parents and 25 thera- lycra splint wear. N o significant difference was established
pists who were interviewed about the content and the descrip- for directness, index peak, velocity as a percentage of dis-
tion of levels. Thereafter, reliability was tested between 25 tance in the primary movement, normalized jerk in the sec-
parents and 25 therapists, and between pairs of therapists for ondary movement, and unilateral upper limb function
145 children between 4 and 18 years. Data collection is in across all treatment conditions. The strength of the differ-
progress. ence in normalized jerk and the percentage of jerk in the pri-
Result: The MACS is a valid measure reflecting ‘the children’s mary movement from baseline to 3 months after lycra splint
manual ability when handling objects in daily activities’. The wear was greatest in the sub-populationof children with dys-
reliability between parents and therapists was 0.96, calculat- tonic hypertonia.
ed by intra-class correlation coefficients (ICC; 95% confi-
dence interval 0.89-0.98) indicating excellent agreement. Reference
1. Randall MJ, Johnson LM, Reddihough DS. (1999) TbeMelbourne
The reliability between therapists was 0.96 (0.96-098), with
Assessment of Unilateral UpperLimb Function: test
similar results for all ages. administration manual. Melbourne: Royal Children’sHospital.
Conclusion:The MACS is based on self-initiatedmanual abili-
ty with a particular emphasis on handling objects in daily
activities. It is a valid and reliable classification for children
with CP between 4-18 years of age. Clinicians and parents
seem to agree about meaningful differences between levels,
resulting in excellent interrater reliability

21
53 FALLANG tion problems (p=0.02), fine motor (p=O.OOl),and execu-
Quality of early motor behaviour and tive dysfunctions (p=0.013) was significantly higher than
neuromotor development at school age in that for controls. Patients had more school problems than
preterm infants controls (p=0.028),and the PKU group required more glob-
B I 0 R G FALLANG", MIJNA HADDERS-ALGRAB al tutoring.
"OsloUniversity CollegeHealth Sciences, Norway; Conclusions:Patients with PKLJ in our series presented with
bUniversityHospital Groningen,Department of Neurology, more school problems than controls. This may be related to
the Netherlands the disturbed cognitive function observed. We would like to
highlight the importance of school follow-up because,
A substantial proportion of 'apparently normal' preterm despite having normal intelligence, 50%of patients with PKLJ
infants exhibit minor and moderate dysfunctionsin neuromo- presented with school problems and required more atten-
tor outcome at a later age. Birth characteristics, minor abnor- tion and special tutoring.
malities on neonatal ultrasound of the brain, and motor
milestones have limited value in the early detection of these Acknowledgement: Thanks are due to the financial support of
children. REDEMETH grant number N-2003RED G054B-0and Inergen C03D5.
The objectives of the present study were to investigate
whether parameters of non-optimal reaching at early age are
associated with dysfunctional neuromotor and behavioural
development at school age. 55 CIEERTS
Methods: Characteristicsof reaching kinematics were studied (Dis)cognitive epilepsy with 'continuous
in preterm children and full-term children at 4 and 6 months' spike-wave during slow wave sleep'
corrected age. The kinematics of the displacement and maxi- characteristics: prevalence and treatment
mum velocity of the reaching hand were analyzed. At the age Y GEERTS, W VAN EMDE BOAS
of 6 years, children were reassessed by means of the Touwen Dutch Epilepsy ClinicsFoundation - SEIN - Zwolle, the
neurological assessment and the Movement ABC. Netherlands
Results demonstrated that in preterm infants without
cerebral palsy, a lack of successful reaching at 4 months and Aim: To determine the prevalence of cognitive epilepsy syn-
non-optimal reaching at 6 months is related to the develop- drome with 'continuous spike wave during slow wave sleep'
ment of a complex form of minor neurological dysfunction (CSWS) characteristics in children with epilepsy and learn-
) fine manipulative disability at 6 years.
( p ~ 0 . 0 1and ing problems. The study also aimed to determine whether
Conclusion: These early signs of reaching dysfunction indi- there is a relationship between EEG characteristics and clini-
cate the presence of clinically significant brain dysfunction. cal features and response to treatment.
Methods: We retrospectively assessed all children referred
with learning difficulties to our centre between October
1999 and February 2005. All of them had at least one long
54 CIASSIO term EEGMdeo recording including a full night of natural
Cognitive function and school performance of sleep and at least one psychological examination.
children with pheny1ketonuria receiving early Resufts: Ninety-six children with epilepsy were primarily
and continuous treatment referred for learning problems. Epilepsy varied in severity
R G A S S I ~ " , M A VILASECA~,c BOW', R C O L O M E ~ ,A LOPEZ-SALA", and type. Wenty-one children had CSWS or CSWS-like EEG
E F U S T E ~ ,J CAM PISTOL^,^ characteristics.At the time of referral, a definite decline was
aNeuropediatricsDepartment; bPKUFollow-upUnit, suspected or documented in 15 children.
HospitalSantJoan de Deu University of Barcelona, Spain Ten children had a right lateralization,seven had a left pre-
dominant, and four had bilateral lateralization; predominant
Objective: The aim was to study the relationship between localisation was centre-parietal in eight, frontal in six, and
school performance and cognitive function in a group of 26 temporal in seven patients. N o specific relation could be
early and continuously-treatedpatient, with phenylketonuria found between clinical and EEG characteristics.
(PKU) by comparing the results with 21 healthy sex- and age- Ethosuximide was often added and carbamazepine was
matched controls. usually discontinued. Five children received steroids.
Methods: Age of patients was 12.323.7 years, and age of con- Change of treatment was followed by improvement in EEG-
trols was 11.823.6 years. The cognitive functions study abnormalities in 13 patients, including total disappearance
included intelligence measurement, visual and auditory of CSWS in five children. EEG improvement was not always
memory, auditory verbal learning abilities, attention, visu- correlated with clinical improvement.
ospatial, fine motor language, and executive functions. Conclusions: (Dis)cognitive epilepsy associated with CSWS
Participants were asked to answer a questionnaire about is a relatively rare, but severe, epileptic syndrome. N o effec-
school performance (type of school, academic degree tive treatment has been found until now. More extensive
achievement, need for special tutoring and/or repeating multicentre studies have to be conducted with large num-
classes, and type of special tutoring). bers of patients to determine specific clinical or EEG charac-
Results: The intelligence score was significantly lower in teristics, and to develop effective treatment strategies.
patients with PKU (p<O.OOOl). Although the means of all
tests were within the normal range, performance was worst
in patients with PKU. The percentage of patients with atten-

22 EACDAbstractsZOO5
56 GIACCHERO team, and the resources available within the centre.
Biochemical profile of paediatric patients Results: Questionnaireswere distributed to approximately 36
affected by neurological or neuro-motor sets of parents each year and on average 19 (53%)responded
disabilities (134 responses in total over the 7 years). Receiving this annual
R GUCCHERO, L BERNARDO, P GRANATA, F GHELMA, feedback was stressful for some team members but parental
M GIOVANNINI opinions were generally very favourable, especially with
Department of Pediatrics, San Paolo Hospital, University of respect to the keyworker role of special needs social workers.
Milan, Italy Parental feedback resulted in the restructuring of speech and
language therapy and clinical psychology support, revision of
Objective: To describe the biochemical plasma nutritional pro- the way in which information is given to parents about their
files of paediatric patients affected by neurological or neuro- child’s progress, and has provided useful evidence of need
motor disabilities. when successfullyapplyingfor funding to improve the physical
Methods: Ninety seven participants (55 males 42 females; environment of the centre. Relevant aspects of parental feed-
mean [SD] age 7y 7mo [4y 5mo], range 1-17y) with neuro- back have contributed towards the annual appraisals of team
logical or neuromotor disabilities who were consecutively members.
admitted from January 2002 to March 2005 at our depart- Conclusions: Formal annual parental feedback has been
ment were assessed at hospital admission for plasma bio- extremely useful in moddying the work ofcertain team mem-
chemical profile. Fasting blood samples were analyzed using bers and in highlighting ways to improve communication
Modulm. Disabilities were grouped as: (1) autism (n= 14); between the team and parents throughout the year. Some of
(2) cognitive-motor delay (n=43); (3) genetic syndromes the written parental feedback has been helpful as supportive
(n=11); (4) metabolic diseases (n=7); and (5) tetraparesis evidence when bidding for additional resources.
(n=22).Statistical analysiswasperformed using ANOVA or the
Kruskal-Wallis test. Significance of multiple comparisons was
adjusted with Bonferroni’scorrection.
Results: Plasma levels of glycaemia (mean [SD] 85.0 [12.5] 58 GONZALEZ
mgdl); haemoglobin (Hb) (12.6 [l.6] gdl); azotemia (28.0 Psychometric proflle of children treated for
[9.7] mgdl); albumin (4.3 [0.3] g/dl); total protein (7.1 [1.0] tumours of the cerebellum: diagnostic
g/dl); sodium (140.8 [3.6] mEq/l); potassium (4.5 [0.6]mEqA); hypotheses about neuropsychological
chlorine (105.1 [11.0] mEq/l); calcium (9.5 [0.9] mgdl); alka- impairment
line phosphatase (540.7 [ 196.71 UA); total cholesterol (162.7 P A G N I E R ~ ,G LYARD~,c MASSOUBRE~,
s G O N Z A LNEKZH~A,N N ~ A,
[34.1] mgdl); LDLcholesterol (88.6 [33.6] mgdl); HDL-cho- A GROSS EL IN^, D FRAPPAZ~,D PLANTAP, c BERGER~
lesterol (54.7 [12.1] mgdl); and triglycerides (96.0 [50.7] aSMAEC Villeurbanne; bCHLJGrenoble, Department of
mgdl) were within the n o d range. Difference among groups Paediatric Oncology; THLJ Saint-Etienne, Department of
were found for Hb (overallp=O.O23;group 3 [mean 13.6, SD Psychiatry; dCentreLeon Berard, Lyon; eCHUSaint-Etienne,
1.21vs group 5 [mean 11.9, SD 2.4]p=O.O41);azotemia (over- Department of Paediatric Oncology, France
all p=0.002; group 4 [mean 15.3, SD 5.7) vs other groups
O.OOl<p<0.05); HDL-cholesterol (overall p=0.018; group 4 Over the past 20 years, the intellectual impairment of chil-
[mean 43.8, SD 7.11vs group 1 [mean61.1, SD 9.5],p=O.O39); dren treated for tumours of the cerebellum has been assessed
and albumin (overallp=O.O32;group 1 [mean 4.5, SD 0.21 vs with psychometric evaluation using Wechsler scales. A disso-
group5 [mean4.1, SDO.4],p=O.O21). ciation between verbal IQ (WQ) and performance IQ (PIQ),
Conclusions: In this population of paediatric patients, bio- with VIQ greater than PIQ, is usually reported. However, this
chemical profiles were in the normal range but differences result is insufficient to explain the specific cognitive deficits of
occurred among categories of neurological or neuromotor this population and, consequently, to propose individualized
disabilities. rehabilitation programmes and social adaptation.
Objective: The purpose of this study was to explore whether
the population of children treated for tumours of the cere-
bellum shows heterogeneous psychometric scores in favour
57 GIBBS of neuropsychological impairment.
The benefit of regular parental feedback for Material and method: Fifty children treated for cerebellar
the work of a child development team tumours, medulloblastomas (n=33), ependymomas (n=9),
J CIBBS, HJOSHI, J ROBINSON, J MOULDS, VAL EEANE and astrocytomas (n=8)were assessed with Wechsler scales:
Countess of ChesterHospital Child Development Centre, WPPSI-R for children aged under 7 years and WISC-111for chil-
Cheshire, LJK dren aged over 7 years. Inclusion criteria were: individuals
aged between 4 and 15years, treated minimally by surgery, no
Objective: To review the benefits of annual structured feed- longer undergoing therapy for at least 6 months, without
back from parents on the work of a multidisciplinary child severe behavioural perturbations, and followed in one of the
development team. followingcentres: Department of Paediatric Oncology of CHU
Methods: An annual questionnaire was distributed over 7 of Grenoble;Centre LEon Berard, Lyon; and the Department of
years to parents of preschool children with severe develop- Paediatric Oncology, CHU Saint-Etienne.Psychometricevalua-
mental delay who were receiving support from a multidisci- tion included a measure of Full-scale IQ (FSIQ, MQ, and PIQ,
plinary child development team. Views were invited on the and each verbal and performance subtest.
work of individual team members, the work of the whole Results: For the three types of tumours, FSIQ, WQ, and PIQ

23
were within normal limits but there was great variation, 60 GRAHAM
and PIQ was always inferior to VIQ. Scores of verbal and Key working and the role of the the key
performance subtests revealed competences in verbal con- worker
ceptualization (similarities), lexico-semanticlinguisticknod- lCGWihM
edge (vocabulary), visual analysis from mental imagery University of Brighton, North Hampshire Hospital, UK
(picture completion), deficits in speed of treatment and
working memory (arithmetic digit span and coding), sus- Since the mid-l970s, the concept of ‘key working’ with
tained attention and visual associative memory (coding), regard to the provision of support to families of vulnerable
and logical and chronological organization of ideas (arith- children or children with a disability has been well docu-
metic and picture arrangement). mented. Yet almost 30 years later, in the recently published
Conclusion: Psychometric evaluation represents an obliga- National Service Framework for Children, Young People, and
tory step before neuropsychological evaluation. This step Maternity Services, it is reported that there are still fewer
allows us to assess diagnostic hypothesises about neuropsy- than one-third of the families with a child with disabilities
chological profile in order to adapt therapeutic means. who have a ‘keyworker’.
There is no universal term for the concept of ‘key work-
ing’, with professionals from different backgrounds and
agencies referring to a similar role by a different name.
59 GOUGH Differing models of ‘keyworking’ in practice may be depen-
Surgery for early progressive deformity in dent on and influenced by the context in which they operate,
children with spastic diplegia: does it work? and on the status and professional background of the person
M COUGH,, A E MCNEE, L C EVE, N R FRY, A P SHORTLAND involved in the role.
One Small Step Gait Laboratory, Guy’sHospital, London, UK The overall impression emerging from the literature is that
‘keyworking’ has to be employed within a multi-agencyteam
Objective: The outcome of multilevel surgical intervention, when working with children with disabilities and their fami-
guided by gait analysis, for early progressive lower-limb lies. The diversity of the role reflects the unique nature of the
deformity in diplegia is unclear. This study describes the out- children whose needs cross all disciplines and cannot be neat-
come in 10 children with spastic diplegic cerebral palsy who ly defined within health, education, or social care agencies.
had multi-levelsurgical intervention at a mean age of 7 years The policy and legislative framework exists for the intro-
5 months with a mean follow-upof 3years 6 months (SD 0.9). duction of the ‘keyworker’ system to health, education, and
Methds: Ten children with spastic diplegia participated (5 social care practice. Therefore, it is difficult to discern why
independent ambulators 5 assisted ambulators; mean there are less than one-third of families receiving this sup-
GMFCS level 2.5). The children had multilevel k e d lower- port mechanism.
limb deformities which had progressed, despite nonopera- This literature review will form the basis of a future study
tive management (including botulinum toxin, serial casting, that will investigate multi-professional team (including the
and orthoses) had a preoperative three-dimensional gait child and family) perceptions of the role of the key worker in
analysis at a mean age of 6 years 6 months(SD 0.9), followed the management of children with disabilities.
by multilevel surgical intervention at a mean age of 7 years 5
months (SD 0.77). Seven children had three post-operative
gait analyses, and three children had two postoperative gait
analyses at approximatelyyearly intervals. 61 GREITANE
Results:Comparing the final postoperative and preoperative Life satisfaction of primary caregivers of
gait analyses, the level of mobility was improved in seven adolescents with cerebral palsy
children, similar in one child, and reduced in the other two ,- ELlNA AUZINA
children. Children’sRehabilitation Center, ‘Mesesam liakas’, Riga
Popliteal angles and minimum hip extension in stance were Latvia
improved (p~O.001) at the first postoperative analysis, but this
improvement was lost at the second postoperative analysis. objective: Families with members with disabilities are viewed
Postoperative normalized velocity showed an improvement as having difficulties in family life. They often are socially
which was maintained (p~O.001)at the final analysis,as were isolated.
improvements in passive ankle dorsiflexion @<0.001),maxi- Adolescence is a transitional period of physical, emotion-
mum dorsiflexion in stance (p<0.002), and minimum knee al, and social changes. Many long-term difficulties of adoles-
flexion in stance (p=0.022). One child had repeat multilevel cents with cerebral palsy (CP) can be associated with family
surgery after his second postoperative gait analysis, and repeat relationships. Family members and caregiversoften have feel-
surgeryis planned for another child followinga third postoper- ings ofguilt, can be overprotective, and sometimes reject.
ative analysis. Our goals were: (1) to analyze life satisfaction and goals of
Conclusions: Although the long-term prognosis for mobility parents of adolescentswith CP; (2) to compare life satisfaction
in children with diplegia who develop early progressive of adolescentswith CP and different levels of functioning.
lower-limbdeformities that do not respond to nonoperative Methods: Participants included 30 parents of adolescents
management is likely to be poor, surgical intervention can with CP who attended suppodself-support groups for ado-
provide a significant medium-term benefit, and should be lescents with movement disorders. Instruments used includ-
considered to prolong mobility in this group. ed the Life Satisfaction Questionnaire for evaluation of indiv-
idual life satisfaction, the Demographic Questionnaire, and

24 EACD AbstractsZOOS
the GMFCS. 63 GUILLEM
Preliminary results: Family members of adolescents with CP Comorbidity associated with higher scores
have a variety of physical (including chronic physical condi- in the Autism Diagnosis Interview - Revised
tions) and emotional problems. Forty per cent of families ( ADI-R)
have financial problems due to lack of salaried work. Many P G U I L L E M ~c
, T R O N C ~ ,v G U I N C H A T ~ s c cmsA
, TARADE~,
were not satisfied with their leisure situation (60%)and con- aRegistre des handicaps d e l'enfant et Observatoire
tact with friends and acquaintances (40%). Family members Perinatal, Grenoble; CentreAlpin de Diagnostic Precoce
of adolescents with CP of FL 111-V (GMFCS) were more isolat- de I'Autisme,Grenoble,France
ed and had a heavier financial burden compared with other
parents. Objective: We attempted to describe specific behaviours, as
Conclusions:Most family members of adolescents with CP in characterized by Autism Diagnosis Interview - Revised (ADI-
support/self-support groups are not satisfied with their R), in children with autism with and without comorbidity. We
financial leisure situation and social isolation. analyzed data for the different ADI-R sub-domains (at the 4- to
Family-centred policies and services could benefit the 5-years age period) of reciprocal communication and social
wellbeing of both adolescents with CP and their families. deficit, and items of repetitive/stereotypicalbehaviours.
Method: For this study we used data from a morbidity regis-
ter: 420 children were born between 1985 and 1995 with a
diagnosis of pervasive developmental disorders (PDD) at 7
62 GROLEGER SRSEN years. Among those, 95 families agreed to participate in an
Concurrent validity of the Gross Motor ongoing collaborative study, and were assessed with the ADI-
Function Classification System and Paediatric R. ADI-R items were grouped as follows: normal or mild
Evaluation of Disability Inventory (score 0 or 1) and severe (score 2 or 3).
,- I VREAR Results: Overall, the prevalence rate of children with PDD
Institutefor Rehabilitation, Ljubljana, Slovenia was 26.5/10 000; CI 95% (24.0-29.2). Our sample included
80 children considered to be autistic according to the ADI-R.
Introduction: Functional disabilities and abilities determine Sex ratio was 4.3 (M/F) and average age at time of study was
a child's independence in activities of daily living. There are 12years; CI95%(11.4-12.6). Inthissample, 15%ofchildren
few instruments available for measuring of functional dis- had a morphological or karyotypic anomaly, 15%had epilep
abilities. One instrument is the Paediatric Evaluation of Disab- sy, 15% had been hospitalized in a neonatal care unit, 10%
ility Inventory (PEDI), which was used to evaluate functional were born before 37 weeks of gestation and 8.8%with a birth
abilities of children admitted to the Institute for Rehabilitation. weight under 2500g, 6%had cerebral palsy, and 16.3%had
For classification of children with cerebral palsy (CP), the severe learning disability (IQc50). The group of children
Gross Motor Function Classification System (GMFCS) was with autistic without comorbidity (n=40) had higher scores
used. The aim of the study was to determine the construct in compulsions/rituals items (25% of severe score vs 3.1%
validity of GMFCS and PEDI. p = O . O l ) . The group of children with autism and associated
Methods: The majority of children admitted to the Institute comorbidity had higher scores in language (35.2% severe vs
from1990 to 2004 were classified into GMFCS levels (from 12.5%,p=0.03), inappropriate questions (37.5% severe vs
data charts or at admission). All children with CP admitted 14.8,p=0.09), and in inappropriate facial expression items
after August 2003 were evaluated with the PEDI. Scores for (5 1.5%severe vs 29.0%,p=0.06).
all domains of the PEDI were analyzed. We compared scaled Distinctions between different behaviour profiles is
scores of functional skills and the caregiver assistance scales important for professionals managing children with autism,
between two groups with GMFCS levels 1-111 (with some and for a better understanding of pathogenesis.
independent vertical mobility) and GMFCS levels IV-V (gen-
erally full-timewheelchair users).
Results: Five hundred and seventeen children with CP were
admitted to the Institute during the study period; 384 chil- 64 GONAL
dren were classified into different GMFCS levels (from level I The relationship between hand performance,
to?: 21.6%, 15.9%,13.5%,19%,and29.9%;and87children daily living activities, and quality of life in
were assessed with the PEDI. When comparing both groups autism
of children, we found that those with GMFCS levels 1-111 GONCA B U M I N , AYLA GUNAL
were significantlybetter in mobility skills and less dependent Hacettepe University School of Physical Iberapy and
in self-care and mobility (p<O.Ol) compared with children Rehabilitation, Ankara, ntrkey
with levels IV-V
Conclusions: Results confirmed that the presence of func- Objective:Autism is a developmental disabilitythat is defined
tional disability according to the PEDI mobility domain is by behavioural characteristics. Most children with autism do
strongly connected to the GMFCS level. This is in agreement not establish relationships with people. Also, many of these
with results of other studies. Despite important differences children do not tend to engage in purposeful activity, which
in functional status among children defined in different lev- may negatively affect gross and fine motor skills, and hand
els of the GMFCS, there are fewer differences in social func- performance may deteriorate as a result. The purpose of this
tional domains, as GMFCS is not a marker for all aspects of study was to determine whether hand performance affects
functional ability or limitation. daily living activities and quality of life in children with autism.
Methods: The study was performed in one special education

25
primary school and rehabilitation centre in Ankara. 'Iiventy 66 GCUZZETTA
children were included in this study. The Paediatric Evaluation Early prediction of cognitive development in
of Disability Inventory (PEDI)and Wee Functional Independence preterm infants at neurological risk: the visual
Measure (WeeFIM) were administered to assess daily living information processing paradigm
activities. The Jebsen Taylor Hand Function Test was used to F TINELLI", s MAZZOTTI",A BANCALE", G FERRETTI", A GUZZETTA~.,
assess hand function and the Child Health Questionnaire was R BATTINI",A B O L D R I N I ~ ,G CIONI"
used to assess quality of life of children. aDepartment of Developmental Neuroscience, Stella Maris
Spearman correlation was used to analyze the relation- Scient@cInstitute, Pisa; bNeonatalIntenslue Care Unit,
ship between PEDI, WeeFIM, Jebsen Taylor Hand Function University of Pisa, Italy
Test, and the Child Health Questionnaire.
Results: There was a significant correlation between PEDI and Objective: Improvements in neuroimaging, electrophysiolo-
WeeFIM (r=0.857,pcO.01) and the PEDI and the Child gy, and clinical techniques have significantly enhanced the
Health Questionnaire (r=0.765,p<0.01).There was no cor- accuracy of early prediction of neuromotor development in
relation between hand performance scores and PEDI, preterm infants. Unfortunately, the same does not apply to
WeeFIM, and Child Health Questionnaire scores (p>0.05). the prediction of cognitive development, as most of the com-
Conclusions: The findings of this study indicated a relation- monly used early psychologicaltests have not shown a strong
ship between children with autism's dailyliving activitiesand correlation with outcome. New instruments for the assess-
quality of life. Activities of daily living should be evaluated ment of infant intelligence, based on information processing
and treated to increase quality of life. There was no correla- abilities (recognition, memory, habituation), have recently
tion between hand performance and activities of daily living shown good predictive power. Their use in clinical settings,
and quality of life. Further studies are needed to determine however, has been so far very limited.
whether hand performance affects children's activities of The aim of the study was to assess psychological develop
daily living and quality of life. ment during the first year of life using the Fagan test of infant
intelligence (FTII) in preterm infants (gestational age <33
weeks) with periventricular leukomalacia (PVL). We also
aimed to evaluate the possible effect of early development of
65 GCURURAJ basic visual functions on visual information processing.
Neuroimaging in cerebral palsy: correlation Methods: As part of a follow-up programme, 22 preterm
with neurological deflcits infants with PVL grade 1 or 2 were consecutively enrolled in
AK GURURAJ , L SZTRIHA,m NORK, D HAAS D the study. The examinations were performed at 69, 79, and
Departments of Raediatrics &Radiology, Faculty of 92 weeks postmenstrual age, and consisted of the FTII and a
Medicine, AIAin & TawamHospitals, U4E University,AI full battery ofvisual functions. Outcome was measured using
Ain, UnitedArab Emirates the Griffiths' scale at 1 and 2 years of age, and by a standard-
ized neurological examination.
The study objectives were to examine clinical and neurologi- Results and conclusions: The second FTII test (9 months)
cal abnormalities in children with cerebral palsy (CP) and to showed a good correlation with the Griffiths' scale at 2 years,
correlate the signs with radiological abnormalities detected in particular with total DQ and subscales of locomotor,
by CT and/orbrain MRI. social, and eye-hand coordination. Early and late FTII (6 and
In a prospective hospital-based study, 65 children with CP 12 months) did not correlate with outcome. Early transient
were examined neurologically and their perinatal history was oculomotor difficulties could account for the low-predictive
reviewed. CranialCT and/orMRI were studied. The association value of the first FTII test. These results indicate that visual
between the gestational ages, perinatal history, neurological information paradigms help in early prediction of cognitive
deficits, and radiological appearances were studied. Of the 24 outcome of infants with WL, in particular when performed
children born pretem and 41 term children, 23 had spastic at 9 months.
diplegia; 57% of these children had significant periventricular
leucomalacia (WL) which was more common among preterm
children. Of the 13 children with hemiplegia, 12 had unilateral
lesions on brain imaging. Spastic tetraplegia was associated 67 HADDERS-ALGRA
with extensive bilateral diffuse brain damage. Extrapyramidal Postural adjustments during reaching in
CP was far more common among term-born infants, and 80% children with cerebral palsy: dysfunction or
of these showed significant abnormalities in the basal ganglia adaptation?
region. Ataxic CP was uncommon, and there was no sigmficant M R - a J C VAN DER HEIDE
GRA,
correlation between neurological signs and abnormalities on Department of Neurolow, Developmental Neurology
brain imaging. UniversityHospital Groningen,Groningen, the
In conclusion, the radiological findings were closely relat- Netherlands
ed to the type of CP and the neurological deficits, except in
the ataxic type. We believe that CT and MRI are helpful in Objective: Children with cerebral palsy (CP) are often ham-
understanding the pathology and timing of the lesions in CI! pered by postural problems. In the control of posture, twofunc-
tional levels can be distinguished. The basic level deals with
the generation of direction-specific adjustments:dorsal muscles
are primarily activated when the body sways forward and ven-
tral muscles are activated when the body sways backward.

26 EACD Abstracts 2005


The second level is involved in adaptation of the direction- locate visual information quickly. Auditory distraction was
specific adjustments. The present study aimed at studying used to gradually increase task difficulty. Cognitive challeng-
differences between children with CP and typically develop- ing, aimed at encouraging more balanced and realistic
ing (TD) children in postural adjustments during reaching. thoughts, was used to modify perfectionist thinking which
Methods: Postural adjustments during reaching were assessed was reported to be a significant factor.
in 58 preterm children with CP and 29 TD children aged 2 to Results: Baseline and outcome measures included assess-
11 years using multiple surface EMGs and kinematic record- ment of processing speed, quality of life, anxiety, and depres-
ings. Functional performance was assessed with the Pediatric sion levels. Early individual case analysis indicated that
Evaluation of DisabilityInventory (PEDI). processing speed had improved over the course of training.
Results: In most children with CP the basic level of postural Conclusions: Enhancing speed of processing is an effective
controlwas intact. But the postural adjustments of the children and essential intervention for children who have survived
with CP differed in two ways from those of the TD children: childhood brain tumours.
(1) in contrast to the TD group, children with CP showed a
strong preference for a cranial-caudal recruitment order; (2) References
1.Murdoch BE, Boon DL, Hudson LJ. (1999) Effects of Deatment
children with CP had difficulties modulating EMG-amplitude
for Puediatric Cancer on Brain Structure and Function.
to task-specific conditions. The second problem and the Murdoch B (Ed) CommunicationDisorders in Childhood
need to stabilize head position during reaching may explain Cancer. London. Whurr Publishers.
why children with CP often exhibit a top-down recruitment. 2. Hogarty GE, Flesher S, Ulrich R, Carter M. (2004) Cognitive
Thus top-down recruitment might be regarded as a function- enhancement for schizophrenia: effects of a 2-year randomized
trial on cognition and behaviour. Arch Gen Psychiatry
al stratem. However, the finding that topdown recruitment 61:866876.
was related to severe brain lesions and to worse performance 3. Ball K,Berch DB, Helmers KF, Jobe JB, Leveck MD, Marsiske M,
in activities of daily life, as measured by the PEDI, suggests Morris JN, Rebok GX! Smith DM, Tennstedt SL, Unverzagt FK!
that top-down recruitment might also be regarded as an Willis SL. (2002) Effects of cognitive training interventions
expression of dysfunction. with older adultsJourn AmerMedAssoc 288.2271-2281.
4. Palmese CA, Raskin SA. (2000) The rehabilitation of attention in
Conclusions: Children with CP have (1) difficultiesin adapting individuals with mild traumatic brain injury using t h e m - I 1
the degree of muscle contraction to environmental con- programme. Brain Injury 14 535-548.
straints, and (2) a dominant top-down recruitment. The latter
problem might be an additional dysfunction or a functional
compensation.

69 HAWORTH
Relationship between the Behaviour Rating
68 HARTER Inventory of Executive Function and
Enhancing speed of processing in survivors of measures of cognition and behaviour in
childhood brain tumours children with traumatic brain injury
DIANNE GUMLEY, CATHERINE HARTER, CATHERINE WARD C M HAWORTH, H E MILLER, RJ MCCARTER, S BROERE,
Department of PsychologicalMedicine, Great Ormond A L CURRAN, PM SHARPLES
Street Hospital, London, UK Frenchay Hospital; Bristol Royal Hospitalfor Children,
Bristol, UK
Objectives:Treatment for cancer is associated with a range of
adverse long-term side effects. Long-term neuropsychological Background: Executive function deficits are a well-recog-
effects include below average IQ scores, poor concentration, nized complication of traumatic brain injury (TBI), but can
poor or reduced memory, visual-motor integration difficul- be difficult to detect in children. The Behaviour Rating
ties, and slow processing speed.’ Slow processing speed may Inventory of Executive Function (BRIEF) was designed to
be particularly debilitating due to the significant impact that assess executive function, but few data exist in TBI children.
it can have on children’s academic progress. Aims: To compare executive function in TBI children and
Few studies have implemented interventions aimed at controls using the BRIEF, and to relate BRIEF results to mea-
improving processing speed in children. However, those that sures of cognition and behaviour.
have investigated interventions for adults have demonstrated Methods: Prospectivecohort study. TBI was classified as severe
positive effects of processing speed cognitive training inter- (Glasgow Coma Score [GCS] 3-8), moderate (GCS 9-12), or
ventions in individuals with schizophrenia,2 older adults,3 mild (GCS 13-15). Controls were non-injured children. Ass-
and traumatic brain injury populations.* In view of this, our essments were performed 12-24 months after TBI. Intellect
objective was to implement a group cognitive training inter- was assessed by WISC-111; attention by Test of Everyday Atten-
vention aimed at improving specific difficulties with process- tion in Children (TEA-Ch); memory by Children’s Memory
ing speed in children with brain tumours. Scales (CMS); behaviour by Child Behaviour Checklist (CBCL);
Methods: Participants were selected from a pool of children and executive function by Tower of London (TOL) test and par-
who survived childhood brain tumours and were aged 12-16 ent-completed BRIEF.
years. Children whose score on the Processing Speed Index Results: Thirty three patients with TBI (15severe, 10moderate,
of the WISC-111 was significantly lower than their verbal IQ 8 mild; mean age 12y 1Omo) and 17 controls (mean age 12y
(15 points orgreater) were invited to attend. In line with pre- 2mo) were enrolled. Children with severe TBI and moderate
vious interventions, speed of processing training focused on TBI scored significantly higher than controls on the Behaviour
enhancing visual search skills and the ability to identify and Regulation Index (BRI; p=O.OOl, p=0.039), Metacognition

27
Index (MI;p=0.021,p=0.014), and the Global Executive Comp 71 HENSEY
osite (GEC;p=O.007,p=O.008) ofthe BRIEF; childrenwith mild School-age children and adolescents with
TBI did not differ significantlyh m controls. There was a sigtllf- neural tube defects: are we meeting their
cant correlation between BRI and VIQ (p=0.002), PIQ needs?
(p=0.003),CMS (p=0.037), TEA-Ch (p=0.039), and CBCL Ext- 0 HENSEY, S DOCKERAY, M MCGINN, M MCGINN
ernalising Index (p<O.OOOl),Internalizing Index (P<O.OOOl), 7%eCentralRemedial Clinic,Dublin, Ireland
and Total Problem Score (p<0.0001); between MI and PIQ
(p=O.O07), CMS (p=0.023), and CBCL Externalizing Index Background: The Central Remedial Clinic (CRC) is a nation-
( p = O . W ) , Internalizing Index @=0.036), and Total Problem al organization providing a range of specialized services for
Score (p=0.002); and between GEC and VIQ (p=O.MO), PIQ children and adults with physical disability. One such service
(p=O.oob), and CBCLEktemalizing Index (p<O.OOOl),Intern- provided is specializes in spina bifida.
arizing Index (p<O.OOOl), and CBCL Total Problem Score Aims: (1) To assess the current level of need among school-
(p<0.0001). There was no correlation between TOL and BRI, age children and adolescents (aged 5-18 years) with neural
MI, or GEC (p>0.05). tube defects. (2) To obtain a measure of patient and parental
Conclusion: Children with severe and moderate TBI have satisfaction with the specialist spina bifida service provided
evidence of executive dysfunction measured by the BRIEF. by the CRC. (3) To implement improvements in the service.
BRIEF scores correlate with measures of cognitionhehav- Methods: The parents of 42 children were invited to partici-
iour, but TOL test did not. pate in the study. All lived in the Eastern Regional Health
Authority Area and attended the specialist spina bifida ser-
vice at the CRC. All but one agreed to participate. Each partic-
70 HEINEMAN ipated in a semi-structured interview which included the
Assessing neuromotor condition in infancy: a completion of a questionnaire.
review of methods currently available Results: Of the 41 children, 27 were attending special schools
J<R H E I N E W , M HADDERS-ALGRA MD and 14 were in a mainstream setting; 29.3%were incontinent
Department ofNeurology,Developmental Neurology, in relation to bowel function and 56.1%were incontinent in
UniversityMedical Centre Groningen,Groningen,the relation to bladder function. The majority required assistance
Netherlands with toileting: 73.2%were dependent in relation to bowel
function and 51.2%in relation to bladder function. A signifi-
Objective: The present paper reviews methods described in cant number were dependent in relation to personal care (61%
the literature to assess neuromotor condition of infants. Early for washing, 58.5%for dressing).While there was a high satis-
detection of children who have a high risk of developmental faction rating for the service provided by the CRC (92.7%gen-
disorders enables clinicians to provide early intervention at an erally happy), 30 families (73.2%) identified the need for
age during which the bmin has highly plasticity. Furthermore, additional services, particularly home adaptation and coun-
these assessments can be used to evaluate the effect of early selling for both the child and parents. Among the improve-
intervention. ments suggested by parents were increased information, more
Methods: Neuromotor assessments for infants based on a liter- therapy, and improved communication within the multidisci-
ature search in PubMed and Medline were reviewed. plinary team.
Assessments were included if they were applicable to infants Conclusions:We have highlighted a high level of need within
aged 0 to 18 months and ifthey had been mentioned in at least this population. While parents may be happy with the cur-
two articles. menty assessmentsfulfilled the selection criteria. rent service provided, there is much room for improvement.
Results:We distinguishedfour categoriesof assessment.The first The key demand is improved coordination of services, not
consists of traditional neurological examination,which focuses only among the services provided by the CRC, but also
on posture, muscle tone, reflexes, and reactions. These tech- among all caregivers providing services at multiple sites in
niques are widely used, though little is known about their relia- the Dublin area.
bility The validity to predict major developmental disorders,
such as cerebral palsy, is good and the predictive validity for
minor disorders,such as Developmental coordination Disorder,
is moderate. The second category focuses on achievement, i.e. 72 HOGAN
motor milestones.These assessmentstend to have a good relia- Neurocognitive function in children with
bility and a moderate to good predictive validity for major obstructive sleep apnoea syndrome due to
developmental disorders. Little is known about their predic- adenotonsillar hypertrophy
tive validity for minor disorders, but prediction tends to be A M HOGAN", N N O N U G H A ~ ,D HARRISON", FJ KIRKHAM F J ~ ~ ,
poor to moderate. The third category consists of assessments A DATTA~,v MCGRIGGOR~,J STEVENSON", J ST EVENS^, C M HILL^
which combine parts of neurological exams with observation aDevelopmentalBrain-BehaviourUnit; bSouthampton
of motor milestones.The fourth category focuses on the quali- University Hospital Dust, University of Southampton;
ty of motor behaviour. An example is the evaluation of general %rtsmouth University;dInstltute of Child Health,
movements in young infants, which has a good reliability and University College,London, UK
good predictive validity for major and minor motor disorders.
Conclusions: Qualitative evaluation of motor behaviour has Objective: It is clear from epidemiological studies that there
the best predictive validity for major and minor motor disor- are increased rates of behavioural, cognitive, and education-
ders and good reliability. However, currently no qualitative al disturbance among children with sleep disturbance in
method for infants older than 4 months is available. general. However, these problems are particularly common

28 EACDAbstracts2005
in the context of upper airways obstruction due to adenoton- children with CHD and healthy schoolchildren respectively.
sillar hypertrophy. In the worst cases this may result in There was a significant difference in total Movement ABC
obstructive sleep apnoea syndrome (OSAS), characterized score: 8.9k6.7 and 4.023.9 for CHD and control children
by prolonged partial upper airways obstruction (hypopnea) respectively. The highest discrepancy between the two
and/or intermittent complete obstruction (apnoea), that dis- groups was found in manual dexterity: 4.523.9 and 2.122.5.
rupts both gas exchange (hypercarbia and hypoxia) and nor- Conclusion:Children with CHD show significantly impaired
mal sleep (arousals). The causal pathway for neurocognitive motor skills compared with healthy school children, espe-
deficits is unclear. Beebe and Gozal proposed that frontal cially in manual dexterity.
lobe function may be adversely affected by hypoxia and sleep
arousals characteristic of OSAS. Animal models have also
suggested that hypoxia leads to long-term changes in frontal 74 JACKSON
dopaminergic pathways. However,without evidence of func- Developing a computerized recall system for
tional brain abnormality,the implications for a similar patho- thyroid function monitoring in children with
physiology in children with OSAS remain implied but not Down syndrome
confirmed. P D IACKSON, K KEENE, Z M DUNHILL
Method: To address this limitation of the literature we used a Child Health Department, Royal Hospital For Sick
combination of neuropsychological, non-invasive, transcra- Children, Edinburgh, UK
nial, Doppler, and event-related potential techniques. Here
we present preliminary analyses obtained from 17 children Objective: To ensure that all children with Down syndrome
(3-7 years) with OSAS (apnoea-hypopnoea index > 1) con- (DS) living in the Lothian area were screened on an annual
firmed by attended polysomonography, and 14 healthy con- basis, as recommended in the evidence-based guidelines
trols of similar age. published by the Down's Syndrome Medical Interest Group
Results: These data revealed significantly increased levels of for the UK and Republic of Ireland (DSMIG) in 1995.A previ-
abnormal behaviourassessedby the FkhaviourRating Inventory ous audit had shown that in some areas as few as 50%of chil-
of Executive Function (BRIEF[P]) in children with OSAS dren with DS were known to have been tested. Our aim was
(pe0.05).However, mean IQ scores (WPPSI-111) were in the to set up a computerized recall system linked to our national
average range in both groups. Importantly, velocity of blood special needs system (SNS).
flow in the middle cerebral artery was significantlyincreased Method:An additional field was added to the SNS database to
in children with OSAS (p<O.OOl) and correlated positively ensure an annual recall of all children with DS. A letter of con-
with BRIEF(P) scores. sent for testing was completed by parents. Each year, the
Conclusion: The finding of significant group differences on school nurse completed a blood spot test on the child at
behavioural measures supports previous studies. We now school and the results were analyzed at our central laboratory.
extend the literature by providing novel evidence of abnor- Conclusions: Blood spot testing is accurate and acceptable
mal brain function. to children. Introduction of the computerized database for
recall for thyroid function in combination with school-based
Acknowledgement testing improved the effectivenessof screening.
This study was funded by HOPE medicalresearch charity

75 JAHNSEN
73 HOLM Health-related quality of life in adults with
Motor skills in children with complex cerebral palsy compared with the general
congenital heart disease: a case-control study population
MARIANNE LOENNECKEN LINDSTAD, U N N INGER MBINICHEN, R IAHNSEN, L VILLIEN, G AAMODT, J STANGHELLE, I HOLM
PER MORTEN FREDRIKSEN, INGER HOLM Sunnaas Rehabilitation Hospital, Oslo, Norway
Rikshospitalet University Hospital, Oslo, Norway
Objectives: The aims of the present study were to investigate
Objective: The purpose of the present study was to examine self-reported health problems and quality of life in adults
the prevalence of impaired motor skills in children with con- with cerebral palsy (CP) compared with the general popula-
genital heart disease (CHD) compared with healthy school- tion in Norway, and to identify variables potentially related to
children. health problems and low quality if life.
Metbods: Flfty two children (age range 7-13y) with CHD, all Methods: A multidimensional postal survey was conducted
operated on as neonates, were included and compared with including the SF 36, which contains 36 items on health-relat-
a group of 327 healthy schoolchildren with the same age and ed quality of life. Inclusion criteria were persons with diag-
sex distribution. The Movement ABC test was used to evalu- nosed CI: aged over18 years, and living in Norway. Exclusion
ate motor skills;it is designed to assess manual dexterity, ball criteria were intellectual disabilities documented in the med-
skills, and balance. The total score ranges from 0 to 40 with ical records of potential participants.
higher score indicating poorer performance. Total scores Results:In total, 406 persons (53%)responded: 49%females
between the 5th and 15th centiles suggest a degree of motor and 51% males aged from 18to 72 years with a mean age of
difficulty that is borderline; scores below the fifih percentile 34 years (SD l l y 5mo). Non-respondents coincided well
indicate a definite motor problem. with the respondents according to age, type of CI: and geo-
Results: Mean age was 1Ok1.5years and 9.9k1.6 years for graphical distribution. There were 38%of participants with

29
hemiplegia, 36%with diplegia, 17%with dyskinesia, and 8% 77 JOHANSSON
with quadriplegia. Pain assessment in children with severe
Self-reportedhealth-related quality of life (SF 36) in adults neurological impairments: a pilot study
with CP compared with a Norwegian reference group (Loge M IOHANSSOy, L JYLLI, E BROGREN CARLBERG
1998, n=2323) presented as mean sum scores (100 is the Handicap C Habilitation Stockholm;Department of
best health score). Surgey,Pain Deatment Service,KarolfnskaInstitute;
Conclusion:These results show that health problems are sig- Astrid Lindgren Children‘sHospital, Karolinska University
nificant in adults with CP compared with the general popula- Hospital, Stockholm;Neuropediatric Research Unit,
tion, with negative impact on physical and social function Department of Womanand Child Health, khrolinska
and on quality of life. Results indicate the need for lifespan Institute, Stockholm, Sweden
follow up of individuals with CE
Background: During the past decade, pain has been present-
ed as a common and repeated problem in children and ado-
76 JAKOBSSON lescents with severe neurological impairments. This has
Cooperation between different individuals brought about a demand for adequate pain management
involved in school situations of pupils with a strategies. A basis for optimal management is valid and reli-
medical diagnosis able assessment of pain. Few behavioural pain assessment
I-L IAKOBSSON tools have been developed and tested around the world, but a
Goteborg University,Department of Education, Sweden Swedish version is lacking.A modified version of the Canadian
Non-Communicating Children’s Pain Checklist (NCCPC)
Objective:When a pupil is given a medical diagnosis, the ped- was tested for reliability and validity in Stockholm,Sweden.
agogical consequences can be described and understood Objective: To investigate whether the modified Swedish ver-
from different theoretical perspectives. How does coopera- sion of the NCCPC discriminates pain from other emotional
tion between different individuals involved in the school sit- states in children with severe impairments. If so, the aim was to
uation affect the opportunities of participation and learning investigate how children’sbehaviour is altered. Furthermore,
of individual pupils? we investigated whether parent and professional assessments
Methods: This paper presents some results from a pedagogical are associated and whether they can be repeated in a stable way
study undertaken in collaboration with sociological, medical, within and between these two groups of assessors.
and psychological researchers. Theoretically,the study relates Methods: Parents of children with severe impairments were
to special educational and sociologicaltheories as well as psy- invited to participate in the study simultaneously with each
cho-medical theories. School situations of 50 pupils, repre- child’s local physiotherapist. Assessments were completed
senting four different aetiological syndrome-diagnoses, in situations of calm, stress, or pain within the child’s every-
were studied through ethnographic research strategies: mainly day life. Parents or primary caregivers and physiotherapists
observations and interviews. During the analyses there was a assessed the child’sbehaviour separately and repeatedly.
continuous dialogue with parents, school personnel, pupils, Results: Preliminary results indicate that the Swedish NCCPC
researchers, and professionals from relevant disciplines and can discriminate pain and that the assessment can be reliably
activities. repeated. These and other findings will be discussed in fur-
Results: According to the results of the study, communica- ther detail.
tion and collaboration between various individuals involved Conclusions: The Swedish NCCPC appears to be a good tool
in the school environment was crucial for pupils’ opportun- for identlfylng pain in children with severe impairments. As
ties of participation and learning at school. Parents play a this is a pilot-study, no general conclusions can be drawn.To
central role in communicating with professionals and took facilitate adequate assessment and treatment of pain in this
the role of coordinators and advocatesof their children. population additional, participants will be included in the
Discussion: Questions about cooperation between home, study.
school, other contexts, and institutions involved in the life of
pupils with disabilities are discussed. For example, in what
ways are relationships between home and school important?
What other contexts are related to school situations? What
are the characteristics of communication and relationships 78 JONGERIUS
between different contexts and what are the consequences Botulinum toxin A to treat excessive drooling
of good or poor working communication? in children with cerebral palsy
Conclusions:There is a demand for connection between dif- PETER IONGERIUS, KAREN VAN HULST, JAN ROTTEVEEL
ferent perspectives of research and practical experience to Radboud UniversityNijmegen, Nijmegen, the Nethedands
develop schools in becoming more inclusive.
Objectives:To evaluate the effectiveness of single-dose intra-
glandular injections with botulinum toxin A (BTX-A) in the
salivary glands to treat excessive drooling in children with
cerebral palsy (CP).
Methods and materials: A controlled clinical trail was per-
formed to compare the results of short-term administration
of scopolamine to BTX-A injections. Forty-five children with
CP and severe drooling entered a baseline period and were

30 EACDAbstracts2005
subsequently treated with scopoderm-TTS (SC) during 10 detect this. However,within this sample of parents of children
to 12 days. After a wash-out period and repeated measure- who received neonatal intensive care there was also a strong
ments, patients received one dose of BTX (Allergan) with a tendency to underrate their child’sdevelopmental status.
maximum up to 50 U using general anaesthesia and ultra-
sound to locate the submandibular glands. Salivary flow rate
(swab method) and frequency and severity of drooling
(Drooling Quotient [DQ]) were measured. Analysis of vari- 80 KAYA
ance was used to evaluate score differences. Statisticalsignif- The effects of play therapy on sensory
icance was set atp<0.05. integration
Results:Scopoderem and BTX-A had a substantial anticholin- EKlL KAYA, MINE UYANK
ergic effect with significant differences in salivary flow rate Hacettepe Unfversfty,Ankara, a r k e y
scores compared with baseline. The effect of BTX-A (42.4%
reduction in salivary flow) exceeded that of SC (24.7%). Objective: Play therapy is the use of the natural mileu of play
Measurements of the DQ and the outcome of questionnaires as a treatment environment for the development of chil-
were in accordance with this. The response rate to therapy dren’s language, sensory-perceptual-motor skills, and social
was good ( 4 9 , 6 4 % )for BTX-A. integration.
Presentation: The concept of injections of BTX-A into the Methods: To examine the effectiveness of play therapy on sen-
submandibular glands will be explained and the value of sory-perceptual-motorskills, 4 0 nrkish childrenwith learning
ultrasound guidance will be illustrated. Outcomes of salivary disabilitybetween 3 to 6 years of age were selected and separat-
flow and drooling measurements will be presented. ed into experimental and control groups. Both groups under-
Conclusion: Bilateral intra-glandular BTX injection into the went the following tests before and after treatment:
submandibular glands to treat drooling in children with CP is Figure-Ground Perception, Finger Identification Test, Posture
a safe method without adverse reactions related to BTX-A or Imitation Tests, and Ayres Southern California Sensory Integ-
the procedure. ration Subtests. During 12 weeks, play therapy was applied to
the treatment groups during 4 sessions per week Play therapy
together with the conventional occupational therapy were
applied to the control group during 4 sessions per week.
79 JONGMANS Results and conclusion: Results of the Figure-Ground
Developmental screening of young children Perception, Finger Identification Test, Posture Imitation Test,
born ‘at risk’: agreement between parents and and total score of the Ayres test showed statistical signifi-
professionals cance between the two groups (pcO.05). This differencewas
M I IONGMANS, M H FOX MA, K S D E ZWART MA a result of the positive effect of play therapy, which was
Department of SpecfalEducatbn,Unfversftyof Utrecht,the applied to the treatment group for visual perception, soma-
Netherlands to-sensorial perception, and motor performance of children.

Objectfoe:Several studies have indicated that parental informa-


tion on a child’sdevelopment within the first 2 years of life can
be reliable (e.g. in reporting on a child’s gross motor mile- 81 KEZTA
stones). Therefore,a parent’sjudgment ofwhether or not their Developmental and neuropsychological study
child is able to perform an activity might be ofvalue in detecting of visual attention deficits and linguistic
children at risk for current, or perhaps later occurring,develop impairments in children with dyslexia
mental problems. The aim of the present study was to examine L KEITA~,N BE DO IN^, v HERB ILL ON^, H LEVY-SEBBAG~,
concordance between parental estimatesof their child’s devel- A MER~GOT~
opment with the outcome on a standardized assessment of aLaboratofreEMC/DDL,Unfversitc3Lyon; bH6pitalLpn-
cognitive and motor abilities of young children. Sud, Lyon; H6pftalL a Ronche, Gretaoble, France
Methods: In this cross-sectional survey, parents of 188 chil-
dren (aged between 5 and 33 months) who were treated at a Objectfoe:Cognitivedisorders in developmentaldyslexiawere
neonatal intensive care unit due to either (extreme) prema- investigated using letter-based and object-based hierarchical
ture birth or medical complications following birth (e.g. full- stimuli.
term children with perinatal asphyxia) completed the Methods and results: In a divided attention paradigm
Denver Prescreening Developmental Questionnaire (PDQ- (Experiment l ) ,the target appeared either at the localor the
11) within three weeks of visiting a follow-up clinic. During global level in hierarchical stimuli that were randomly pre-
this time, children were assessed with the Grifiiths Scales. sented either in the right- or in the left visual hemifield. A left
Results:Sixteenchildren performed below 1 SD on the Griliiths hemisphere specializationfor local processing was observed
Scales. The parents of all these children had already noticed in first-grader regardless of stimulus category, whereas this
some sign of atypical development in their child (sens- specializationwas graduallyrestricted to letter-basedstimuli in
itivity=100%). However, of the 172 children with scores third-graders, fifth-graders, and young adults. Performances
within the normal range of the GriffithsScales,only 28 parents of 18 dyslexic children (nine with phonological dyslexia,
agreed with this observation (specificity= 16%). Sex, age, or nine with surface dyslexia) revealed atypical hemisphericspe-
any other variable had no influence on this outcome. cialization for letter-basedand object-based hierarchicalstimuli
Conclusions: Parents of children with an atypical develop- only in phonological dyslexic children.
mental trajectory within the first 2 years of life are able to In a focused attention paradigm (Experiment 2), participants

31
selected one level to discriminate targets in centred hierar- Local projects are needed to test these guidelines and sug-
chical stimuli. Children with surface dyslexia were not dis- gestions and to assist in arranging rehabilitation systems in
turbed by the classical global interference, but were strongly practice.
impaired by local interference for letter-based and object-
based stimuli. Therefore, these children may suffer from a
visual attentional deficit which leads their attention to be 83 KRABBE
captured by details to the detriment of global processing of ‘Can we talk together?’ Strategic language use
hierarchical stimuli (and printed words). among children who acquire alternative
Experiment 3 confirmed the specific disorders of phono- means of communication
logical versus surface dyslexia with a focused attention para- M K HAGEMOEN, K OPSAL, S KRABBE, K HELLBERG, A BOR0Y,
digm and divided-field presentation. The ability to avoid S VON TETZCHNER
interference from local information, regularly improved TheNational Hospital Department ofPediatrics, Child
from first- to third- and fifth-graders,but not in children with Neurology Section, University of Oslo, Oslo, Norway
surface dyslexia. However, the processing of letters gradually
improved at the local level in normal readers but not in chil- Objectives: Many studies show that children who are given
dren with phonological dyslexia who may have difficulties in communication aids rarely make use of the equipment in
developing specific processing of linguistic information. social interaction. The aim of the study was to obtain knowl-
Conclusion: The tasks used in these experiments are pro- edge about factors that are important for young communicators’
posed for clinical assessment of cognitive disorders related everyday use of their communication aids for everyday. The
to different forms of dyslexia. study also aimed to evaluate change in the children’s com-
munication strategies following an intervention programme
which focused on supporting communicative autonomy and
the development of strategicallyaided language use.
82 KOIVIKKO Methods: Six children were video recorded during various
Cjood practice in rehabilitation of children: communication tasks when interacting with a parent. Record-
a problem of planning? ings were taken before and after the intervention. Children’s
M I KOIVIKKQ, S M SIPARI and parents’ communication strategies at the two observa-
Department of Paediatric Neurology, Tampere University tion periods (more than one year apart) were compared.
Hospital; VajauliikkeistenKuntoty-Society,Finland Results: Examples are given of changes in strategic language
use following intervention. The aim of the intervention strat-
Objective: To adopt a multidisciplinary and innovative specialist egy was to encourage each aided communicator’s joint
approach with the purpose of determining good practice for engagement during shared activities with adults and chil-
rehabilitation of children. Ongoing service provision is dren. Children and parents changed both form and structure
emphasized. The main questions addressed include: What is in their communication strategies. The children developed
important?What is to be done first?How should proper con- more autonomic ways of communicating and parents adapt-
sideration be given to the role of families?How can profes- ed to their child’s developmental achievement.
sionals agree on a goal? Conclusions: The study resulted in a model for implementing
Participants: Fifteen local groups of rehabilitation special- augmentative and alternative communication in focused com-
ists across Finland and six national societies participated. munication situations. The model emphasizes the necessity of
The specialists represented health and social care, educa- involving and coordinating the total social network of people
tion, day care, and families of children with special needs. related personally or professionally to the child. The model
Metboh: From 2002 to 2005, group discussions (Delphi) illustrates the importance of continuity and long-termplanning
assisted in creating a consensus on ethical values and a in working with alternative communication.
framework for the past, present, and future. Results were
integrated according to the ICF model and priority was given
to everyday activities.
ResuZts and conclusions: (1)The starting point for rehabili-
tation is informed consent from the family. This means that
they receive thorough information of the rehabilitation plan
and agree with the plan. Commitment to the rehabilitation
plan is based on discussion, honesty, and trust. (2) To attain
this, a local hmework needs to be established, which means
that the local community must be prepared to arrange reha-
bilitation, special education, and support for the family. This
is not always the case, which can lead to delays, ineffective
services, and false promises. (3) The local framework is the
basis for individual planning, but does not replace it as not
all aspects can be forseen. On the other hand, individual
planning needs to comply with the framework. (4) The local
framework requires guidance with knowledge to combine
the information from external and local professionals. It also
serves as a basis for communal planning.

32 EACDAbstracts2005
84 KRIFI children born at 39-40 weeks were studied.
Hierarchy of phonetic features categories: Methods: Children were assessed at 5 years of age with the
studies in developmental dyslexia Kauhannhsessment Battery for Children.
-", N BEDOIN", v HERB ILL ON^ Results:Out ofthe 2357 survivingverypretermchildren,1624
Saboratoire EMCLyon 2 University;bNeuropaediatrics (68%) were assessed by psychologists as well as 326 (49%)
Unity,Debrousse Hospital, Lyon, France controls. There was a significant difference in the mean PMC
(IQ equivalent) scores (12.7; 95%CI 10.5-14.9) betweenvery
Objective: Skilled readers are sensitive to phonetic features preterm children (93.7, SD 19.3) and controls (106.5, SD
shared by consonants within one printed stimulus, and be- 17.8).After taking into account maternal age, parity, maternal
tween successive stimuli.l s 2 We investigated the relative weight level of education, social level of the family, and family com-
of voicing, manner, and place of articulation to guide respons- position this difference remained significant (10.9; 95% CI
es in a syllable-matchingtask. 8.7-13.0). The differences were 8.3 (95%C16.4-10.3) for the
Method: Participants presented with a printed target-sylla- sequential processing subscale, 12.3 (95%CI 10.0-14.6) for
ble, c\! had to select one of two proposed CV syllables the simultaneous processing subscale, and 9.3 (95%
according to intuitivelyestimated acoustic similarity.Manner C17.3-11.2) for the achievement scale. When using the test
and place similarity were examined in experiment 1, manner reference norms, 21% of very preterm infants had a PMC
and voicing in experiment 2, place and voicing in experi- between 84 and 70 (<-1SD to >-2SD) versus 8%of control
ment 3. children; 10%had a PMC between 69 and 55 (<-2 SD to >-3
Results:Adult skilled readers' responses were mainly guided SD) versus 3%of control children; 2%had a PMC less than 55
by manner similarity, especially for voiced consonants, s u g (<-3 SD) versus none for the controls. Among very preterm
gesting a modulator role of voicing on this effect. Place simi- children, a significant trend was found for lower scores of
larity also guided matches, particularly for first consonants. PMC with decreasing gestational age: with PMC scores from
We outlined the development of these rules in children with 96.8, SD 19.6 at 32 weeks to 84.1, SD 16.7 at 24 weeks.
normal reading ability (second, thud, and fourth graders). Conclusion:Among very preterm children, cognitive impair-
Children with dyslexia without phonological impairment did ment is common at preschool age and can not explained by a
not use phonetic rules for syllable matching: regardless of the less favourable environment.
phonetic features category, their choices never differed from
chance. Alternatively, phonetic features categories seemed to
guide responses in children with dyslexia and phonological
deficits. However, they followed phonetic rules that were 86 LARSSON
clearly distinct from skilled readers' rules. First, they pre- Muscle strength and endurance during
ferred manner of articulation only for stop consonants; sec- repeated knee extensions in children with
ond, the modulator role of voicing disappeared; third, their hemiplegic cerebral palsy
responses were more frequently guided by place of articula- B LARSSON", s KARLSSON~,B GERDLE MD"
tion than by voicing, but only for last consonants (contrary to aDepartment of Rehabilitation Medicine, Faculty of Health
skilled readers). Sciences,Linkoping;bDepartmentof Biomedical
Conclusion: Printed syllable matching tasks may improve Engineeringand Information, Umed University Hospital,
our understanding of phonological knowledge organization Sweden
in children with dyslexia.
Objective: To investigate muscle strength and endurance in
References children with hemiplegic cerebral palsy (CP).
1. Bedoin N. (2003) Sensitivity to voicing similarity in printed Methods: Rventy-four children with hemiplegia and 22 con-
stimuli:effect of a training programme in dyslexic children.
Journal of Phonetics51:541-546. trols, aged between 7 and 15 years, participated in the study.
2. Krifi S, Bedoin N, Merigot A. (2003) Effects ofvoicing similarity The relationships between maximum peak torque and surface
between consonants in printed stimuli in normal and dyslexic electromyography (EMG) variable mot mean square (RMS)
readers.Current Psychology Letters:Behaviour Brain and were obtained during 100 concentric knee extensions. Each
Cognftfon10: 1-7. http://cpl.revues.org/document93.html
child with hemiplegia performed bilateral isokinetic strength
testingwith the less-affectedleg tested first. The control group
tested one leg matched with the less-affectedleg. Surface EMG
was recorded from rectus femoris, vastus lateralis, and biceps
85 LARROQUE femoris.The children with CP were also tested using the Gross
Cognitive deficits of 5-year-old very preterm Motor Function Measure (GMFM). Principalcomponent analy-
children: the Epipage study sis (PCA)was used for multivariate analyses.
B WRROOUE, L MARCHAND, M KAMINSKI, THE EPIPAGE STUDY Results: GMFM-66 ranged from 73 to 100 mean 92 (SD 8).
GROUP Muscle strength and muscle endurance were significantly
INSERM 17149Killejuif;France lower in the affected leg in the group with CP N o significant
difference was found between the less-affectedleg in the CP
Objectfve:To investigate the prevalence of cognitive deficits at group and the tested leg in the control group. Generally,
preschool age in a population-based cohort of very preterm higher RMS values were obtained for agonist muscles of the
children. less-affected leg than in the affected leg in the CP group. The
Population: All children born at less than 33 weeks of gesta- children were divided into two groups: the older group
tion in nine regions of France in 1997 and a control group of (n= 14), 1 1 to 15 years, and the younger group (n= lo), 7 to 10

33
years. There was no difference between the groups in muscle long after the initial trauma. After seven years of follow-up,
endurance for the younger children.For the older children,the only one of 17children seemed to have normal development.
affected leg was weaker than the less-affected leg, both with Objectives: To observe the effects of acquired brain injury on
respect to maximum peak toque and muscle endurance. The learning abilities in children.
multivariate analyses revealed that peak torque correlated Method: The study evaluated 18-month and 5-yearoutcomes
with GMFM and RMS in rectus femoris muscles and vastus lat- of 28 children who were under 2 years old when consecu-
e& in the affected leg. Co-activation was found in biceps tively admitted to the department for subdural hematoma.
femoris but it did not affect muscle strength. Diagnosis of SBS was made in the absence of any other plau-
Conclusion: Children with CP showed less strength enhance- sible mechanism of injury.
ment in the hemiplegic leg during pubew. Results: Median age at admission was 4 months 22 days, and
the initial clinical picture was severe: coma in half the cases,
convulsionsin more than 80%,status epilepticus in 70%,hemi-
87 LARSSON plegia in 80%,and absence of eye contact in more than 40%.
The role of working memory and phonological At 18months, the main sign was a downturn in the cranial
awareness in non-speaking children with circumference curve, with some children showing complete
cerebral palsy who are learning to read cessation of brain growth. At 5 years about half the children
MARIA LARSSON, ANNIKA DAHLGREN SANDBERG had major sequelae and could not be educated, more than
Department of Psychology, Goteborg University, Goteborg, one-third had significant sequelae, and only one-quarter
Sweden appeared to have remained normal.
Discussion:The Kennard principle,*which maintains that chil-
Objective: To many non-speaking children with cerebral dren recover better from brain injury than adults, often turns
palsy, the ability to read and write often seems difficult to out to be false when applied to children with SBS. Diffuse
acquire, even when they are within the normal range of intel- brain injury or lesions located in strategic areas can impair
ligence. Studies of phonological awareness (PA), i.e. awareness learning ability This immediate impact is further exacerbated
of the sound structure in language which is seen as the best by the delayed effect of failure to learn, though it is not until the
predictor of literacy achievement, have revealed conflicting child falls behind in age-appropriate skills that the delay
results. In some studies, non-speaking children have per- becomes evident. This is particularly deleterious in younger
formed as well as their speaking peers, but still have many children who, by virtue of their age, have acquired fewer skills
problems in their reading and writing abilities. There could before their injury.
be some other factor apart from PA which makes the groups Conclusion: The prognosis of brain injury is reserved. From
differ in their literacy ability Therefore, we wanted to exam- a medico-legal standpoint, consolidation must not occur
ine the possible role of working memory literacy in this pop- before 18years.
ulation. Could it be that, due to lack of articulatory ability, the
non-speaking children have a faulty phonological working References
1. Bonnier C, Nassogne MC, Evrard I? (1995) Outcome and
memory?That is, do they lack the ability to make sound images
prognosis of the whiplash shaken infant syndrome (WSIS): late
in memory that are strong enough to execute the operations consequencesafter a sign-free interval.DevMed ChfldNeurol
of storing and manipulation necessary in reading? 57: 943-956.
Method: Sixteen non-speaking children with cerebral palsy 2. Finger S. (1988) Margaret Kennard and her ‘principle’in
aged between 5.2 and 12.8 years were matched to a group of historical perspective.Bruin Inuty: Tbeoretfculand
Controversfullssues.New York Plenum Press. p 117-132.
speaking children for cognitiveage. The two groups were pre-
viously tested on phonological awareness with no significant
difference in results. In this study, their test results of work-
ing memory were investigated in relation to phonological
awareness and reading. 89 LAURENT-VANNIER
Results and conclusions: Analysis of the data is currently A study of children with brain-injuries caused
ongoing and will be discussed in terms of the functions of by traffic accidents: cognitive sequelae
working memory and their possible effect o n reading F HANNEQUIN~,A LAURENT-VANNIER~, D G B R U G E L ~ ,
development. E PINEAU-CHARD ON^, s P I L L O Y ~P, H M E Y E R ~ ,M DE A G O S T I N I ~
aServfcede rkbducation despathologies neurologiques
acquises de l’enfant,HbpitalNational de Saint Maurice;
bINSERM U472 VfllejugcDepartementd’anesthesieet
88 LAURENT-VANNIER reanimation, H6pftalNecker EnfantsMalades, Paris, France
Five-year outcomes in 28 cases of shaken
baby syndrome (SBS): rethinking the Background: Traffic accidents are a major public health prob-
Kennard principle lem for children. They account for most of the traumatic brain
H TOURE, M CHEVIGNARD, D G BRUGEL, A LAURENT-VANNIER injuries (TBI) in children and adolescents in the Parisian
Service de reeducation des pathologies neurologiques area, as in other western countries. Severe TBI is often asso-
acquises de 1’enfant,Hbpital National de Saint Maurice, ciated with cognitive sequelae and learning difficulties. In
Saint Maurice cedex, France France only a few studies have been undertaken on this
topic. The aim of this study is to investigate the cognitive
Introduction: Bonnier et al. (1995)’ observed that some sequelae of TBI caused by traffic accidents in children, and to
sequelae of shaken baby syndrome (SBS) do not occur until observe changes over time.

34 EACDAbstructsZOO5
Method: The clinical sample consisted of 109children (71 males, especially the drawing of the sun and the stickers, but that
38 females) with TBI caused by traflic accidents. This samplerep- scores were affected by age, with a left displacement of the
resented 84% of all children with TBI admitted to the St. Maurice tasks; as the children grew up this effect was more effective in
Hospital during a period of 2 years. Eighty-one per cent of chil- the higher sociocultural group.
dren had severe TBI according to initial Glasgow Coma Scale Discussion: This left displacement was observed as the chil-
(GCS: score <8). The children were administered a neuropsy- dren were learning to read and write, and was more impor-
chological test battery as soon as possible after the accident,usu- tant and earlier in the group, probably more stimulated with
ally during the first few months after TBI. Half of the children tasks using reading and writing.-
(n=47) were examined for a second assessment, on averagel.6 Conclusion:The normalization of these seven tasks allows us to
years later. The battery included tests for specific cognitive func- assess abnormal asymmetry objectively in children with unilater-
tions, as well as tests of intellectualability (Wechsler Intelligence al neglect.
Scales for Children: WPPSI-R or WISC-III). The present report
deals with measures of intellectualability References
1. Laurent-VannierA, Pradat-DiehlP, Chevignard M, Abada G. (2001)
Results:A signrficant associationwas found between the sever- Negligence spatiale unilaterale et motrice chez I'enfant.Rev
ity of initial GCS and Performance IQ. At first testing, 17%of Neurol (firis) 167 4 1 4 - 4 4 2 .
children scored lower than 70 for Verbal IQ and 24% scored 2. Laurent-VannierA,Pradat-DiehlI: Chevignard M, AbadaG, De
lower than 70 for Performance IQ. The social class of parents Agostini M (2003) Spatial and motor neglect in children.
Neurology 6 0 202-207.
was found to be related to the Verbal IQ. Analysis in the fol- 3. Laurent-VannierA,ChevignardM, Pradat-DiehlI: Abada G , De
low-up group showed that the score for Performance IQ Agostini M. Assessment of Unilateral Spatial Neglect in Children
increased significantly, while no improvement was found for with the Teddy Bear Cancellation Test. (Forthcoming)
Verbal IQ.
Conclusion:The present investigation confirms the relation-
ship between the severityof injury and cognitive sequelae, as
described in the literature. The findings on intellectual abili- 91 LE SOURN-BISSAOUI
ties in the follow-up part of this study suggest that, during the Idiom understanding in autism and Asperger
first year after injury children, showed an improvement in syndrome
performance, however, verbal ability did not improve. S LE SOURN-BISSAOUI, S CAILLIES, F GIERSKI
Universite de Reims, Accolade, Reims, France

Individuals with Asperger syndrome often have intact lan-


90 LAURENT-VANNIER guage ability although they tend to engage in interactive
The Saint Maurice battery: an assessment of communication. This difficulty in using language in a social
unilateral spatial neglect context has been referred to as a deficit in pragmatic lan-
ANNE LAURE NT-VANNIER", MATHIDE CHEVIGNARD", CAROLE DAVID", guage. This difficulty has been observed particularly in
CLEMENCE MATHAP, MARIA D E AGOSTlNl' understanding non-literal language in which the role of con-
asernicede reeducation des pathologies neurologiques text is crucial. The current investigation explored under-
acquises de l'enfant, Hbpital National de Saint Maurice94 standing of idiomatic expressions of adolescents with autism
410 Saint Maurice France; bINSERM LJ472WllejucFrance and Asperger syndrome. A lot of idiomatic expressions are
ambiguous and, as a consequence, ambiguity resolution is
Introduction: Only a few cases of unilateral spatial neglect according to the context. To test hypotheses explaining these
have been reported in children, perhaps because of the lack pragmatic difficulties, related skills such as opacity, referen-
of a test adapted to children.'S2 The Teddy Bear's cancella- tial, and theory of mind, were also measured. Results indicat-
tion test' was augmented by six other tasks. The Saint ed that, relative to normal controls, individuals with autism
Maurice battery was then normalized for children from 3 to 6 or Asperger syndrome have worse performances o n these
years of age. tasks. The findings are discussed in relation to main psycho-
Methods: In addition to the cancellation test, five simple clin- logical theories: theory of idiom acquisition, theory of mind,
ical tasks that are amenable to children, and that appeared to and weak central coherence.
be sensitive to neglect, were chosen. Children were asked to
draw a picture of the sun, to put 20 stickers on a sheet, to
draw a man, to indicate the middle of four lines (two long
and two short), and over 5 years to copy a scene. To measure 92 LEBEER
motor behavior objectively,children were asked to grasp the Dynamic assessment of cognitive modifiability
two sticks of a drum. Each task was scored to measure lateral as a basis for targeted educational intervention
asymmetry objectively. One hundred and seventy normally J LEBEER
developing children from two sociocultural levels and three University of Antwetp Medical School, Belgium
different classes were administered the complete battery.
Results: Concerning the Teddy Bear test, normally develop- This research reports on dynamic assessment in a series of
ing children omitted no more than two teddy bears over 3 307 children with cognitive developmental disabilities with
years, there was no omission over 7 years, and there was varying aetiology and range of functioning. Dynamic assess-
never an omission in the left column over 5 years. ment @A) is a systematic interactive way to assess changes of
Concerning the five othervisuo-motor tasks,analysis ofvari- cognitive functioning after a phase of teaching and learning.
ance showed that the performances were rather symmetrical, The term was introduced by Feuerstein Rand and Hoffman

35
(1979) in which they propose a Learning Propensity Assess- 04 LYNGBYE
ment Device (LPAD) as an alternative to classical psychomet- Calcium density in children with severely
ric IQ testing, which they criticised as being too static to delayed development
assess cognitive potential. Dynamic assessment is based on TROELS LYNGBYE", JETTE STJERNHOLM",BENTE LANGDAHL~
the postulate of the modifiability of cognitive functioning. aPediatricDepartment, Aarhus Sygehus/Hammel
Generally, DA is applied in a pre-test-mediation-post-test Neurocentet; UniversityHospital ofAarhus; bMedical
format. Ninety per cent of children showed a significant Department, Aarhus Sygehus, University Hospital of
quantitative as well as a qualitative increase in cognitive per- Aarhus, Denmark
formance after mediating strategies for analysis, problem-
solving strategies, and vocabulary. Although primarily diag- Introduction: Pathological fractures are often seen in chil-
nostic, dynamic assessment triggered a dynamics of change dren with severe neurodevelopmental delay. An epidemio-
of learning in the child making the child become more moti- logical investigation was undertaken to determine the freq-
vated and more open to learn, which in turn often created a uency of osteoporosis in this population.
shift of perspective in parents and teachers. The improved Material: Acohort was identified. Inclusion criteria were: chil-
learning under conditions of mediation was the basis of dren born between 1976 and 2001;aged over 3 months; severe
designing an academic and cognitive intervention plan. This developmental delay; and living in the county ofAarhus.
is more involved than a plan based on static developmental Exclusion criteria: Trisomy 18 and 13 were excluded. Child-
tests and diagnosis. Although, or perhaps because, results of ren below school age with developmental age above 6
DA are highly dependent on mediating qualities of the evalu- months. Older children above 10 months.
ator, it is claimed that it gives a better clue to understanding Method: The children were scanned by an exa scanner
the child's functioning and learning than standardized IQ Results: Eighty-three children died in the observation period;
testing. Although static testing remains useful to rank chil- 42 were eligible;consent for scanningwas obtained for 14 chil-
dren, dynamic evaluation offers a better alternative to educa- dren; 13 children were osteoporotic and one was osteopenic.
tional targeting of low functioning children which aims at Discussion: It seems that children with severe neurological
modifying the child's level of functioning. disability and severe development delay are osteoporotic.

93 LOPEZ-SALA 95 MAIWORM
Neuropsychological assessment a year after Physiotherapy and the construction of the
moderate-severe childhood traumatic brain future: a Brazilian protocol to evaluate
injury vertebral column complications in children in
ANNA LOPEZ-SAIA, ANNA SANS ROSER COLOME, CRISTINA BOIX, the poorest communities of Rio de Janeiro,
SUSANA FORNE, JAUME CAMPISTOL Brazil
StJoan de DBu Hospital, Barcelona, Spain LT SILVA', C R GODINHO", E SANTOS~,
A I MAIWORM"*~,
L M v VASCONCELLOS", A P FERNANDES", sD SANTOS-RILHO",
Traumatic brain injury (TBI) is one of the most frequent caus- M BERNARDO-FILHO"
es of acquired brain disability in childhood. Motor cognitive Wniversidadedo Estado do Rio deJaneiro, UMJ; bHospital
and behavioural sequelae in both short and long term are Universitario Pedro Ernesto, UEW; cColBgiode AplicaGao,
common after a childhood brain injury. Neuropsychological UERJ,Rio deJaneiro, Brazil
rehabilitation in long-term follow-up has not evolved as far
as medical intervention has in the acute phase. Motor and Objective: Almost 50 million Brazilians are forced to live in
apparent linguistic improvement appear early after TBI. poor conditionswith inadequate health, nutrition, job, home,
Neuropsychologicaldeficits, however, limit future academic, and educational facilities. These are mostly children and ado-
social, and work potential. lescents up to 19 years of age. Ethical discussions and reflec-
We present 38 children who attended the Neuro- tions about daily life and the hture of these communities are
psychological Unit of the Neurology Department at St Joan de highly relevant to try to provide equal perspectives. Physio-
DCu Hospital Barcelona between ages of 6 and 19 years who therapy in multidisciplinary teams improves the quality of
were studied at least one year after moderate-severeTBI. Data life of the people. The aim is to present a Brazilian protocol
collected included severity of injury scores, physiologic vari- elaborated by a group of physiotherapists to evaluate verte-
ables, magnetic resonance imaging, and in-patient hospital bral column complications in children in the poorest com-
progress. Intellectual and behavioural impainnent was deter- munities of Rio de Janeiro, Brazil.
mined and rated by a wide battery of neuropsychologicaltests. Methods: Firstly, the priorities of the communities were
Results indicate that all patients showed significant neu- determined after the analysis of questionnaires. The Vert-
ropsychological deficits a year after TBI, with repercussion ebral column of those (from 7 to 19 years old) from four of
ns in school and social adjustment. Attention, executive the poorest communities of Rio de Janeiro will be evaluated.
function, memory, and behavioural disturbances were the Lectures about the ergonomic conditions in general and the
most prevalent chronic deficits. consequences of possible alterations and complications
Consequences after moderate-severe childhood TBI are associated with the vertebral column will also be offered to
demonstrated in new learning and behaviours. specific workgroups of the communities. Complementary
Mild cognitive deficits can only be detected after specific actions will be implemented by non-government and gov-
cognitive testing and adequate follow-up. ernment organizations. The project will be evaluated by

36 EACDAbstracts2005
merit (intrinsic quality of the actions and relevance). The 9'7 MARSCHIK
impact, effects, and benefits will be also evaluated. Is vocabulary size at 18 months of age
Results:The priorities found in the questionnaires were civil predictive of language abilities at preschool
documentation, care of children up to 3 years of age, and the age?
professional capability of adolescents and adults. Cultural P B MARSCHIK, C EINSPIELER
activities, sport, and leisure for all preventive intervention, Medical University of Graz, Institute of Pbysiology, Austria
health area information, nutrition rights, and obligations are
necessities identified in the questionnaires. Backgmund: Some longitudinal studies on lexical develop
Conclusions: Physiotherapy evaluating the vertebral column ment emphasized that early lexical delay may be indicative of
will assist in providing adequate posture of people from 7 to enduring language problems, dyslexia, or learning disabili-
19years of age and will educate workers' group to consolidate ties, whereas others have shown that children may outgrow
the presented actions. These actions could help in the con- these problems.
struction of a future with quality of life for all. Objective: To investigate the correlation between parental
reports on lexical development and direct observational mea-
sures at the beginning of the second year, as well as the extent
96 MALETIC-SAVATIC to which these two methods could predict language abilities
Identification of neural stem cells in the three to five years later.
human brain: implications for child neurology Method:Twenty-fourhealthy and neurologically normal chil-
L N MANGANAS"-~, xs Z H A N G ~ ,G ENIKOLOPOV~,H BENVENISTE', dren (16 females 8 males) were investigated longitudinally
H COLOGNATO", M WAGSHULA, P DJURICA, M LUALETIC-SAVATIC~'~ from the age of 14 months to 5 years and 7 months. At 18
aState University of New York, Stony Brook; bColdSpring months of age, half of them had not yet acquired 50 words.
Harbor Laboratories, Cold Spring Harbor, N e w York, USA Parental reports, Bayley and Griffiths Developmental Scales,
word-naming tests, and the Peabody Picture Vocabulary Test
Objective:The objective of this study is to identlfy a biomark- were used.
er of neural stem cells (NSCs) which enable identification Results: Parental reports correlated with direct observations.
and quantification of these cells in the living human brain Our results demonstrated that the size of the vocabulary at
using non-invasiveimaging modalities applicable to children 18months of age did not predict children's language abilities
affected by neurological disorders. We are particularly inter- at preschool age.
ested in exploring NSC fate (proliferation, differentiation, Conclusions:Unstable language centile scores appear to be a
migration,or death) and function in infants/pretermneonates common phenomenon during normal development; which
as pre- and perinatal neurological insults frequently lead to appears to be unpredictable and variable. Clinicians and par-
several chronic neurological disorders, such as cerebral palsy ents need to be aware of the intra- and inter-individualvaria-
and mental retardation. tions observed in typically-developingchildren.
Methods: One dimensional 1H-NMR spectra were collected
using mouse-derived cultured neurons, astrocytes, oligo- Acknowledgements: This study is supported by the Austrian Science
dendrocytes, and NSCs, in order to identify spectroscopic sig- Fund (FWF), project number P16984-B02.
~ t u ~ for
e s each individual cell type. Signal processing
algorithms based on frequency, selective singular value
decomposition (SVD) methodology were developed for the
extraction of specific spectroscopic frequency peaks from 98 MAZZONE
data with low signal-to-noiseratios. Finally, we used single- Cognitive and psychological evaluation of two
voxel proton MR spectroscopy of the human brain in order patients with Ring chromosome 15 syndrome
to analyze the presence of NSCs in the human hippocampus. LUIGI MAZZONE~,LILIANA RUTA", VALENTINA GENITORI D'ARRIGO~,
Results: Our preliminaryexperiments have demonstrated that DIEGO MUG NO^, MARIADONATELLA COCUZZA~,CONCEWA SIMONA
mammalian NSCs have a specific metabolomic marker that PERROTTA~
can be identified by 1H-NMR spectroscopy. Furthermore, aDepartment of Child Neurology and Psychiatry, University
NSC can be quantified on the basis of their metabolomic fin- of Catania; bDepartment of Medicine and Genetics,
gerprint. SVD-based algorithms for data processing of the University of Catania, Italy
spectra obtained from the human brain imaging, indicated
that we can detect NSCs in the human hippocampus and not Ring chromosome 15 (r(151) syndrome, first described by
in the control voxel obtained from the cortex. Jacobsen in 1966, is a rare genetic disorder characterized by
Conclusions: The ability to identlfy human NSC by non-inva- specificfacial features, cafe au lait spots, and failure to thrive.
sive brain imaging may have profound implications for diag- The cause of this genetic alteration is de novo hemizygous
nostic, prognostic, and therapeutic purposes. Our data enable deletion of the distal sub-bands of the long arm of chromo-
not only identification but also monitoring of the fundamental some 15 (q26.3 q26.2 and/orq26.1).
changes of NSC that might occur in different neurological dis- We report on cognitive, behavioural, and temperamental
orders, or if used in therapeutic treatments for a varietyof neu- features of two patients with ring chromosome 15 (r[151) syn-
rologicalconditionsfrom birth to elderly age. drome: a male aged 16years and a female aged 7 years.
Methods: Both patients were evaluated with the Wechsler
Acknowledgements:We are grateful to Martine Ziliox for help with Intelligence Scale for Children Revised (WISC-R) Child
thelH-NMR spectroscopy.
Behaviour Checklist (CBCL), Conner's Parent Rating Scale
(CPRS), and EAS Temperament Survey for children.

37
Children’sDepression Inventory (CDI) and Multidimensional 100 McCLELLAND
Anxiety Scale for Children QkfASC) were administered to the Sinusitis in children presenting as severe
male patient. neuropathology
Results: The male patient had normal IQ (WISC-RFull-scale V M MCCLELLAND, D BAKALINOVA
IQ 94, Verbal IQ 94, Performance IQ 96) while the female University Hospital of North Staffordshire, Stoke-on-Dent,
with mild mental retardation had IQ (WISC-R Full-Scale IQ UK
62, Verbal IQ 67, Performance IQ 70). Both patients’ CBCL
scores were within the normal range but in the female Introduction: Children presenting with acute neurological
patient externalizing subscales were higher than internaliz- signs often require urgent cranial imaging. We describe five
ing. She also had a higher level of hyperactivity and inatten- children in whom imaging revealed severe paranasal sinus
tion on CPRS compared with the male patient. infection as the underlying cause.
In the male patient, MASC physical symptoms and harm Patients: The presenting features included loss of vision,
avoidance subscales and MASC total score were higher than seizure, abnormal behaviour, expressive dysphasia, and
age expected scores; CDI score was within the normal range. reduced level of consciousness. In each patient the features
EAS scale indicated low sociabilityin both patients; a high of sinusitis were mild or had resolved. The development of
level of activitywas present in the female patient. complications led to the above neurological manifestations
Conclusion: Our report suggests that ring chromosome 15 triggering presentation. The complications included sphe-
(r( 151) syndrome can have different behavioural and tempera- noid mucocoele, extradural empyema, subdural empyema,
mental phenotypes.Younger age and a lower IQ level could be subgaleal abscess, and probable compressive optic neuropa-
associated with externalizing features like hyperactivity and thy. One child presented with classical features of sinusitis
inattention,while a normal cognitive level could be associated and periorbital cellulitis but was found incidentally to have a
with internalizingfeatures probably related to low self-esteem. cerebral abscess on cranial scanning. W o patients required
Low sociabilitywasa shared feature of both patients. craniectomy and all but one required functional endoscopic
sinus surgery to eradicate the source of infection.
Conclusions: Intracranial and intraorbital complications of
sinusitis still occur and can be life-threatening.These patients
highlight the need to consider sinus-related disease in the
Behavioural and temperamental phenotype in differential diagnosis of children presenting with abnormal
children with Velo-cardio-facial syndrome neurology. Imaging the sinuses as well as the head may iden-
& I & A, LILUNA R U T A ~ ,VALENTINA GENITORID’ARRIGO~, tlfy the source of infection, allowing for prompt treatment
DIEGO MUG NO^, MARIADONATELLAC O C U Z W ~ , CONCETTA SIMONA and avoidance of potentially devastating consequences.
PERROTTA~
aDepartment of Child Neurology and Psychiatry, University
of Catanla; bDepartmentofMedica1Genetics, University of
Catanfa,Italy 101 McCLELLAND
GClutaric aciduria Type 1 presenting as epilepsy
Background Velo-cardio-facial syndrome (VCFS) is a genetic VM MCCLELLAND, D BAKALINOVA, R P SINGH
disorder associatedwith 22qll deletion, a characteristic physi- UniversityHospital of North Staffordshire, Stoke-on-Dent,
cal phenotype, cognitivedeficits, and a high rate ofbehavioural UK
problems and major psychiatric disorders during adolescence
and adulthood. Behavioral and temperamentalevaluation was Introduction: Glutaric aciduria I).pe 1 (GA-1) is an autosomal
undertaken in children with VCFS and differentdegrees of cog- recessive disorder resulting from deficiency of glutaryl coen-
nitive level. zyme A dehydrogenase. Clinical expression is variable but
Metbods: Three female and two male patients (mean age 8 onset usually involves an acute encephalopathic episode in
years 5 months) with VCFS were assessed with Wechsler infancy associated with intercurrent infection. Subsequently,
Intelligence Scale for Children Revised (WlSC-R),Child Behav- there is gross motor delay with severe dystonia-dyskinesia
iour Checklist (CBCL), Conner’s Parent Rating Scale, and EAS reflecting basal ganglia injury. Cognitive function is relatively
Temperament Survey for Children for cognitive, behavioural, preserved. Seizures may occur with the acute encephalopathy
and temperamental evaluation. but are unusual in the long-term.We describe an atypical pre-
Results: ?fKopatients had normal cognitive level (WISC-Rtotal sentation of this condition.
IQ 100 and 85 respectively) and three patients had mild men- Case report: A previously well 6-year-old female was referred
tal retardation (WISC-Rtotal IQ 67 62 and 69 respectively). with recurrent seizures that proved difficult to control with
CBCL profiles were within the normal range but the inter- first line anti-epileptic drugs. There was no history of
nalizing anxious/depression subscales were the highest in all encephalopathy.Development had been normal and she had
patients. The EAS scale showed high levels of shynessand emo- no neurological abnormality.Electroencephalogramwas pro-
tionality and low sociabilityin four of five patients. foundly abnormal with mixed multifocal and generalized
Crmclusion: VCFS is associated with a variable cognitive, seizure discharges. Metabolic investigations revealed m e 1
behavioural, and temperamental profile. In our sample a m - glutaric aciduria. The basal ganglia were unaffected on cranial
ious/depressive traits,high level of shyness, emotionality and magnetic resonance imaging. However, the Sylvian fissures
low sociability were common features. These could be early were prominent, which is one of the landmark radiological
predictors of major psychiatric disorders in adolescence and features in this condition.
adulthood which were reported in over 10%of these patients. Conclusion: Seizures have been described in a few patients

38 EACDAbstracts2005
with GA-1 who also had severe motor problems or cognitive measured using a hand-held goniometer. Three dimensional
difficulties. This is the first report where seizures were the ultrasound scans were made of the medial and lateral gas-
sole presenting feature. This case highlights the importance trocnemius (MG, LG) and volume and length data were
of metabolic investigationswhen first or second line treatment obtained.
of seizures is unsuccessful: an underlying metabolic disorder Results: Following SC, maximum ankle dorsiflexion (knee
may not only cause seizures but may complicate their man- flexed) increased inevery case (mean 7y 8mo;p=0.001). Ankle
agement. Understanding the heterogeneity of GA-1 is also dorsiflexion (knee extended) MG and LG belly length and
important when counselling families regarding prognosis. volume did not alter significantly following casting. In the
group of typically-developing children MG ad LG volume
increased linearlywith weight (R2=0.86).Children with SCP
102 McDONALD had significantly smaller MG and LG belly lengths and vol-
Investigation of global developmental delay: umes than their typically-developingpeers @<0.05).
evidence-based guidelines Conclusion:These data suggest that SC increasespassive ankle
LAB MC DONALD, AC RENNIE, J TOLMIE, P GALLOWAY, dorsiflexionrange with the knee flexed but not when the knee
R MCWLLIAM is extended. Children with SCP have reduced gastrocnemius
Royal Hospitalfor Sick Children, Yorkhill,Glasgow, UK volume and length in the presence of mild ankle contractures.
SC does not alter gastrocnemius muscle morphology.
Objective:To create evidence-basedguidelines for the investi- From these results it appears that SC has a greater effect
gation of global developmental delay in pre-school children. on the soleus than on the gastrocnemius. However if SC is to
Metbods: The investigation of global developmental delay in provide a plantigrade ankle position in standing and walking
pre-school children varies between centres and between while maintaining extension at the knee length also needs to
paediatricians. Following a literature search and review of be gained within the gastrocnemius musculotendinous unit.
the evidence, base guidelines were developed to assist in the
assessment and management of such children presenting to Reference
1. Brouwer et al. (2OOO).JPediutrOrtbop 2 0 221-225.
secondary level services.
Results: Evidence supporting the use of genetic and bio-
chemical investigationson a screening basis was found. There
was no evidence to support the use of metabolic investiga-
tions, neuroimaging, or electroencephalography in the abs- 104 MEESTER-DELVER
ence of other positive findings on history or examination. The Capacity Profile: a classification of the
Conclusion:Detailed history and examination are paramount future need for additional care in children
in the assessment of children with global developmental with developmental disabilities
delay. Investigations can be a useful adjunct in determining A MEESTER-DELVER", A BEELEN", R H E N N E K A M ~F, NOLLET",
aetiology. Evidence-based guidelines have been developed M HADDERS-ALGRA~
to assist doctors in the selection of appropriate investigations aDepartment of Rehabilitation, Academic Medical Centre,
for this group of children. These guidelines are designed to Amsterdam; bDepartment of Paediatrlcs, Academic
be a useful working tool for paediatricians. MedicaI Centre,Amsterdam, the Netherlands and Clinical
and Molecular Genetics Unit, Institute of Cbild Health,
Great Ormond Street Hospital for Sick Children, University
CollegeLondon, London, UK; 'Department of Neurology,
103 McNEE UniversityMedical Centre, Groningen,Groningen,tbe
The effect of serial casting on gastrocnemius Netherlands
muscle length and volume in children with
cerebral palsy. Objective: For parents and other caregivers of young chil-
A E MCNU", E WLLM C S P , J-P LIN, L C EVE", N R FRY", dren with developmental disabilities it is essential to be
M C MORRISSEY~,A P SHORTLAND" informed about the future need for care of these children.
*&diatrfc Neurosciences, Guy'sHospital London; We developed the Capacity Profile (CAP), an instrument to
bDivisonof Biomedical Sciences, King's College,London, classify the need for additional care in young children with
UK non-progressive permanent developmental disabilities. The
CAP distinguishes additional care as the consequence of
Objectives:Serialcasting (SC) aims to improve ankle dorsiflex- impairments in five domains: physical health; motor func-
ion range in children with spastic cerebral palsy (CP).* Gastroc- tions; sensory functions; mental functions; and voice and
nemius muscle length is thought to increase following SC but speech functions. The intensity of additional care in each
this has not been studied. We were interested in the change domain can be classified ranging from 0 (no additional care)
in gastrocnemius muscle morphology following SC in chil- to 5 (needs help for each activity). The intensities of care in
dren with CP, and how morphology in CP and typically-devel- the various domains form the CAP of the individual child. We
oping children differ. investigated interobserver reliability and stability over time
Materialslmethods:We studied below-knee SC in eight chil- of the CAI!
dren with CP (two hemiplegia, five diplegia) aged 6 to 8 years. Metbods: Sixty-sevenpersons aged 18years with non-progres-
Participantswere assessed before casting and 5-weeksfollow- sive permanent developmental disabilities participated in the
ing the first casting. We also assessed 11typically-developing study. Multidisciplinary information at the age of three years
children aged 4 to 13 years. Passive ankle dorsiflexion was on these individualswas available.To determine interobserver

39
agreement, three physicians independently assessed multidis- not differ significantly.
ciplinary information available when the participants were 3 Conclusion: There are significant differences between TBI
years old and determined the CAI! The participants had a clini- children and controls for a range of behaviours. Group dif-
cal re-assessmentwhen they were 18 years old by a physician ferences in externalizing behaviours reflect pre-morbid sta-
who was unaware of CAP status at age three. Agreement tus, but differences in internalizing behaviours and social
between CAP scores at age 3 years and age 18 years provided competence appear to be due to TBI.
information on stabilityof the CAP scores.
Results: Interobserver agreement was good: weighted kappa
(k)values ranged from 0.62 to 0.85. Stability of CAP scores
over the years was moderate for the physical health domain 106 MONTALTO
(k=0.47) and was good to very good for the other domains Early behavioural phenotype in a child with
((k=0.70 to 86). inv dup (15)
Conclusion: The CAP has good interobserver reliability and M MONTALTO, M IMPASTATO, A LO BUE, A BARBAGALLO,
stability over time (except for the physical health domain). A FONTANA, S MANGANO
Thus, it is a promising instrument to predict the future Dipartimento Materno Infantile, Unita di Neuropsichiatria
requirements for children with developmental disabilities. Infantile, Universita di Palerrno, Italy

Introduction: Supernumerary chromosomes may occur in


as many as 0.05% of live births and inv dup (15) represents
105 MILLER approximately half of these. The clinical spectrum exhibited
Behavioural outcome at one and six months by participants with inv dup (15) includes muscle hypotonia,
after severelmoderate and mild traumatic mild dysmorphisms, seizures, cognitive deficits, and behav-
brain injury (TBI) in childhood: relationship ioural disorders.
to pre-injury behaviour Objective: To describe the early behavioural phenotype in a
H E MILLER, A L CURRAN, RJ MCCARTER, LP HUNT, P M SHARPLES female nondysmorphic patient with inv dup (15).
Frenchay Hospital, Bristol, and Bristol Royal Hospitalfor Methods: The patient underwent a psychiatric examination
Children,Bristol, UK (infant observation); intellectual profile was evaluated by
Brunet-Lezine test; behaviour assessment was performed by
Introduction: Traumatic brain injury (TBI) is a major cause parents interview and BECS; laboratory investigations: kary-
of paediatric hospital admissions. Disturbed behaviour is otype from peripheral blood lymphocytes with standard
well recognized in children with TBI but it is unclear if this is technique and FISH for chromosome 15.
due to injury or pre-morbid functioning. Case report: The patient is a female born after term gestation
Objectives: (1) To define behavioural outcome in children with a normal delivery. In the neonatal period, seizures were
with TBI compared with controls and (2) to relate pre-injury treated with phenobarbital and crisis of cyanosis with desatu-
behaviour in children with TBI to behaviour in controls and ration. At our first observation at the age of 1 month, we found
to behaviour post-TBI. indifference to sounds. In the course of the first 5-7 months of
Methods: Longitudinal prospective cohort study. TEiI was clas- life we observed severe hypotonia with developmental delay
sified as severe (Glasgow Coma Score [GCS] 3 to s),moder- and visual inattention. At age 9 months we observed infantile
ate (GCS 9 to 12), or mild (GCS 13 to 15). Controls were spasms without hypsarrhythmia which remitted after initia-
non-injured children matched for age, sex, and socioeco- tion of ACTH therapy At age 14 months visual attention was
nomic status. Pre- and post-injury behaviour was assessed slightly improved but we observed stereotyped movements
using the parent report Child Behaviour Checklist (CBCL). such as body-rocking. At age 18 months a coordination and
Statistical analysis was by two-way repeated measures socialization deficit persisted, there were difficulties in stand-
ANOVA and one-wayANOVA with Scheffe’smultiple compar- ing up and palmar grasping, poor language with only guttural
ison measures. sounds, and few two-syllabledwords. She was strongly attract-
Results: Eighty-six children with TBI and 47 controls were ed to bright colours and sounds. Stereotypical behaviour,
recruited. Mean age of children with TBI was 11 years (SD 3y such as body-rocking and hand rubbing on smooth surfaces,
10mo); mean age of controls was 11 years 1 month (SD 3y persisted. Social interaction was very poor.
1Omo).For post-injurybehaviour there were significant differ- Cytogenetic study demonstrated a supernumerary chro-
ences at 1 and 6 months between severehnoderate and mild mosome (47 XX +mar). FISH analysis was diagnostic of inv
TBI children and controls for all aspects of the CBCL, i.e. dup (15).
Externalizing Index, Internalizing Index Social Competence, Conclusions: This case report emphasizes the importance of
and Total Problem Score (p<O.OOl).For pre-injury behaviour genetic testing in the characteristic early behavioural pheno-
there were significant differences between TBI groups and type (visual inattention, attraction to bright colours stereo-
controls for CBCL Externalizing Index (p=O.Ol) but not for typed behavior) in children without facial dysmorphisms.
Internalizing Index (p=0.30), Social Competence (p=0.51),
or Total Problem Score (p=0.08). Comparison of pre- and
post-injury behaviour up to 6 months in children with TBI
showed no significant change in CBCL Externalizing Index
@=0.35) but significant changes in Internalizing Index
(p=O.Ol), Social Competence (p<O.OOl),and Total Problem
Score (p=0.003); in the latter analysis the two TBI groups did

40 EACDAbstractsZOO5
107 MURPHY and improve the quality of life of the child and family. This
Tics et al: group work in young people with study shows the results of children born preterm who have
Gille de la Tourette syndrome participated in early intervention and were followed up 6
T L MURPHY years later.
Gille de la TouretteClinic, Great Ormond Street Children's Method: The study included 10 children of 30.5 (SD 1.85)
Hospital, London,UK weeks' gestational age and 1410 (SD 374.58) grams birth-
weight. All the children were diagnosed with CP in the
Objective:GiUe de la Tourette Syndrome (TS) is a well-known Hacettepe University Pediatric Neurology Department. They
yet often misunderstood neurodevelopmental disorder. In were then referred to the Hacettepe University School of
addition to the characteristic motor and vocal tics, young Physical Therapy and Rehabilitation CP Unit for the early inter-
people with TS report a number of comorbid difficulties vention programme. The clinicaltype of the children was spas-
such as attention-deficit-hyperactivity disorder, obsessive tic CP and extremity distribution was: diparetic, n=7;
-compulsive symptoms, and anger management difficulties. quadriparetic, n =2, and hemiparetic, n = 1. Mean diagnosis
A study employing structured social skills group work in age of the children was 6.8 months (SD 4.7). The early inter-
young people with TS has prompted positive feedback from vention programme consisted of positioning and handling of
patients (Lambert and Christie 1998). However, it was felt infant and parent education in Bobath's Neurodevelopmental
that the strategies used in previous groups needed to be Therapy. Children attended early intervention 5 days a week
adapted to best suit young people with TS. Strategies used for 12 months and at regular intervals families were trained and
within a group intervention have been tailored specifically to advised about home exercise programmes. Mean of Gross
the needs ofyoung people with TS and comorbid difficulties. Motor Function Measurement (GMFM) at 12 months of age
Method: Seven males (age 11 to 14y) with a diagnosis of TS was 37.45 (SD 13.64).All the children continued physiothera-
participated in six once-monthly group sessions. Each ses- py in difTerent pediatric rehabilitation centres. At 6 years of age
sion focused on a specific difficulty commonly associated the children's parents were contacted and appointments made
with TS. The group sessions lasted for 90minutes. The sessions in our unit to examine the children's health status. We used the
concentrated o n managing tics, building self-esteem, school GMFM to assess motor functions.
learning and bullying, anger management, obsessive-com- The Functional Independence Measure for Children
pulsive symptoms, and a concluding session. Interventions (WeeFIM) and the Pediatric Evaluation of Disability Inventory
such as psychoeducational modelling, behaviour rehearsal, (PEDI) were used to assess the functional performance of the
and interpersonal problem-solving were used. Drawing on children.
previous evidence, strategies such as cognitive restructuring Results: We classified the children according to Gross Motor
and emotional regulation were also used in the intervention. Function Classification System (GMFCS). Five were at Level
The groups were co-facilitatedby two clinical psychologists. I, three at Level 11, and two at Level Ic! The mean of total
Results: Feedback from the young people and their parents GMFM scores was 79.05 (SD 25.58). For the WeeFIM, mean
was positive. An audit of the group showed that the partici- ofself-carescoreswas42.12 (SD 12.43),mobilityscores were
pants found meeting other young people with TS, in addi- 27.37(SD 8.21), and cognition scores were 29.75 (SD5.42).
tion to learning new strategies specific to their particular There was a significant correlation between total GMFM
difficulties,to be helpful. scores and PEDI functional skills, self-care, and social func-
Conclusions:A group intervention was specifically designed tions raw score (pC0.05, r 0.78)
for young people with TS and comorbid difficulties.The inter- Conclusion:Early intervention has a significant effectiveness
vention focused on developing cognitive behavioural strate- in achieving gains or improving motor activities and func-
gies in managing a number of diaculties associated with TS. tional independence, especiallyin children born preterm.
Feedback from the group was positive. Approaches and strate-
gies for future work in this area are discussed.
109 NEENAN
Autistic spectrum disorder without cerebral
palsy following preterm delivery
108 MUTLU F M NEENAN, R E M O R T O N , E MARDER
Six-year follow-up of children with cerebral UniversityHospital Nottingbam, Nottingham, UK
palsy born preterm
A M U T L U ~ ,M K GUN EL^, G T U R A N ~ , A LIVANELIOGLU" Introduction: It is now recognized that preterm infants have
aHacettepeUniversity,School of Physical Tberapyand a high risk of developing neurologically disabling problems
Rehabilitation,Ankara; bHacettepeUniversity, CH, Ankara, such as cerebral palsy (CP) in childhood. This has been well
Turkey validated by recent evidence from the Epicure study. Autism
is not one of these documented problems.
Objective: Low birthweight or preterm birth are major natal Method: We present five children, three females and two
factors in cerebral palsy (CP). In the literature, long-term males, each of whom had a complicated and difficult neona-
results of preterm infants born between 24-28 gestational tal course following a preterm delivery. In none of the five
weeks show that 25%of infants have major disorders and 11% patients did CP ensue. There were no long-term problems
developed CP Babies with low birthweight of less than 1500g from retinopathy of prematurity in any of the patients.
have a 40 times higher risk of developing CP Early intervention However they are all on the autistic spectrum. The age of ini-
of physical therapy should begin neonatally to increase the tial diagnoses spanned from 2.5 years to 13 years.
rate ofacquisition of motor skills,minimize possible disability, Conclusion: These five children demonstrate a non-motor

41
clinical problem characterized by being on the autistic spec- Objective:The transition phase from childhood to adulthood is
trum. The important aspect of these patients is that together considered as critical to the development of participation. This
they show that it possible to be on the autistic spectrum even may lead to specific(unmet) needs and associated utilization of
if avoiding some or all of the problems of preterm birth. health care. In the literature a decreased number of contacts
These include CI: visual deficits, and learning difficulties. with health care practitioners during the transition phase is
These observations have important implications for coun- reported and it is uncertain which (unmet) needs exist.
selling families and for the ongoing assessment and educa- Therefore, the current study is aimed at a descriptionof unmet
tional advice for the children. Being on the autistic spectrum needs and health care utilization ofyoung adults with cerebral
followingpreterm deliveryis likely to represent another mani- palsy (CP) in the transition into adulthood.
festation of widespread damage to the developing immature Methods: As part of the CP Transition Study in the south-west
brain caused by extreme preterm birth. Netherlands, adolescents and young adults aged 18to 22 years
and diagnosed with CP without severe learning disabilities
(n=90)were assessed with respect to their unmet needs and
110 NEWMAN health care utilization. The outcome measures are the
Co-sleeping in children with cerebral palsy Canadian Occupational Performance Measure (COPM) and
C I N E W U , 0 HENSEY written questionnaires on unmet needs and received health
CentralRemedial Clinic,Dublin, Ireland care in the past year. This study is performed as part of Pediatric
RehabilitationResearch in the Netherlands (PERRIN) .
Objective:To determine the frequency of children with cere- Results: Three-quarters of the participants reported unmet
bral palsy (CP) who sleep in the same bed as their parents needs. The number of unmet needs varied from 0 to 15 (mean
and to identdy which factors predict this practice. 3.4). Unmet needs on functional mobility (e.g.transport, walk-
Methods: Cross-sectional study conducted by mail. Quest- ing long distances) were reported most (50%of participants).
ionnaireswere posted to the parents of 249 children attending With regard to information, two-thirds of the participants
our centre for physical therapy. Children aged 6 to 12 years reported at least one unmet need, with a requirement for more
with a diagnosis of CP were included. One hundred and seven- information regarding the consequences of CP reported
ty-three out of 249 questionnaires were returned, a response most (47% of participants). In the past year, 73% visited a
rate of 69.5%.The parents completed the Sleep Disturbance medical specialist,while the rehabilitation physician was vis-
Scale for Children to assess the child's sleep over the previous 6 ited by 38%.Therapists were visited by 40% of the partici-
months. Uni- and multivariate analyses were performed to pants, mostly a physiotherapist (38%). However, 17%
determine associations of co-sleeping with sleep disorders, reported a need for more physiotherapy, of whom a quarter
and physical and socio-familial factors. already received physiotherapy.
Results: Bed sharing occurred at least three times a week in 28 Conclusions:A significant number of young adults with CP
families (16%).This took place every night in 16 families (9%) report unmet needs in different aspects of their lives such
and three to five nights per week in 12 families (7%).Children as mobility disability-specific information and received
slept in their parents' bed in 10 families (6%),parents slept in health care.
their children's bed in nine families (5%),and both patterns of
bed sharing occurred in nine families (5%). Co-sleeping was
associated with disorders of initiation and maintenance of 112 NIJHUIS
sleep (OR=5.6 95% CI, 1.6-19.4) and sleep-wake transition Rehabilitation for children with cerebral
disorders (OR=6.7 95%CI. 2.&22.1). Children with epilepsy palsy: a complex interaction between
were more likely to co-sleep whether they had active seizures rehabilitation professionals, special education
(OR=7.5 95%CI, 1.3-42.8)or not (OR=7.8 95%CI, 1.4-42.5). professionals, and parents
Children with spastic quadriplegia were less likely to share a B I G N I I H U I S " ' ~H
, A REINDERS-MESS EL INK^, A C E DE B L E C O U R P ~ ~ ,
bed with theirparents (OR=0.195%CI, 0.0-0.9). ~ , NAKKEN", J w GROOTHOFF~, K POSTEMA M
WG O L I J V E ~ ' H D ~ ~ ~
Conclusion: About 1/6 families of children with CP co-sleep aCentrefor Rehabilitation, UniversityMedical Centre
regularly, whereas this is generally reported to occur in around Groningen,Groningen;bCentrefor Rehabilitation,
1/20 school-age children in Caucasian populations. Co-sleep Revalidatie Friesland Beetsterzwaag; CDepartmentof
ing may be a response to child and/or parental fears of noctur- Special Education, University of Groningen;dNortbern
nal seizures in those who suffer from epilepsy. It could also Centrefor Health Care Research, University of Groningen,
reflect how certain parents cope with the frequent sleep prob- Medical Centre, Groningen, the Netherlands
lems of children with CP
Objective:The main study objective was to describe the inter-
action between the three main stakeholders in the care
process: rehabilitation professionals, education profession-
111 NIEUWENHUIJSEN als, and parents. This interaction was described in terms of
Unmet needs and health care utilization in team characteristics and stakeholders' perceptions of both
young adults with cerebral palsy the care process and current team collaboration practices.
NIEUWENH U I I S E N ~ M
, DONKERVOORT~,D J H G WIEGERINK~J', Methods: A descriptive study on rehabilitation teams provid-
M E ROEBROECK~,HJ ST AM^ ing care for children with cerebral palsy (CP; mean 7y Omo,
aErasmusMC-UniversityMedical Centre,Rotterdam ; SD l y 2mo; 29 males 15 females GMFCS level mean 2.29, SD
bRiJndamRehabilitationCentre, Rotterdam, the Netherlands 1.53) and their parents. Five rehabilitation centres and co-
operating schools for special education participated in the

42 EACDAbstracts2005
study. Datawere collected by an analysis of institutional docu- ble alveolar contact in all of the words. Further results are
ments and questionnaires. expected both from EPG-data and perceptual evaluations.
Results: AU three stakeholders were represented in the 44 Conclusions:The results suggest that EPG can be valuable in
child-specific teams, each with an average of 10.5 members. treating motor speech disorders due to CE
These team members comprised 161 rehabilitation profes-
sionals, 157 education professionals, and 44 parents, the lat-
ter made up of 13 different professions.
In relation to invitation and attendance at team meetings 114 NORDSTROM
some differences were found between the three stakeholders Participation in peer relations in inclusive
and also between the five settings. Parents were invited to two schools
of the five settings, while the level of attendance at team meet- u L F JAN SON^, IRENE NORDSTROM~
ings was higher for rehabilitation professionals (88.9%)than aStockholm University,Stockholm; bOrebro University
for special education professionals (41.8%). Overall, team Hospital, Sweden
member relationships were described as adequate. However,
relationships between professionals from the same stakehold- Social participation is a central objective when including
ers group were reported to be better than relationships with children with disabilities in pre-school and school. However
professionals from other stakeholder p u p s . Perceptions on several questions can be raised concerning this objective:
family-centredcare, experienced quality of collaboration prac- what kind of participation is afforded, where, in which activi-
tices, and processes in team meetings differed sigruficantly ties, and how is it experienced? The author’s previous
between the three stakeholders. research in pre-school and school shows that participation in
Conclusions:The results showed the complexityof the collab- peer activities raises problems, tending to result in marginal-
oration process. Rehabilitation professionals, special educa- ization and exclusion. What are the conditions for accep-
tion professionals, and parents work together in large teams tance in the peer group?Why should functional difference be
around one child while team members are involved in a differ- an obstacle?How is marginalization socially constructed and
ent way and have different experiences with the care process legitimized within the peer culture?
and team collaboration practices. The study looked at two Swedish schools with pupils with
disabilitiesin ordinary or in special classes.Children also attend
integrated afternoon leisure homes. Consequently,pupils with
disabilities and those without meet in many settings.What h a p
113 NORDBERa pens in these encounters and how are they experienced?Data is
Electropalatography in treatment of children obtained through observations in playgrounds, classrooms,
with speech disorders due to CP and afternoon homes by means of individual interviews and
A NORDBERG, E BERG focus groups with children (with disabilities, without disabili-
Habiliteringen Centrum, Gothenberg,Sweden ties, and mixed group), teachers’ assistants, and physiothera-
pists, occupational and speech therapists, and psychologists.
Objectives:In this study two males, aged 6 and 11 years, with Preliminary results indicate positive attitudes but few and
dyskinetic cerebral palsy (CP) received speech therapy with troublesome contacts between functionallydifferent children.
visual feedback through electropalatography (EPG). Despite Marginalization is a subtle, seldom explicit process often
receiving traditional speech therapy for years, their motor resulting in a decision by the child with disabilities to withdraw.
speech disorders are still moderate to severe. Both males Reasons given by informants, such as few meeting places for
show defective production of alveolar phonemes -hence this functionally different children, and lack of knowledge, seem
was the primary target of the training. more like rationalizationsthan real causes for marginalization.
The aim of the present study was to discover whether train- Pupils who move from ordinary to special classrooms tend to
ing with EPG would improve the participants’ speech, the report that they are accepted more and make more friends in
focus being on their articulation of the alveolar stops Wand /d/. the new situation. The authors will discuss how ideas about
Metbods: EPG is a technique which records the location and disability are constructed and maintained within the nondis-
timing of tongue contacts with the hard palate during speech. abled peer culture and, though not explicitly expressed, still
It has been used since the 1960s, e.g. in phonetic research create conditions for restricted participation.
and lately also as a visual feedback method in diagnosis and
treatment of speech disorders. Patients who have not bene-
fited from traditional speech training have been helped
by EPG training. The EPG-therapy took place over eight weeks. 115 O’HARE
Three recordings of EPG-data and audio recordings were Effects of computer-based intervention using
made before and after the EPG-training. acoustically modified speech, Fast ForWord
Results: Preliminary results show that both males have Language, in receptivelanguageimpairment:
improved their articulation of initial /t/ and /d/. One of them outcomes from a randomized controlled trial
very rarely reached the alveolar place of articulation before A O’HARE, W COHEN, A HODSON, J BOYLE, T DURANI,
training while after training he reached the alveolar place in E MCCARTNEY, M MATTEY, L NAFTALIN, J WATSON
most of the words. The other boy varied the place of articula- Department of Child,Lqe, andtiealth, University of
tion a lot before training. While sometimes reaching the alve- Edinburgh, University of Strathclyde, UK
olar place he would most often make a velar stop or make no
contact with the palate at all. After training he showed a sta- A t m : To study the effect ofexposure to Fast ForWord Language

43
0;FW) games on the language levels of children with receptive However, when matched for verbal mental age, not all ele-
specific language impairment, through the design of a random- ments of prosody were equally afTected in the children with
ized controlled trial with blind assessment of outcome. autism, who had significant difficulties both comprehending
Methods: Seventy-seven children between the ages of 6 and affect (p=0.003) and employing prosody to convey afTect
10 years with specific receptive language impairment (SLI) (p=0.001).
participated in a randomizedcontrolled trial of FFW( a comput- Conclusions:The dysprosodic speech of children with autism
er-based intervention using acousticallymodified speech. All can confer a life-long communication impairment even after
children maintained their regular speech and language ther- improvement in other areas of language.
apy and school regime throughout the trial. Standardized Prosody is a complex construct serving a wide variety of
measures of receptive and expressive language were used to communicative functions including affective,pragmatic, and
assess performance at baseline and to measure outcome at 9 functional. This is the first report to detail how these compo-
weeks and 6 months. Children were allocated to one of three nents are impaired in autism.
groups: Group A (n=23) received the FFW intervention as a
home-based therapy for 6 weeks. Group B (n=27) received Acknowledgement: Funder: Chief Scientist Office, Scotland.
commercially available computer-based activities designed to
promote language in order to control for computer games
exposure. Group C (n=27) received no additional study
intervention. 117 BSTENSJB
Results: Multivariate analysis of variance (MANOVA) was Assessment of the GMFCS as a guide for
used to determine the effects of group data collection point clinical work with children with cerebral palsy
(baseline vs 9wks vs 6mo) and the group by data collection 2 0STFNSI0
point interaction, which in the light of the equivalence of the Oslo University College, Oslo, Norway
pre-treatment scores provides a measure of any post-base-
line between-group differences resulting from intervention. Objective:Assessment of the Gross Motor Function Classification
There was no significant main effect of group for the System (GMFCS) as a guide in clinical decision-makingand ser-
primary outcome measures of receptive language scores vice planning for young children with cerebral palsy (CP).
(F[271]=0.007, p=0.993), expressive language scores (F[2 Method: Participants were 95 children (55 males and 40
70]=0.207 p=0.814), or total language scores (F[2 females, mean age 58m0, SD 181110). Severity of CP spanned
70]=0.261p=0.771) on the CELF 3UK.Similarly, there was all five levels of the GMFCS. Muscle tone range of motion and
no significant main effect of group for the other secondary selective motor control were measured in the more involved
outcome measures (p>0.085). leg. Everyday functioning was assessed using the Pediatric
Conclusions: Groups made significant gains in language lev- Evaluation of Disability Inventory (PEDI) and a Likert scale.
els but there was no additional effect for either computer Results: Children walking unaided (GMFCS levels I and 11)
intervention. The data failed to provide support for the tem- were less impaired in motor functions and more capable in
poral processing deficit account of receptive SLI. self-care mobility and social function than children walking
with devices or moving on the floor (levels 111 and rv).With
the exception of muscle tone children with limited self-initi-
ated mobility (level v> were more impaired in motor func-
116 O'HARE tions and more limited in everyday activities compared with
Prosodic ability in children with high children at levels I11 and N Moderate to high increase in tone
functioning autism in combination with low selective motor control was most
A O'HARE, J M C C A " , S PEPPE, F GIBBON, M RUTHERFORD commonly seen in children at level V The GMFCS was a strong
Speech Sciences, QueenMargaret University College,Child predictor of everyday activities. Significant differences in use
Lrfe andHealth, University ofEdinburgh, UK of environmental modificationsappeared between all GMFCS
levels with the exception of levels N and V The majority of
Aims: To investigate both receptive and expressive prosodic the 980 modifications provided to support everyday activi-
skills in children with autism and compare the resulting pro- ties was in use among children at level N and N Both these
files with those of typically-developingchildren matched for groups benefited considerably. For functional indepen-
verbal cognitive age. dence, twice as many children at level N benefited compared
Method: Thirty-one children aged 6 to 13 years with high- with children at level y whereas for caregiving demands both
functioning autism and a verbal mental age of over 5 years, groups benefited equally About half of the children at level
and 72 typically-developing children matched for verbal I11 and only a few at level I1 benefited.
mental age, sex, and socioeconomic status participated. All Conclusions:The findings support use of the GMFCS to guide
of the children completed the PEPS-C test, which is a com- clinical decision-making and service planning for young chil-
prehensive test of both expressive and receptive prosodic dren with CP and their families.
ability.
Results:An independent samples t-test showed that the chil-
dren with autism performed significantly poorer overall on
the PEPS-C than their verbal mental age matched peers
(p=O.OOl).In addition the prosody results correlated highly
with the language measures (r=0.559,p=O.OOl) but not
with the nonverbal measure or the articulation measure.

44 EACDAbstracts2005
118 PALAZZI ‘clusters’ of impairments and their relationship to other
Reproductive decision making in tuberous impairments.
sclerosis Results: Sixty per cent of the CP population have multiple
STEFAN0 PALAZZI, PATRICK F BOLTON impairment and 28%have severe multiple impairment. Being
Institute of Psychiatv, London, UK multiply impaired was significantlyrelated to walking status (a
higher proportion are unable to walk;p<O.001); CP subtype
Objectiue: In order to assess the factors involved in repro- (a higher proportion have non-spasticCP;p<O.001);and ‘ori-
ductive attitudes after the diagnosis of a genetically deter- gin’ of CP (a higher proportion have postneonatal C P
mined condition, a survey was carried out on tuberous p < O . O O l ) . Beingseverelymultiplyimpairedis onlysignificant-
sclerosis (TSC), a multisystem disorder characterized by an ly related to CP subtype (a higher proportion have bilateral
autosomal dominant inheritance. spastic or dyskineticCP;p<O.OOl).The most common combi-
MetMs: English TSC association members (mainly parents) nations of multiple impairments were: motor and visual
agreed to complete a postal questionnaire. The available data impairment only (26%); motor and intellectual impairment
set allows comparison ‘stoppers’ and ‘non-stoppers’ and only (25%); and motor, intellectual, and visual impairment
exploring some factors explaining parental choice including only (23%).These findingswill be described in more detail.
socio-demographics, consanguinity, relevant beliefs, knowl- Conclusions:A high proportion of children with CP experi-
edge of heritable risk,the affected child’sbirth order, and the ence multiple impairments likely to require multidisciplinary
perceived burden of their disability. assessment and support. This has implications for the orga-
Results: More than 10%of the target population participated nization of health services and for families who require sup-
in the study (n=510) and 396 completed the outcome item. port to co-ordinate care for children with complex needs.
Seventy three per cent had enacted some birth control after
one or more of their children had been diagnosed with TSC. Reference
1. Surveillance of Cerebral Palsy in Europe (SCPE). (2000)
Stopper-motherswere younger than non-stoppers (95% CI
Surveillance of Cerebral Palsy in Europe: a collaboration of
42 to 45y vs 45 to 5 9 respectively) at the time of the survey. cerebral palsy surveys and registers.Dev Med ChildNeuroI
Stoppage was declared more frequently when a second born 42:8 1 6 8 2 4 .
was affected (p<O.OOl). Birth control initiatives were also
associated with having more often a higher school degree,
religious, and moral convictions. Discussing the child’s rest-
lessness, repetitive behaviours, and anxiety level with a pro- 120 PARKINSON
fessional, and ‘other-than-TSfeatures’ in fathers were other Self-reported quality of life in children with
associations. Externalizing behavioural manifestations only cerebral palsy: a European population-based
were associated with stoppage in this sample. study
Conclusions:After TSC diagnosis the emergence of changes KATHRYN PARK INS ON^, ALLAN COLVER~,HEATHER DICK INS ON^,
in reproductive attitudes is clear. Further analysis will take ULRIKE RAVENS-SIEBERER~ ON BEHALF OF SPARCLE GROUP
into account their multiple sources and whether declared aUniuersityof Newcastle Centrefor Health Services
decisions correspond to actual reproductive behaviours. Research,Newcastle, UK; bQuality of Life C Child and
Adolescent Health, Robert-Koch-Institute,Berlin, Germany

Objectiue: To describe the quality of life of 8 to 12 year-old


119 PARKES children with cerebral palsy (CP) across eight centres in
Multiple impairments in children with Europe as reported by the children themselves.
cerebral palsy in Europe Methods: The Study of PARticipation of Children with cere-
JACKlE PARKEQ, ElTHNE MCLAUGHLIN O N BEHALF OF THE bral palsy Living in Europe (SPARCLE) incorporates assess-
SURVEILLANCE OF CEREBRAL PALSY IN EUROPE (SCPE) ment of the children’s subjective quality of life. Children
COLLABORATNE GROUP aged 8 to 12 years complete a recently developed generic
Queen’sUniuersity,Becfast, LJK questionnaire measure of quality of life - KIDSCREEN with
the following domain structure: physical well-being, psycho-
Objectiue: To describe the prevalence of combinations of mul- logical well-being, moods and emotions, self-perception,
tiple impairments in the childhood population of children autonomy, parent relations and home life, financial resources,
with cerebral palsy (CP) in Europe and discuss implicationsfor social support and peers, school environment, and social
health services. acceptance. The sample was drawn from population registers
Method: The standardized dataset of surveillance of CP in of children with CP held by nine centres in Denmark, France,
Europe’ was analyzed. There were 5036 individuals with CP Germany, Ireland, Italy, Sweden, and the UK. Depending on
born between 1975and 1990,alive and resident in the area at numbers, centres included either the whole eligible popula-
age five. Multiple impairment was defined as a motor impair- tion or a random sample stratified by walking ability Data are
ment plus one or more associated impairments (IQC70; reported on those children with sufficient cognitive ability to
vision <6/24 better eye; hearing >20dB loss; ever had complete KIDSCREEN. Demographicinformation was collect-
seizures) and severe multiple impairment such as no walking ed from parents.
plus one or more severe associated impairments (IQC50; Results: Data will be reported on about 500 children. We will
vision <6/60 better eye; hearing >70dB hearing loss; active show how we verified the properties of KIDSCREEN in a
seizures). Descriptive statistics,bivariate analysis, and multi- group of children with disabilities. Preliminary analysis based
variate general log linear modelling was used to identify on 296 children shows that Cronbach’s alpha is satisfactory

45
for all domains (range=0.70 to 0.84)except for self-percep- 122 PELC
tion which is lower at 0.55. We will present descriptive statis- Recognition of emotional facial expression
tics of KIDSCREEN scores comparing children between the and interpersonal relationships in ADHD
centres and relating the scores to children's characteristics K PELC", c KORNREICH~,M L F O I S Y ~ ,B DAN*
such as severity of impairment, age, sex, and type of school aDepartrnentofNeurology, Hbpital Universitaire des
attended, as well as the family's socioeconomic status. Enfants, Reine Fabiola, Free University of Brussels (Urn),
Conclusions:KIDSCREEN had not been used before on a dis- Brussels;bInstitute of Psycbiatty, CHUB, ULB, Brussels,
abled population and the data collected by SPARCLE show it Belgium
is a suitable questionnaire to measure the quality of life of
children with disabilities. Introduction: Impaired interpersonal relationships in atten-
tiondeficit-hyperactivity disorder (ADHD) have been well
Acknowledgement:Supported by the European Commission Research documented. They have been hypothesized to be secondary to
Framework 5, Grant number QLG5-CT-2002-00636. impairment of receptive nonverbal language. Recognition of
emotionalfacial expressions is an important aspect ofreceptive
nonverbal language and it has been shown to be central to the
121 PECINI organization of emotional and social behavior.
Language reorganization after early-onset Method:We studied identification of facial expression of four
brain focal lesion: a functional magnetic emotions (joy, anger, disgust, and sadness) in a group of 30
resonance imaging study children aged 7 to 12 years who meet the DSM-IV criteria for
C PECINI, L BIAGI, M TOSETTI, D BRIZZOWRA, A CHILOSI, ADHD and who have no comorbid learning disability, specif-
P CIPRIANI, G CIONl ic learning difficulties, developmental coordination disor-
Department of Developmental Neuroscience, Stella Maris der, pervasive developmental disorders, conduct disorder,
Scientific Institute, Pisa, Italy bipolar disorder, or substance abuse, and 30 matched unim-
paired controls. The test we used included 16validated pho-
Objective: Language reorganization after early-onset lesions tographs depicting these emotions in varying intensities
has been recentlyinvestigatedby means offunctional magnetic constructed by morphing.
resonance imaging (fMRl). Early left hemisphere lesions are Results: Children with ADHD showed a general deficit in
usually associated with enhanced activationof frontal and tem- decoding emotional facial expressions with specific deficit in
poral areas of the right hemisphere, whereas recruitment of identifying anger and sadness. Self-ratingof the task ditficulty
regions ipsilateral to the lesion is limited.With the specificaim revealed a lack of awareness of decoding errors in the ADHD
of investigatingthe relationship between lesion characteristics group compared with controls. Within the ADHD group
and language lateralization at MRI, we conducted an fMRI there was a significant correlation between interpersonal
study on a group of participants with congenital or early problems and emotional facial expression decoding impair-
acquired focal lesions. ment which was more marked for anger expressions.
Methods: Eleven Italian patients with early-onset ( < 3 years) Conclusion: These results suggest suboptimal nonverbal
brain focal lesions, one in the right and 10 in the left hemi- decoding abilities in ADHD which may have important impli-
sphere, performed phonological tasks (rhyme generation cations for the remediation of social skill deficits in children
and/or a rhyme and letter judgement) during MRI acquisition. with disorder.
BOLD responses were acquired by 1.5T General ElectricSigna
Horizon IX System (GE Milwaukee USA) equipped with Echo-
speed gradient coil and amplifier hardware. BOLD maps were
generated by using BRAIN VOYEGER. 123 PELILLO
Results: Patients with postterm brain injury of the left hemi- Histopathological alterations of collagen
sphere presented a peri-lesional reorganization of language structure in tendons of quadriplegic and
areas that was associated with some reading and/or phono- diplegic children: preliminary study
logical processing difficulties.Patients with at-term lesions in F PELILLO", F GRIZZI", M MORI", N GAGLIANOB, 6 FRANCESCHINI",
the left hemisphere involving the cortical-subcorticalstruc- N PORTINARO
tures activated cortical regions of the right hemisphere which aHumunitasClinical Institute, ViaManzoni 56,Ronano,
were homotopic to the left language areas. These participants Milan; bDepartment of Human Morphology, University of
did not show any consistent language deficits. Patients with a Milan, Milan, Italy
pre-term lesion in the periventricular white matter preserved
the activation of the left language areas in cases of small Introduction: Cerebral palsy has a variety of clinical patterns
lesions, whereas a shift to the right hemisphere was observed that make each case different in clinical practice. Collagen
when the lesion was larger and presumably involved the structure was studied to evaluate the difference in the histo-
white matter fibres connecting anterior and posterior areas. pathology of tendons in children with quadriplegia and
All these patients showed normal language skills. diplegia.
Conclusion: Language reorganization after early focal lesion Materials and methods: Samples were taken from hamstrings
seems to depend on timing and size of the lesion. Moreover, in nine children with diplegia and 13children with quadripleg-
earlier lesions tend to be associated with a better language ia during programmed hamstring lengthening after ethical
outcome. approval was obtained. Samples were prepared in sections
stained with Ematoxilin-Eosin,PicraSirius, and Orcein. Then
sections were digitized using a computer-aidedimage analysis

46 EACDAbstracts2005
system, and morphometric analysis was performed as well as Conclusion: Children with brain tumours have significantly
an immunohistochemicalstudy on collagen type I, 11,and IV in reduced HRQL, 1 and 6 months after diagnosis. Possible
the two groups. determinants include psychological status of the child, family
Results: Although preliminary, the present study has shown support, and family stress levels.
significant differences in cellular and collagen distribution in
elastic fibres, and the amount and pattern of distribution
between diplegic and quadriplegic children.
Conclusion: The presence of a typical histopathological pat- 125 PETROVA-STOYANOVA
tern in tendons in the diplegic and quadriplegic patient may Loneliness in the families of children with
correlate with the clinical features and needs of the two rare disease
groups. It also may be helpful to draw a correct diagnostic G PETROVA-STOMNOVA", A SHOP OVA^, I OUZUNOVAC',, s
algorithm to determine the best therapeutic strategyfor each SHOPOVA", IL KALEV",E SIMEONOV", I HRISTOZOVA~
kind of patient, according to the different tissue mechanical aUniversity Hospital, Alexandrovska Pediatric Clinic,
properties. Sofia; bMedicalUniversity, Sofia; COncohematology
Department, University Hospital, TzaritzaJoanna, Sofia,
Bulgaria

124 PENN Having a child with an uncommon disease presents manydif-


Health-related quality of life 1 and 6 months ficulties. The parents have to find strength not only to
after diagnosis in children with brain tumours encourage their child to never lose faith, but at the same time
and its relationship to child and family variables they have to explain the nature of the disease, how it affects
SHORT MAN^, S P LOW IS"-^, RJ M C C A R T E R ~ ,
A P E N N " ' ~ , ~R, the quality of life of the family, and explain what the doctor
M STEVENS, A L CUR RAN^, P M SHARPLES"*~ can do.
aBristolRoyalHospitalfor Children, Bristol; bFrenchay The authors present their results from a longitudinal 3-year
Hospital, Bristol, UK CIJniversityof the Witwatersrand, study for coping strategies in 70 families separated into two
SouthAfrica groups, who faced rare diseases of high social impact such as
lisosomal storage diseases (Gaucher Fabry MPS), osteogenesis
Objectives: (1) To investigate health-related quality of life imperfecta, cystic fibrosis, blood clotting disorders, asthma,
(HRQL) in patients with brain tumours 1 and 6 months after lymphomas,leukemia, and other malignancies.
diagnosis.(2) Relate HRQL to child and familyvariables. Methods: Anxiety scale attitude questionnaire.
Metbods: Longitudinal prospective study of children with Conclusions: Families in the first group (rare diseases) have
brain tumours and normal matched controls. HRQL was mea- more difficulty coping with the disease, and they feel neglect-
sured using parental report Pediatric Quality of Life Inventory ed and isolated from society. The second group (socially
OpedsQL), cognition using WISC-111, and WPPSI, psychological acceptable diseases) receives better support from society
outcome using Birleson Depression Scale (BDS), Revised and they attempt to save their children's life at any cost, irre-
Children's Manifest Anxiety Scale (RCMAS), and Impact of spective of its quality.
Events Scale (IES). Primary carer's emotionalhealth using Beck
Depression and Anxiety Inventories (BDI-I1 BAI), familial
stress, family functioning, and family support using the Impact
on Family Scale (IFS), Family Assessment Device (FAD)and
126 PORTINARO
Family Support Scale (FSS), and coping strategy using the
The complex dynamics of infantile cerebral
Coping Health Inventory for Parents (CHIP). palsy
N PORTINARO, F GRIZZI, F PELILLO, M MORI
Results: Thirty-six patients with tumours and 31 matched
Humanitas Clinical Institute, Via Manzoni 56,Rozzano,
controls, and 23 patients with tumours and 18matched con-
Milan, Italy
trols, completed T1 and T6 data respectively. Mean age at
T1 was 9 years 1 month and range 2 to 16 years 7 months. '
Introduction: Cerebral palsy (CP) is a dynamic disease, dis-
Patients with turnours' scores were significantly reduced for
continuous in space and time, that advances through qualita-
PedsQL, total, and psychosocial summary scores, and all four
tively different states. The aim of the present study is to
domains (physical, emotional, social, school) compared to
develop a mathematical computer-aided model in order to
matched controls at T1 (all p<O.OOl) and T6 (aU ~ ~ 0 . 0 5 ) .
investigate the clinical evolution of the disease.
There was no relationship between PedsQL total score and
Methods: A computer-aided model was developed using the
age, sex, verbal, or performance IQ at T1 or T6 (allp>0.05).
mathematical principles of 'chaos' and 'catastrophe' theories.
There was a significant relationship between PedsQL total
The model was based on data taken from prototypical cases
score and RCMAS (r=-0.55 p=0.009) and BDS (r=-0.68
of diplegia characterized by different clinical conditions.
p=O.OOl) at T1 but not at T6. There was a significant relation-
Results: The developed model is valuable for discriminating
ship between the PedsQL total score and IFS at T1 (r=-0.54
and classifying the heterogeneous forms of infantile CP, and
p=O.OOl) and T6 (r=-0.42p=0.022), between PedsQL total
also in identifying the so-called 'catastrophe points'. These ref-
score and FSS Social Organisations at T1 (r=0.42 p=O.Ol)
erence points enable the clinician to understand better the
and T6 (r=0.51p=0.016), andFSSTotallevelofsupportatT6
transition points occurring between two successive path-
(r=0.47 p=0.027). There was no correlation between the
ological states.
PedsQL total score and IES, BDI, BAI, CHIP, or FAD (all
Conclusions: The model we present simulates the complex
p>0.05).

47
behaviour of CI? It may be of help to plan therapeutic and 128 PULLAPERUMA
surgery strategies to organize clinical and molecular acquired Functional regression due to a non
knowledge. The detection of irreversible transitions may be neurological cause
also useful in anticipatingsurgical treatment and reducing the SUNIL PULLAPERUMA, DIANE SMYTH
follow-uprehabilitation programme. The description of CP as St Mary's Hospital, Praed Street, London, UK
a complex dynamical system may be helpful in estimating the
influence of exogenous factors (prenatal, perinatal, and post- Functional regression is a common referral to neurology/
natal) on its duration and intensity. neurodisability services for investigation and management.
We present two children with severe nutritional rickets referred
for investigations for possible neurological regression.
12'7POUNTNEY Child 1: A normal developing 9-year old male gradually lost
To assess the effectiveness of postural the use of his feet over a six-month period and is now using a
management programmes in reducing hip wheelchair for his mobility. He did not have cognitive impair-
dislocation in children with bilateral cerebral ment but his mother died from spinalTB.His general practition-
palsy er (GP) referred him for a neurological assessment. He did
T E POUNTNEY, EM GREEN, A MANDY not have neurological signs although he showed clear evi-
ChaileyHeritage Clinical Services,East Sussex, UK dence of clinical nutritional rickets, with several fractures
and muscle weakness. He is currently in rehabilitation.
Introduction: Hip subluxation/dislocation occurs in 60% of Child 2: A 17-year old female with learning difficulties and
childrenwith cerebral palsy (CP) who are not waking at 5 years epilepsy has been followed up by our Epilepsy Service. Her
of age. It has a debilitatingeffect on function, pain levels,ability brother also has a similar disability. Her epilepsy is currently
to sit, and personal care. Management has been largely by sur- well controlled with Epilim. Over the past 12 months her
gical interventions and focused on single hip outcomes. father, a surgeon, recognized that she had progressive genu
Wenty-four hour postural management encourages active valgum. The GP referred her for an orthopaedic assessment and
movement maintenance of muscle length and increased func- she was awaiting surgical intervention. Her father disagreed
tion. A retrospectivestudy found that provision of positioning with this decision and requested that the GP assess her bone
equipment before subluxation improved outcomes in terms of functions. She suffered stiffness over the lower limb muscles
hip migration. as well as pain around the knee joints, however she did not
Objective: This study aims to detefmine whether the early have neurological signs. Bone function results revealed a
introduction of postural management equipment can reduce diagnosis of nutritional rickets.
levels of hip subluxation at 5 years compared with an historical Conclusion:Although children are referred for neurological
control group. assessment for functional deterioration, general paediatric
Design: Prospective longitudinal cohort study. illnesses are a common finding in a multicultural childhood
Partidpants: Forty-nine children with bilateral CP of 18 population and nutritional impairment for neurological/
months of age or less were recruited h m the south-east of functional regression should not be forgotten.
England. Postural management equipment was prescribed
accordingto clinical need. Childrenwere reviewed at 3- month
intervalsuntil they reached the age of 5 years.
Outcomemeasures:X-ray measures of migration percentage at 129 RAMECKERS
30 and 60 months; status of both hips, i.e. both hips safe, one Short-term effects of botulinum toxin A on
hip subluxed, both hips subluxed; Chailey Levels ofAbility, and goal-directed movement tasks with the affected
use of equipment. hand in children with spastic hemiplegia
Data analysfs: Student's t-test to compare levels of hip migra- E A A RAMECKERS*.'*"*~, B C M S M I T S - E N G E L S M A N ~ ~ ~ ,
tion in the current cohort at 30 and 60 months using the most L A W M S P E T H ~ J~ S~ H, V L E S ~ J, D U Y S E N S ~
migrated hip with an historical control group. aRehabiNtationFoundation Limburg (SRLj, location
Regression analysis to determine factors influencing hip Franciscusoord Valkenburg,the Netherlands; bMotor
migration, including Chailey Levels of Ability, distribution of Control laboratory, Department ofKinesiology,m,
CE: migration percentage, and equipment use. Leuven, Belgium; cAcademicHospital Maastricht ( M j ,
Preliminary results: Thirty-five per cent (cf 60%) of children the Netherlands; dDepartment of Rehabilitation, Radboud,
had one or both hips subluxed; significantly more children had University of Nijmegen; eAvans+ Universityfor
both hips safe who used a lying support at night; no sigtuficant Professionals, Breda, the Netherlands
differencewas found between most migrated hips.
Conclusion: liventy-four hour postural management pro- Spasticity muscle weakness and contractures will cause less
grammes have an important role to play in the prevention of efficient arm movements in children with cerebral palsy
hip subluxation in CI? (CP). Botulinum Toxin A (BTX-A) injections in the spastic
arm muscles are effective in decreasing spasticity. N o long
term positive or negative effects in functional tasks have been
shown after using BTX-A in the upper limb of children with
CI? The aim of the present study is to investigate the short-
term (2 weeks) effect of BTX-A on the kinematics of goal-
directed movements with different target sizes and dis-
tances. Wenty-two children with spastic hemiplegia (5 to 16

48 EACDAbstracts2005
years of age) were tested. Children were matched for age and programmes can lead to significant improvements in peak
Zancolli classification, and then randomly assigned to the force production of the upper limb.
treatment in two groups. Eleven children received BTX-A.
Participants held a special puppet (pen) and were asked to Acknowledgments: The authors would like to acknowledge The
Cerebral Palsy Association of Western Australia (Ltd) and Therapy
make discrete or continous shift movements in four condi- Focus for their support and endorsement of this researchprogramme.
tions (target size, small and large, distance short, and long).
All tasks were counterbalanced across participants. The fol- Reference
1. Wiley ME,Damiano DL. (1998) Lower-extremitystrength profiles
lowing kinematic variables were measured: reaction time in spastic cerebral palsy.Dev Med CbfldNeurol40: 100-107.
(RT), movement time (MT), target hits, and errors and Index
of performance (IP). The spasticity (Ashworth), active and
passive mobility ofwrist and elbow, and the maximum volun-
tary contraction (MVC) of the finger flexors were measured. 131 RIPPE
live weeks after BTX-A, no significant difference in Ash- Complications of intrathecal baclofen pump
worth scores was found. MVC did show a significant effect therapy for severe hypertonia in children: a
after BTX-A as result of the amount of decrease of MVC in the long-term follow-up review of 785 patients
BTX-A SOUP. from four centres
D M RIPPE", B TANN", D J GAEBLER-SPIRA~,L E KRACH', J G O O C H ~ ,
E DABROWSKID
130 REID aRehabilitationInstitute of Chicago, Chicago,Illinois;
Differences in upper limb concentric and bGilletteChildren'sSpecialty Health Care, St Paul,
eccentric peak muscle force in adolescents Minnesota; CUniversityof UtahHospitals, Salt Lake City,
with and without cerebral palsy Utah; dChildren'sHospital of Michigan, Michigan, Detroit
SIOBHAN REID", PETER HAMER', JACQUELINE ALDER SON^, USA
DAVID LLOYD"
ai%e University of WesternAustralia, Perth; University Objective: To study the long-term complications associated
ofNotre Dame Australia, Perth,Australia with chronic intrathecal baclofen therapy in children with
severe intractable hypertonia of cerebral or spinal origin.
Objectiue: Quantify the difference in upper limb peak force Methods: Medical records of children with severe intractable
of young people with and without cerebral palsy (CP), and hypertonia from four pediatric rehabilitation medicine clinics
the changes in force production following an eccentric train- were reviewed.A total of 785 children with hypertonia of cere-
ing programme for adolescents with CE bral or spinal origin due to cerebral palsy, traumatic brain
Rzrticfpnts: Fourteen adolescents with hemiplegia aged injury, or spinal cord injury, received intrathecal baclofen
between 10 to 15 years (mean 1 l y 3m0, SD ly) were matched pumps for the management of hypertonia. The medical
to 14 adolescents with no known neurological condition. records of all children followed at each centre were reviewed
Each group was made up of six males and eight females. for complications.
Materlals/methods: Participants with CP completed a six- Results: Complications associated with intrathecal baclofen
week home-based eccentric training programme. The train- pump use including catheter complications (disconnections,
ing protocol was graduated whereby training load was obstructions, migrations, tears, and fractures), infections,
progressively increased over the course of the programme. pump failures,pump migrations,other pump problems requir-
Strength assessments were completed at three weekly inter- ing replacement of the pump, and deaths were recorded.
vals using a Biodex dynamometer. The strength assessment Catheter-relatedcomplications (disconnections, obstructions,
targeted the elbow flexor group and incorporated a series of migrations, tears, and fractures) were the most common with
isokinetic concentric trials requiring three trials each at 30°, 264 total catheter complications. Infection at the pump site
60°,and 9O0/secfollowed by three eccentric trials at 30"/sec. occurred 68 times and replacement of the pump due to pump
Main results: Significant differences existed at baseline for failure or pump migration 17 times. A total of 25 deaths occ-
peak eccentricforce between participants with and without CP urred across four centres in 785 patients (mortality 3.2%)
(F[2261 = 16.764,p<0.05).Across all conditions young people Conclusions:Although intrathecal baclofen therapy has been
without CP, produced approximatelytwice the amount of force shown to improve function, increase comfort, and increase
(m=39.3, SD 11.8) compared with young people with CP ease of care provision in patients with severe hypertonia of
(m=22.1, SD 7.5). Following the eccentric training prog- cerebral or spinal origin, there are significant risks associated
ramme, adolescentswith CP made significantimprovementsin with this treatment. Further study is warranted to better define
peak eccentric force (F[1271 =8.282,pc0.05).This improve- the complications so that points of intervention can be identi-
ment translated as no difference being found between partici- fied to decrease complications associated with intrathecal
pants with and without CP following the training programme baclofen therapy as well as to decrease the additional morbidi-
on the eccentriccondition (F[226]=3.717,p>0.05). ty and mortality resulting from repeated surgery to correct the
Conclusions: Disproportional muscle weakness can lead to complications. Additional research is also needed to better
functional limitations in children with CP Wdey and Damiano examine deaths in children receiving intrathecal baclofen ther-
(1998) reported that children with CP are significantlyweaker apy to see if they are at an increased rate from a comparable
in all muscle groups of the lower limb as compared with their population and how to intervene if possible.
normally developing peers. This data demonstrates a similar
magnitude ofweakness in the elbow flexorsof the upper limb.
These findings ako suggest that short-term eccentric training

49
132 ROEBROECK other aspects ofcognition and higher cerebral function (alexia,
Lost in transition? Unmet needs and agraphia, apraxia, agnosia, amusia). The complex relationship
implementation of special rehabilitation teams between cerebral dominance and hand preference was noted
for young adults with disabilities early on and has fascinated scholars ever since.
M E R O E B R O E C K ~ ,c~ , NIEUWENHUIJSEN~,
M DONKERVOORT~, It was the American neurologist Samuel Torrey Orton who
D J H G W I E G E R I N K ~ SJ ~VAN
, M E E T E R E N ~H, J STAM" in the 1930s first brought to light evidence for anomalous
aErasmusMC, UniversityMedical Centre,Rotterdam ; cerebral hemispheric dominance in certain children experi-
blf.ansitionResearch Group,Rehabilitation Centres region, encing difficulty learning to read, suggesting that dyslexia is
South-westNetherlands;CRijndamRehabilitation Centre, basically a language problem. His theories aroused contro-
Rotterdam. the Netherlands versy and fell into disfavour during the post-Second World
War period but they were later vindicated by subsequent
Objective: In adolescents and young adults with disabling research showing the importance of the speech code, a
conditions from childhood, the continuity of care is dis- major temporal lobe function to the phonetic analysis
turbed after discharge from paediatric rehabilitation. This required for acquisition of reading skills. This was followed in
project aimed ( 1 ) to measure unmet needs of young adults the 1970s and 1980s by numerous studies showing anatomi-
with congenital disorders in their transition into adulthood cal variations, both gross and microscopic, as well as differ-
and ( 2 ) to implement special rehabilitation teams for young ences in electrical activity in the brains of dyslexic children as
adults with a chronic disorder. compared with typical readers. Measures of cerebral blood
Methods: We summarize results of recent studies on unmet flow have shown hemispheric asymmetries in children with
needs of the Dutch Transition Research programme and Adol- attention deficits known to be comorbid with dyslexia.
escents with Spina Bifida in the Netherlands (ASPINE), add- The relevance of hemispheric specialization has been
ressing persons with a variety of disabilities (n=35), cerebral called into question in recent years by research showing that
palsy (CP; 107, and spina bifida (n=178).Indicated unmet behaviours related to learning history can often account for
needs are categorized into transition profiles addressing rele- variations previously ascribed to physiological asymmetries.
vant domains of participation. Based on these transition pro- However, newer studies using functional magnetic reso-
files a modular rehabilitationprogramme is implemented. nance have yielded useful information. It has been estab-
Results: The studies provide an unequivocal picture of unmet lished for example that ( 1 ) typically developing children
needs. Young adults emphasize their need for more informa- show localized activity in the left temporal lobe in response
tion on the diagnosis and its long-term consequences to spoken language challenges; (2) that left-handed persons
(6540% of the adults), addressing functional mobility out- differ from righthanders in the degree of lateralization of
doors (5&70%), and the need for active recreation including activity during language tasks; (3) that dyslexic children dif-
sports and social activities (35-55%). Also frequently men- fer from typically developing children in the degree of
tioned is the need for advanced education work, household employment of certain higher-order centres in reading; and
management, and personal care (25-55%). The majority of (4) that dyslexic participants show a change in localization of
young adults with CP aged 18-22 years (60%)did not visit a brain activity as a result of therapy. These findings lend
rehabilitation physician in the past year. renewed support to many of the classical concepts derived
To address these unmet needs we recently started special from correlation of functional deficit with anatomical lesion.
rehabilitation teams for young adults. These teams offer a
modular rehabilitation programme focussing on the indicat-
ed domains of participation. The aim is to support young
people with disabilities in reaching a higher level of indepen- 134 RUTAR
dence and self-efficacy in adult life. Assessment management and prevention of
Conclusions: There is a poor handover from paediatric to pain in neonates and infants
adult services. Young adults with disabilities indicate unmet T JAW, v RUTAP,D NEUBAUEF~A~
needs regarding participation in several domains. Special aMedicalFaculty, Universityof Ljuboana, bDepartmentof
rehabilitation teams for young adults with disabilities may fill Child,Adolescent C Developmatal Neurology, University
this gap. Children's Hospital,Ljuboam, Slovenia

Objective: Pain assessment and evaluation in neonates and


infants remains a significant problem while its management
133 ROSENBERGER and prevention in this age group are not optimal. Recent
Hemispheric specialization and dyslexia research has shown the efficacy of sugar solutions and topical
PETER B ROSENBERGER anaestheticsfor relief of procedural pain. Some of the neonatal
MassachusettsGeneralHospital, USA pain assessment tools are easily applicable in clinical settings
and may provide more objective evaluation of pain response,
The concept that one of the two hemispheres of the brain the development of pain management care plans, and the
might be responsible for a particular human activity first arose effectivenessof pain relief interventions.
in the mid-nineteenth century.Observations revealed that peo- The objectives of this study were to assess the applicability
ple suffering destructive lesions of the left hemisphere were at of the Neonatal Infant Pain Scale (NIPS) and to evaluate effec-
risk of losing the faculty of language. An entire science devel- tive and safe interventions that relieve pain in neonates and
oped to describe syndromes of functional deficit related to infants. Hypotheses were: ( 1 ) NIPS is applicable in clinical set-
focal brain lesions first for language (aphasia) and then for tings, ( 2 ) oral glucose solution, and (3) application of topical

50 EACD Abstracts2005
anaestheticsare effective in reducing pain-related behaviour in cognitivebehavioural activity performance between all three
neonates and infants undergoing minor invasive procedures. groups. Activity performance was highest among the typical
Methods: Sixty neonates and infants undergoing venepunc- children and was the lowest in the SC group. (3) Participation
ture or intravenous catheter insertion were randomly assigned and physical, as well as cognitive/behavioural activity perfor-
to one of three treatment groups: no treatment, oral glucose mance, significantlycorrelated in FI and SC groups. (4) In both
solution,and topical anaesthetic (EMU). FI and SC groups, more extensive physical task supports were
Statistical analysis: Internal consistency of NIPS was estab- provided than cognitivebehavioural task supports, and more
lished by Cronbach’salphas; interrater reliability of NIPS was extensive assistance was provided than adaptations. (5) Correl-
established by Pearson’s correlations; and concurrent validity ationswere measured between tasksupports and participation
was established by correlations with heart rate. DifTerences among students with CE (6) Physical activity performance is a
between group median pain scores were established by analy- significantpredictor for participation in the inclusivegroups.
sis of variance. Conclusions: These findings suggest that d8erences exist
Results: Internal consistency was high as Cronbach’s alphas between the participation and activity performance of children
ranged from 0.87to 0.91. Interrater reliabilitywasalso high as with CP and typical children, as well as between students with
Pearson’s correlations ranged from 0.86 to 0.94. Concurrent CP accordingto their educationalplacement, and highlight the
validity was medium high as Pearson’s correlations were 0.38 gap between the concept of inclusion and the practical societal
to 0.48.Differences between treatment groups were apparent limitations of schools to enable full participation of students
but did not reach statisticalsignificance. with physical disabilities. Moreover the relationship between
Conclusion:Results of the study confirmed our first hypothe- participation, activity, performance, and task supports stress
sis. The study of differences between the three treatment the importance of multi-dimentional intervention designed to
groups indicate the beneficial effect of glucose solution but do remove existingbarriers within the mainstream environment.
not confirm the second hypothesis, and reveal inefficacyof top-
ical anaesthetic and thus disproves the third hypothesis. Acknowledgements: This paper is based on the doctoral research of
the author being conducted through the auspices of the School of
Occupational Therapy, Faculty of Medicine, Hadassah Hospital-
Hebrew University, Jerusalem,Israel. This research was funded by
the Israel Ministry of Education and by the Levin Center Fellowship,
135 SCHENKER Hebrew University,Jerusalem,Israel.
Relationship between participation activity,
performance assistance, and adaptations in
students with cerebral palsy mainstreamed in
elementary schools 136 SCHERTZ
8 SCHENKER, S PARUSHA Computerization and ontology in representing
School of Occupational Therapy, Faculty of Medicine, The knowledge on developmental disorders: a
Hebrew University,Jerusalem, Israel prototype using Developmental Coordination
Disorder
Objectfves:(1) To compare the level of participation activity, , Z U K ~ ,N A S B E H ~ ,M P E L E G ~
M SCHERTZ~L

performance, and task supports (assistance and adaptations) ahstitUtefor Child Development, Kupat Holim Meubedet
between mainstream students with cerebral palsy (CP) and Herzeliya; bDepartment of Statistics, University of Haifa,
typical classmates in elementary school settings. (2) To Haifa; ‘Department of Management Information Systems,
explore the relationship between participation, activity, per- University of Haifa, Haifa, Israel
formance, and task supports among mainstreamed students
with CE (3) To identify the activity and task supports variables Objective: Current representations of developmental disor-
which predict participation among mainstreamed students ders are in narrative non-computable form and are not stan-
with CE dardized. Use of an ontology entailing subscribing to explicit
M e t M : Participants were a sample of 248 elementary school structured definitions of medical terms and their relationships:
students (mean age 9y 8mo) within three study groups: (1) (1) supports computerized analysis and (2) advances agree-
One hundred students with primary diagnosis of CP fully ment on terminology among professionals. Our aim was to
included in regular schools (FI group); (2) Forty-eight students represent developmental disorders in the form of an ontology
with primary diagnosis of CP in self-containedclasses in inclu- that is clinically valid yet structured for use by machine-com-
sive schools (SC group); and (3) One hundred typical peers putable methods. As proof of concept we developed a proto-
matched by class and sex to the FI group. Students with CP type ontology using Developmental Coordination Disorder
showed heterogeneity in type and Gross Motor Function (DCD) as a representative disorder.
Classification System level. Methods: Using the Protege-2000 knowledge-modeling tool
A criterion-referenced assessment, The School Function we developed an ontology that connects three knowledge
Assessment (SFA), was used to measure the participation and representations of medical concepts: the Unified Medical
activity performance of educationally relevant functional Language System’sSemantic Network of the National Library
skills and the task supports provided to students with special of Medicine, DSM-N taxonomy of the American Psychiatric
needs within inclusive elementary school settings. Association, and the Nelson Textbook of Pediatrics. Relevant
Results: (1) Participation between all groups significantlydif- data from a literature review on DCD was entered into the
fered (p<O.Ol) with typical children achievingthe highest and ontology. We composed queries to examine and evaluate
the SC group the lowest levels of participation. (2) significant knowledge from the ontology. This exercise allowed us to vali-
differences (p<O.OOOl) were identified in both physical and date our ontology clinically and computation*. To assess

51
preliminary clinical validity 10 clinical experts in develop- European Commission, Research Framework 5, Grant number
mental disorders were sent printouts of the ontology for QLG5-CT-2002-00636.
review.
Results: An ontology representing the concept DCD was con- 138 SCHIRRIPA
structed using 44 classes, 80 slots, and 98 instances. We com- Involving young people with disabilities in
posed and successfullycompleted six queries (e.g. ‘Concepts theatre and drama: a measure of perceived
that can be both a risk factor and a cu-morbidityof a given dis- coping improvement
ease or syndrome’). Eight experts confumed the ontology ElORGIO MAURO SCHIRRIPA, ROMEO LIPPI, RAFFAELA MARCH1
structuringas valid and useful; two did not respond. UOC of Infant Neuropsycbiatry, ASL, ViterboViale Trento,
Conclusions: We have successfully created and validated a 18/h Viterbo,Italy
prototype of an ontology for developmental disorders. It
supports computerized analyses and should improve agree- Introduction: Starting in 1997 in the city of Viterbo, Italy
ment and communication among experts in the field. young people with and without disabilitieshave taken part in
Acknowledgements:A Ornoy MD, J Rutman MD, Y Sandhaus MD, D a theatre company that has given 1 2 performances to audi-
Lobel MD, L Gabis MD, B Glick MD, V Fatal-ValevskiMD, I Posener ences of 12000 people. The principles of the theatre project
MD, for examiningthe ontology are: understanding and reading the text using memory, orga-
nizing the performance, meeting with the public, and disre-
garding differences and disabilities.
137 SCHIRRIPA Objectives: To measure coping with the perceived effects of
Involving parents of children with disabilities in theatre experience.
socio-health information groups concerning Methods: Construction of a specific instrument - a close end
rights, regulations, and new proposals format questionnaire composed of 45 items - to assess the
GIORGIO SCHIRRIPA, GIUSEPPE TRAMONTANA, BARBARA CARAVALE improvement in nine important areas: imitation, cognition,
O N BEHALF OF SPARCLE GROUP memory, communication, self-management, self-control,
UOC of Infant Neuropsychiatry,ASL,ViterboViale Pento, self-respect,respect for others, and relationships.
18/bViterbo,Italy Participants: The 2004 theatre group (45 people with and
without disabilities) and 55 parents. Distribution of the ques-
Introduction:The main objective of the research project Study tionnaire to the parents and administration of the same ques-
of Participation of Children with Cerebral Palsy living in tionnaire to all the young actors.
Europe (SPARCLE) is to study the iduence of environmencd Results: Seventy-fiveper cent of young people and more than
factors on quality of life in children aged 8 to 12years with cere- 79 per cent of parents perceived an important increase in cop-
bral palsy (CP) in Europe. Within this project in 2004 the ing areas due to the theatre experience.
Health Department of Viterbo, Italy conducted a focus group Conclusions:Drama makes an important contribution to the
with five families. One strong finding of this focus group was healthy adjustment of some young people with and without
the need for better and increased information for the families disabilities. Should this approach be incorporated in future
of such children. neurorehabilitation?
Objective:To fully inform the families on assistance with the
social integration rights of their children and to ascertain
which socio-healthissues the parents might wish to examine
in depth. 139 SCHMID
Metbods: Groups of 6 to 12 parents of children with moderate Monitoring the maturation and development
and serious disability due to CP and other neuropsychiatric of postural strategies in children
pathologies were created according to the children’s age MAURIZIO S C H M I D ~ ,SILVIA CONFORTO“, MATTEO ROSSO’,
(0-6, 7-14, above 14y). Tbo social workers and one health LUISA LOPEZ’, TOMMASO D’ALESSIOA
professional ran each meeting and distributed four simple aAppliedElectronics,Engineering Universita degli Studi,
questionnaires containing 12 questions. The level of knowl- Rome; bCbildNeurology Unit, Universitd degli Studi di
edge of laws and regulations was assessed and types of infor- Roma, Tor Vergata,Rome, Italy
mation that familieswished to more about.
Results: Eighteen meetings were conducted with the partici- Objective:To investigate balance control mechanisms as they
pation of 162 families and 158questionnaires were collected. vary with the absence of visual input and develop in a school
Families living in the city were better informed about rights population rangingfrom 1st to 5th grade.
and regulations than those in rural areas. More than 60% of Methods: Signals were recorded during 60 trials adminis-
parents wished to better examine specific neurological issues tered on a sample population of 148 primary schoolchildren
and free time local activities for children with disabilities. stepping and then quietly standing on a force platform in
Conclusions:The meetings achieved a good level of partici- two different conditions: eyes closed and eyes open.
pation. Families that had been under the care of the health Posturographicparameters were extracted during the standing
department for some time participated more. The recent phase and an algorithm was implemented to identlfy the set-
emphasis of our health service on a family-centred approach tling time after stepping on the balance plate. The effect of dif-
is yielding positive results and reactions. ferent conditions on posturographic parameters was tested
with a two-way ANOVA (gradexvisual cue). A one-way ANOVA
Acknowledgement: We would like to thank the department, staff, was performed on correspondingeyes-closedkyes-openratios.
and families who participated in this project.It was supported by the Results: A large number of posturographic measures have

52 EACDAbstracts2005
been found to be sensitive to vision condition and some of sex, Body Mass Index, body fat). The results will be compared
them also to age and anthropometrical factors, although this with reference values ofthe normal population. In addition we
latter dependency does not explain all the data variability compare outcome in children with myelomeningocele with
with age. A clear change in postural strategy has been found associatedhydrochepalusand Chiari I1 malformationwith those
between 3rd and 5th grade. When the children are younger with lipomyelomeningocelewithout these abnormalities.
than 3rd grade their postural control with eyes closed relies Causal relations between muscle strength, level of every-
on major adjustments characterized by more ample oscilla- day physical activity, endurance, and self-perceived motor
tions and the children probably need to move to different competence will be calculated.
spots and remain on those until the next adjustment. After
that age our data would suggest that the child can apply
minor adjustments that happen over a smaller trajectory but
with a higher frequency and there is no need for big excur- 141 SCHOLTES
sions, although overall the path remains constant. The SPAT: a clinical spasticity assessment for
Conclusions: Simple measures extracted from posturo- children with a spastic paresis
graphic signals tended to be sensitive to condition and age: V A B SCHOLTES, AJ DALLMEIJER, J HARLAAR, J C BECHER
specially designed algorithms can assess and monitor age- Department of Rehabilitation Medicine, W University
dependent variations of strategy occurring during the devel- Medical Centre,Amsterdam, the Netherlands
opment of motor control.
In the most widely accepted definition, spasticity is charac-
terized as a velocitydependent increase in muscle tone during
passive stretch. Of all available clinical spasticity instruments
140 SCHOENMAKERS only the Tardieu Scale (TS) assesses spasticity by passively
Physical activity, exercise, capacity, and stretching the muscle at dserent velocities.Therefore the TS is
muscle strength in independent ambulating the only instrument acknowledging the velocity-dependent
children with lumbosacral spina bifida character. However, its assessment is timeconsuming. We sim-
M A G C SCHOENMAKE RS", P J M HELDERS", J HILLAERT, J W plified the TS to improve clinical application in children with
G O R T E R ~T
, TAKKEN~,UNIVERSITY CHILDREN'S HOSPITAL AND spastic paresis, resulting in a clinical assessment scale comply-
MEDICAL CENTER" ing with the concept of spasticity: the SPAsticityTest (SPAT).
aDepartmentof Pediatric, Physical Therapy,and Exercise With the SPAT the joint is passively stretched at two different
Physiology, Utrecht;bUniversityChildren'sHospital and velocities: 'very slow' to measure maximum ROM (Passive
Medical Centet;Department of Pediatric Rehabilitation Restricted Angle, PRA) and 'as fast as possible' (within one sec-
Medicine, Utrecht,the Netherlands ond) to measure the angle of stretch reflex elicitation (Stretch
Restricted Angle, SRA). The intensity of muscle reaction is grad-
Objective: In previous studies we investigated functional ed at both velocitieswith separatescores. In this studywe exam-
outcome in children with sacral level (lipo) myelomeningo- ined the intra-observer and inter-observer reliability of the SPAT.
cele. Although 89%of them were community walkers most Methods: liventy children with CP (mean age 9y 10mo) were
(90%)of them had balance problems with activities such as tested three times a day by two different observers with dif-
hopping into squares, jumping, and running. This might ferent levels of experience. In each session the PRA of five leg
interfere with regular sporting activities. muscles (hip-adductors, rectus femoris, hamstrings, gastroc-
In this study we want to investigate the causal link between nemius, and soleus) was assessed, followed by the SRA.
muscle strength, balance problems, endurance level of every- Results: Results indicated a good intrarater reliability of all
day and sporting activities, and self-perceived motor compe- PRA testing (ICC>0.80) and acceptable for SRA testing (ICC
tence in two groups of ambulant children with lumbosacral 0.67-0.85) except for soleus testing which was poor (ICC
level paralysis: myelomeningocele (MMC) versus lipomyelo- 0.46).The interrater reliability ranged from very poor in both
meningocele (LMMC). PRA and SRA testing for hamstrings and soleus muscles (ICC
Methods: From February to May 2005, a cross-sectionalstudy 0.46-0.52) to acceptable and good (ICC 0.61-0.87) in the
was carried out at the Spina Bifida outpatient clinic at the other muscles.
University Medical Center, Utrecht. Thirty three children met Conclusion:The SPAT has acceptable to good intrarater relia-
the inclusion criteria (lesion level below L4, IQ>80 aged bility for all leg muscles except soleus.Although the SPAT has a
between 6-lw, and being able to ambulate >500m). standardized testing protocol, development of a training pro-
Muscle strength is measured with a hand-held myometer gramme might be necessary to improve interrater reliability.
in upper and lower extremities. Endurance is measured with
the 6-minute walking test. Exercise capacity is measured using
a maximal exercise test on a treadmill and an expired gas
analysis system (VO, peak). Level of everyday physical activity 142 SCOTO
is measured with a diary. Self-perceivedmotor competence is Ocular onset in a patient with
measured with the Harter Self-PerceptionProfile for Children. Charcot-Marie-Tooth Type 1
Results: Currently, we are collecting all data. A sample of 17 M SCOTO, C ROMANO, S PERRINI, M TUMINO, C MICELI,
children with MMC and six children with LMMC participated M LOMBARDO, R FALSAPERW
in the study (mean age 1Oy 4m0, SD 3y 4m0, and 1Oy 7m0, Department of Pediatrics, University of Catania, Italy
SD 3y lmo respectively).
AU results will be corrected for known confounders (age, Charcot-Marie-Tooth (CMT) are a heterogeneous group of

53
neuropathies with onset during adolescence. CMT is subdi- no relationship between the normalized volume ofthe MG and
vided into several forms of neuropathies but the most com- the passive dorsiflexion range on the affected side (?=0.041,
mon is type 1. Moreover type 1 is classified into 1A and 1B p=0.47).
which share the demyelinated component but are differenti- Discussion: The management of children with SHCP may
ated in protein deficiency, peripheral-myelin protein (PMP involve interventions to the gastrocnemius (surgery, botu-
22), duplication for CMT lA, and protein 0 (PO) deficit for linum toxin injection, etc.) but we have little knowledge of
CMT 1B.The PO gene is localized on chromosome 1. the muscular substrate or its response to treatment.
We report an unusual CMT 1Bwith early ocular onset in a Clinicians infer something of the status of muscles from the
patient of 20 months of age. Physical examination of the range of motion examination and select treatments on the
patient showed gait disorder, marked general hypotonia, basis of their measurements. While children with SCHP have
areflexia to lower and upper limbs, and proximal weakness. plantarflexion contractures and reduced MG volumes on the
The ocular investigation has demonstrated a horizontal nys- affected side, muscle size and dorsiflexion range are not relat-
tagmus and rarefaction of retinal pigment. The electrophysi- ed. Assessment of muscle volume may be important in opti-
ological study showed a marked reduction in sensory and mizing treatment selection in SHCE
motor conduction velocity. Motor nerve conduction studies
on median nerve showed a conduction velocity (CV) of
1 9 d s and the sensory nerve conduction of 21m/s. Brain and
cord MRI are within normal limits. Sural nerve biopsy has 144 SHORTMAN
been denied from both parents. Cognitive outcomes and academic
The spectrum of CMT phenotype that is associated with achievement in children with brain tumours
mutation in the PO gene is wide and ranges from congenital early after diagnosis
hypomyelinating neuropathy to axonal neuropathy. The maj- ROBERT SHORTMAN", RENEE MCCARTER", ANTHONY PENN"+'.~,
ority of mutations have been found in the exon 2 and 3 that STEVE LOW IS^, MIKE STEVENS M D ~ ANTHONY
, L CUR RAN^,
involves the extracellular portion of PO.The exon 4 of PO gene PETA M SHARPLES"'~
was shown to have a dominant negative effect on the wild type aFrenchay Hospital, Bristol; bBristolRoyalHospitalfor
protein. Children, Bristol, UK cUniversityof the Witwatersrand
This case represents the usual form of CMTlB regarding Johannesburg, South Africa
the age onset of congenital neuropathy, but is atypical for the
ocular involvement. Introduction: Brain tumours are the second most common
childhood malignancy. There is evidence that cognitive func-
tion is reduced in long-term brain tumour survivors but few
data exist concerning early outcome. The relative contribu-
143 SHORTLAND tions of tumour surgery and radiotherapy/chemotherapy to
The volume of the medial gastrocnemius in cognitive outcome remain to be defined.
children with spastic hemiplegic cerebral palsy Aim: To measure cognitive outcome in children with brain
A P SHORTL4N D, R MAWUYA, N R FRY, A E MCNEE, L C EVE, tumours one month after diagnosis.
M GOUGH Methods: Longitudinal prospective study of children with
One Small Step Gait Laboratory, Guy's Hospital, London brain tumours admitted to the Regional Neuroscience
UK Centre compared with normally developing children
matched for age, sex, and socioeconomic status. Intellectual
Objective: To compare the volume of the medial gastrocne- outcome was assessed using the Wechsler Intelligence Scale
mius (MG) in children with spastic hemiplegic cerebral palsy for Children (WISC 111 UK), the Wechsler Primary, and
(SHCP) with plantarflexion contractures, and in normally- Preschool Scale of Intelligence - Revised. Attention was
developing (ND) children. assessed using the Test of Everyday Attention (TEA-Ch).
M e t W : Thirteen ambulant children with SHCP (mean age 7y Memory was assessed using the Children's Memory Scales
5m0, range 4y lOmo to 12y 5mo) and 11ND children (mean (CMS). Academic status was assessed using the Wechsler
age 8y lOmo, range 4y 5mo to 13y 5mo) were recruited. Quicktest.
Children lay prone on a plinth with their ankles hanging over Results: 'kenty four tumour patients and 24 age-matched
the end. We used a three dimensional (3D) ultrasound sys- controls have been studied to date. The mean patient age
tem to scan the gastrocnemius musculotendinous units of was 10years 6 months, range 3 years 11months to 16years 8
both lower limbs with the ankle at maximum dorsiflexion. months, and 12 were males and 12 were females. There were
From the 3D dataset we measured the volume of the MG significant differences between tumour patients and con-
muscle belly using the system's software. trols with respect to intelligence (verbal IQ n = 2 4 , ~ = 0 . 0 2 0 ,
Results: Among the ND children, 86%of the variation in MG performance IQ n=22,p=0.009) processingspeed (WISC 111
volume was explained by a linear relationship with body- PSI n= 15, p = O . O O l , Teach Opposite worlds/same world
weight so MG volume was normalized by bodyweight to make n= 14, p=0.019), selective attention (TEA-Ch Sky search
comparisonsbetween groups possible. For children with SHCI: n=15,p=0.005) and General Memory Index (CMS n=17,
MG volume was less in the affected than the unaffected side p=0.018). In participants aged over six years of age (n=14,
(p=O.OOO) ranging from 8%to 45% smaller. Normalized MG mean age 12y, range by 5mo to 16y 8mo) tested for academic
volume of the affected limbs were smaller than those of the ability with the Wechsler Quicktest, significant differences in
ND group (p=O.OOO) while normalized muscle volumes of comparison to controls were found with respect to basic
unaffected and ND limbs were similar (p=0.645). There was mathematics (n= 14,p=0.026) and composite score (n=14,

54 EACD Abstracts 2005


p=0.009).Basic reading (n=14,p=0.61) and spelling (n= 14, 2. King G , Rosenbaum King S. (1996) Parents’perceptionsof
caregiving:development and validation of a measureof
p=0.316) were not significant. processes.DevMed ChildNeurol38: 757-772.
Conclusion: Children newly diagnosed with brain tumours
have significant impairments in cognitive function that have
a negative impact on academic performance and which
appear to be due to the tumour and surgery. 146 SIGURDARDOTTIR
Comorbidities in Icelandic children with
cerebral palsy
145 SIEBES SOLVEIG SIGURDARDOTTIR, KATRIN EINARSDOTTIR
A family-bound use of the Measure of Processes State Diagnostic and Counseling Center
of Care for Service Providers
PENATE c S I E B E S ~BIANCA
, JG N I J H U I S ~ANNEMARIJKE
, Background: Cerebral palsy (CP) is ‘an umbrella term’ cover-
BOONSTRA~,ADRI V E R M E E R ~ ing a group of non-progressive motor impairment syndromes.
aUtrecht University,Department of General and Special CP is caused by an injury to the developing brain and, there-
Education, Utrecht;Partner of NetChild Networkfor fore, children with CP are more likely to have functional difIi-
ChildhoodDisability Research in the Netherlands; bCenter culties unrelated to movement but related to the central
for Rehabilitation, UniversityMedical Centec Groningen nervous system (learning, communication, sensory, epilep-
University of Groningen,Groningen;CRehabilitation tic, and behavioral). The objective of the study was to identdy
Centet; ‘RevalidatieFriesland’,Beetstenwaag, the Icelandic children with CE document their motor abilities,
Netherlands and secondary conditions.
Methods:The primary source of data were records at the State
objective: The Measure of Processes of Care for Service Diagnostic and Counseling Center (SDCC), which is the only
Providers (MPOC-SP)’ consists of 27 items (assessing four tertiary care center in Iceland serving children with various
domains) with evidence of good reliability and validity, disabling conditions. Birth cohorts of 11years were studied,
examining how service providers think about the quality of of children born between 1990 and 2000. They all had multi-
care they provide and the extent to which these services are disciplinary evaluations at the SDCC, including a neurode-
family-centred. The MPOC-SP will be filled out regularly as a velopmental examination and a psychological evaluation
general measure but it is also informative to gather this very (including cognitive testingwith one of the followingBSID-II/
same information for a specific family. The objective of the wPPSI-R/wISC-III).
present study was to examine whether the MPOC-SP can be Results: One hundred and thirty seven children were identi-
completed for a specific family and to determine the validity fied, 72 females and 65 males. Most of them were evaluated at
of the measure when it is used in this way the age of 5 years, except for the youngest children who were
Methods: One hundred and sixteen service providers (19 dis- evaluated at the age of 2 or 3 years, and a few children who
ciplines) representing five paediatric rehabilitation settings were reevaluated at the age of 8 to 11 years. Motor disability
and affiliated schools in the Netherlands filled out 240 fami- varied significantly;about 1/4th of the group had hemiplegia
ly-bound MPOC-SPs. A further 45 MPOCs (parent version)2 while almost lBrd had severe motor involvement (quadripleg-
and 86 general MPOC-SPs from the same study sample were ia). Forty four per cent were born preterm, 42% had mental
included in the analyses. The construct validity of the family- retardation, and 8 children had nonverbal learning disability
bound MPOC-SPwas determined. Subsequently scores of the About 20% of the group had significant behavioral or emotion-
general MPOC-SP family-bound MPOC-SP and the MPOC al problems, and four had pervasive developmental disorder.
were compared using paired samples t-tests. Conclusions:CP is best understood as a neurodevelopmen-
Results: The Pearson’scorrelation coefficientsconfirmed that tal disorder. Long-termoutcome of children with CP not only
the four scales measure different aspects of family-centred depends o n their motor abilities but also on the various
service. The t-tests did not show significant differencesbetween comorbidities. Therefore, it is important to define and address
parents’ and service providers’ importance ratings of items. concomitant deficits with multidisciplinary evaluations in
Contrary to our expectations, the mean scores of the general order to provide the best services for the children.
MPOC-SPsfor a familywere closer to the MPOC scores of that
family than the mean scores of the family-bound MPOC-SPs.
When filled out family-bound the mean scores were signifi-
cantly lower for three scales and significantlyhigher for one 147 SKRANES
scale compared with the general MPOC-SP scores. These dif- Evaluation of a Program for Intensive
ferences become smaller when the number of service Habilitation for children with cerebral palsy of
providers per family rises. different Gross Motor Function Classification
Conclusions: The analyses proved the family-bound MPOC- System levels
SP is avalid instrument. However, our remaining findings are J S SKRANES, 1 E VESTRHEIM, M MODAHL, S JOHNSEN,
in need of further exploration as they are diametrically G PETTERSEN, G MYKLEBUST, 0 LIED, B LERDAI.
opposed to our expectations. Habilitation Unit,Department of Pediatrics, Serlandet
Hospital, Arendal/Kristiansand, Norway
References
1. WoodsideJM, Rosenbaum PL, King SM, King GA. (2001) Family- Objective: To evaluate the effects of a specialized multidisci-
centered service:developingand validating a self-assessment
tool for paediatric service providers.Children’sHealth Cure plinary programme of training and stimulation for a group of
5 0 237-252. children with cerebral palsy (CP).

55
Material and methods: Three groups of CP children aged fractional anisotropy (FA) maps. Areas of significantdifferences
2-4 years were participating in a programme of intensive between VLBW and controls were then compared with clinical
habilitation. Group A consisted of 4 children with Gross data for the VLBW children.
Motor Function Classification System (GMFCS) level IV-y Results: The VLBW teenagers had significantlylower FAvalues
Group B (5 children) with GMFCS level 1-11, and Group C (4 in five anatomical white matter areas compared with controls:
children) with GMFCS level IV-V The programme focused in internal capsule, corpus callosum, centrum semiovale,
on stimulation of functional activity, communication, and peripheral parietal white matter, and central occipital white
executive functions. The children attended four group ses- matter. SGA children did not differ from controls. Within the
sions of 1-2 weeks duration during the programme period VLBW group motor impairment was related with low FA val-
of 18 months. Between the group sessions each child fol- ues in internal capsule, corpus callosum, and peripheral
lowed an individually-basedhome programme. The children white matter. Visual-perceptualdeficits were related with low
were evaluated with Gross Motor Function Measure (GMFM) FA values in corpus callosum, centrum semiovale, and peri
and Pediatric Evaluation of Disability Inventory (PEDI) at regu- pheral and occipital white matter.
lar intervals before, during, and after the programme period. Adolescents with inattention symptoms or with a diagno-
Results:A positive change in function with time was detected sis of ADHD had lower FA values in most of the areas investi-
with both the instruments used for evaluation. On GMFM a gated, while overall functioning was related with low FA
steady increase in scores with time was observed for all values in all the areas.
dimensions for children with GMFCS level IV-V On PEDI, Conclusion: DTI may reveal white matter abnormalities not
best progress was made for the functional skills, mobility, seen on standard MRI that seems to relate to motor, percep-
and social function. For children with GMFCS level 1-11, tual, and behavioural problems in VLBW adolescents. The
intensive training showed positive effects on GMFM dimen- neuroimpairments may be due to disturbed white matter
sion C (crawling, kneeling), and E (walking, running, jump- microstructure and connectivity of commissural and associa-
ing), and on all PEDI functional skills. tion fibres connecting different cortical areas in the brain.
Conclusions: An intensive habilitation programme seems to
have positive effects on children with CP with different GMFCS
levels. Both GMFM and PEDI were sensitiveto positive changes
in function over time. 149 STEVENSON
Behaviour and language in children with
Acknowledgments: This project was supported with grants from
the Department of Health Services,The Royal Norwegian Ministry of
permanent bilateral hearing impairment and
Health and Care Services, and from the Health South Region
the effects of early confirmation.
administration. JIM STEVENSON O N BEHALF OF THE HEARING OUTCOMES STUDY
TEAM
Schools of Psychology and Medicine, University of
Southampton, Southampton, UK
148 SKRANES
White matter abnormalities seen on diffusion Objective: To investigate whether poor language skills acc-
tensor imaging relate to neuroimpairments in ount for the elevated rate of behavioural problems in children
very-low-birthweight children at 15 years of age with hearing impairment.
J S SKRANES~.,T R VANGBERG~,M INDREDAVIK‘, s KULSENG~, Method: One hundred and twenty children with hearing
K A EVENSEN’, M MARTIN US SEN^, o HARALDSETH~,H B LARSSON~, impairment (67 males, 53 females) of mean age 7 years 11
A DALE^, D T U C H ~ ,T V I K ~ A, M BRUBAKK~ months (5y 5mo to 1ly8mo) and 63 normally hearing children
aDepartment of Laboratory Medicine, Children,and (37males, 26females),meanage8yearslmonth(6y4moto9y
Women’sHealth; bDepartmentof Circulation and Medical 1Omo) from eight districts in southern England were assessed
Imaging; CDepartmentofNeuroscience;dDepartment of for receptive and expressive language skills, and speech articu-
Community Medicine and Public Health, Norwegian lation. The relationship between these measures and parent
University ofScience and Technology, lkondheim, Norway; and teacher reported behaviour problems in the children was
eMartinosCentet; MGH, Boston, USA investigated.
Results: Hearing impaired children had higher levels of teacher
Backpund: Very-low-birthweight (VLBW;C 1500g) children (t=2.27, df=172, pc0.05) and parent reported (t=3.54,
are at increased risk of perinatal periventricular leukomalacia df= 160.3,p~0.002)behaviour problems compared with nor-
(WL). mally hearing children. When language measures were includ-
Objective: To compare cerebral DTI findings with the results ed in the analysis as covariates these differences were no longer
from extensive psychiatric, behavioural, cognitive, and motor significant.This indicates the important role played by speech
assessments in VLBW children. and language development in moderating behaviour in hear-
Material and methods: Thirty-four VLBW children, 42 chil- ing impaired children. Using the 25th centile control group
dren small for gestational age (SGA), and 49 non-SGA term score for language measures to identify children with low lan-
controls were examined at 15 years of age in a population- guage abilities we found similar levels of behaviour problems
based study. Tests used included: the Children’s Global in hearing impaired and normally hearing groups. Irrespective
Assessment Scale (CGAS), Schedule for affective disorders and of the presence of hearing impairment, low language skills sig-
schizophrenia for school-aged children WADS),and ADHD- nificantly increased behaviour problems at school (F[11431
Rating Scale W, WISC-111, VMI and supplementary tests; and =8.72, p=O.OOS) and at home (F[1147)=12.51, ~ ~ 0 . 0 0 2 ) .
Movement ABC. DTI scans were performed for calculation of The development of language and behaviour problems is

56 EACD Abstracts 2005


more strongly related in children with hearing impairment 151 SULLIVAN
than in children with normal hearing. Once the language abili- Bowel habit in the child with neurological
ties of children with hearing impairment are taken into account impairment
the negative effects of hearing impairment on behaviour are no PETER B SULLIVAN
longer found. University of Oxford, Department of Paediatrics, Oxford
Conclusions: Increased behaviour problems found in chil- RadclaTe Hospital, Oxford, UK
dren with hearing impairment are mediated by speech and
language abilities and just as with normally hearing children Constipation in childhood is common affecting3%of children
such behaviour problems are greatest among those hearing and is responsible for 4% of referrals to paediatricians. In a
impaired children with the least well developed communica- recent epidemiologicalsurvey of nutritional and gastrointesti-
tion capabilities. nal problems in children with cerebral palsy (CP), constipation
(when defined as opening of the bowels less frequently than
once in every three days) was reported in 98/377 (26%)of chil-
dren with disabilities.
150 SULLIVAN Defecation depends upon coordinated striated muscle
Nutrition and feeding in cerebral palsy: function: external sphincter relaxation;levator ani stabilization
epidemiology and intervention of anus; abdominal wall contraction and coordination of the
PETER B SULLNArq rectoanal inhibitory reflex.This all usually comes under control
University of Ogord, Department of Paediatrics, Ogord in the 2nd year of life and allowssocially acceptablecontinence
Radcliffe Hospital, Odord,UK by age 3 years in the majorityof children.
Chronic constipation can be considered to be an intesti-
Background:The Oxford Feeding Studies' revealed a signifi- nal motility disorder with increased threshold in perception
cant unmet need of children with neurological impairment. of rectal distension, incomplete relaxation of the internal
Enteral feeding via gastrostomy tube is increasingly used as a sphincter, and inability to relax the external anal sphincter
nutritional intervention in children with cerebral palsy (CP). during attempted defecation. The deficiency in the spinal
Results of a longitudinal, prospective multicentre cohort study nerves in myelomeningocoele leads to a lack of control of
designed to measure the outcomes of gastrostomy tube feed- striated muscle with poor sensation, weak external sphinc-
ing in children with CP will be reported. ter, and an exaggerated rectoanal inhibitory reflex. This leads
Methods: Fifty-seven children with CP (28 females, 29 males; tofaecal incontinence. Children with disabilities are particu-
median age 4y 4mo) were assessed before gastrostomy place- larly prone to constipation because of the poor consistency
ment and at 6 and 12 months afterwards. Outcome measures of stool (low fibre and fluid intake) and abnormal colonic
included groWanthropomeuy, nutritional status and motility (myelomeningocoele, immobility, medication).
intake, time spent on feeding, general health, complications The assessment of children with CP aims to establish
of gastrostomyfeeding, and caregiver quality of life. whether the main problem is failure to achieve continence or
Results:At baseline half of the children were more than 3 stan- overflow secondary to constipation. History and examina-
dard deviations below the average weight for their age and sex tion may be complemented by investigations, plain abdomi-
when compared with the standards for normally children. nal radiograph, transit studies using radio opaque markers,
Weight increased substantiallyover the study period; the medi- rectal biopsy, ano-rectal manometry, and endoanalsonogra-
an weight z-score increased from -3.0 pre-gastrostomy place- phy. Management of secondary faecal incontinence in chil-
ment to -2.2 at 6 months, and -1.6 at 12 months. Weight gain dren with disabilities is similar to that in those without
was accompanied by significant increases in skin fold thick- neurological impairment and is based on three principles:
ness, indicating deposition of subcutaneous fat. In most the (1) Evacuation of retained faeces (2) Maintenance laxatives
improvementswere sustained and similar over the two consec- regime to promote regular bowel habits, and (3) Education:
utive six-monthsperiods. h o s t all parents reported a signifi- toilet training (if possible); dietary (fluids and dietary fibre)
cant improvement in their child's health following this advice;and support and reassurance of parents.
intervention, accompanied by a significant reduction in time
spent feeding. Gastrostomyfeeding in children with disabilities
was associated with significant increases in weight gain and
subcutaneous fat deposition, and a significant reduction in 152 TARSUSLU
feeding time. Serious complications were rare, with no evi- Is there a relation between early weight
dence of an increase in respiratory complications. This bearinglstanding and hip problems in
quantitative study demonstrated a significant measurable non-ambulatory quadriplegic cerebral palsy?
improvement in the Quality of Life of caregivers after inser- T TARSUSLUA,F DOKUZTUA, T TUZUNER'
tion of gastrostomy feeding tube, with substantial rises in aAbantIzzet Baysal University School of Physical Tberapy
average domain scores observed for mental health role, emo- and Rehabilitation, Bolu; bAbantIzzet Baysal University
tional and physical, functioning, social functioning, and ener- Bolu, Tiwkey
gyfitality 6 months after gastrostomy feeding was started.
Objective: Standing and weight bearing is a very important
Reference stage in motor development. Problems such as hip dysplasia,
1.SullivanPB, Lambert B, Rose M, Ford-Adams M, Griffiths P, hip subluxation, and hip dislocation can be seen in children
JohnsonA. (2000) The prevalence and severity of feeding and
nutritional problems in childrenwith neurological impairment: who are unable to stand and bear weight on their lower
The W o r d Feeding Study.DevMed ChildNeurol42:674-680. extremities. The aim of the study is to investigate the age of

57
first standing and related hip problems. were more successful in the third assessment when com-
Method: Eighty-four children with quadraplegic cerebral pared with the second evaluation.
palsy (CP 27 females 57 males) were included in the study Conclusfom:Consequently it can be said that the prosthetic
Mean age was 98.93 months (SD 59.08) and functionalstatus control is better at the PFFD level than at the other levels of con-
according to Gross Motor Function Classification System genital lower limb loss. This result is probably related to the
(GMFCS) and clinical type were identified. Muscle tone was length of the extremity in PFFD, better proprioception, bal-
evaluated with the modified Ashworth scale (MAS).2The age ance control, weight bearing from the plantar side of the foot,
at which the children began to stand was ascertained from and the capability of neutralizing the reactional force in gait.
the families.HipX-Raysfor the past 6 months were examined Although the prosthetic fittingat PFFD level presents some cos-
for evaluation of hip subluxationand dislocation. metic problems and musculoskeletel problems remain, the
According to the GMFCS, 49 of the children were at level y functionalassessmentsdemonstrateda successfullevel in daily
31 of the childrenwere at level n! and four of the childrenwere activities.
at level 111. The age of first standing for females was 40.15
months (SD 48.09) and 49.25 months (SD 40.27 for males.
None of children began to stand before the age of 1 year. In our
study 10 children began to stand between the age of 1 to 2 154 VAN HULST
years, 12 of them begin to stand between the age of 2 to 3 years, Swallowing and dysphagia in relation to
and 62 of them begin to stand between the age of 3 years and posterior drooling in children with cerebral
older. Eighty per cent of them could not walk, 13.09%(11 chil- palsy
dren) of the 84 participants had hip dislocation,and 3.57%(3 KAREN VAN HULST, PETER JONGERIUS, JAN ROTTEVEEL
children) had hip subluxation. Four were at level IV and 10 at UniversityHospital Nijmegen, Postbus 9101- 945 IKNC -
level V According to MAS, hip flexor was 4 in 2 (18%)partici- 6500 HB, Nijmegen, the Netherlands
pants, 3 in 8 (72%)participants,and 2 in 1 (9%)participant;hip
adductors were 4 in 9 (81%)3 in 1 (9%)participant 2 in 1 (9%) Introduction: Dysphagia and drooling are common prob-
and hip internal rotators were 4 in 1 participant (9%)3 in 7 lems in children with cerebral palsy (CP). Drooling is fre-
(63%),and 2 in 3 (27%)participants. quently described as the result of mal-coordination of
Discussion: Standing and weight bearing are very important muscles involved in the oral stage of swallowing, involving
in children with CF! It is recommended that generally chil- lip closure, sucking, and chewing. In this sense, it is merely
dren should be encouraged to adopt the standing position at the clinical expression of a defect in the oral phase of swal-
1 year to help motor development and the development of lowing. The standard concept of drooling refers to visible
acetabulum and prevent secondaryhip problems. anterior oral (or labial) spill. However when the child has an
oral motor dysfunction,oral retention in sublingualand buc-
References cal pools can occur,which may result in posterior spill of sali-
1. GoldschmidtEA.(1998) Instability of the hip in cerebral palsy.
va over the tongue base through the faucial isthmus.
Ortbopedfcs1% 40-50.
2. Bohannon RVC: Smith MB. (1987) Interrater reliability of a Rvo forms of drooling exist: anterior drooling and posterior
modifiedAshworh scale of muscle spasticity.P b p Tber drooling.Anteriordrooling (sialorrhea) is caused by ineffective
67: 206-207. swallowing or insufficientlip closure resultingin visible spill of
saliva from the mouth. The term posterior drooling has been
applied to oral secretions that pool in the ompharynx and
hypo-pharynx. Normally this should stimulate a swallowing
153 TOPUZ reflex. In the absence of a trigger to swallow, saliva spills over
Functional evaluation of children with into the pharynx producing congested breathing, coughing,
Proximal Focal Femoral Deficiency gagging, vomiting, and, at times, aspiration into the trachea.
0 ULGER, S TOPUZ, K BAYRAMLAR, F ERBAHCECI Posterior drooling is caused by severe oral-pharyngealmotor
b e Prosthetics and BiomechanicsDepartment of School of dysfunction.(Silent)dysphagiacan cause chroniclung disease,
Physical l%erapy and Rehabilitation in Hacettepe recurrent pneumonia, and failure to thrive.
University, firkey Presentation:At the University Hospital Nijmegen, a swallow-
ing and droolingteam has been founded to assess and evaluate
Objectives: This study is being carried out to determine the the swallowing process of children with CE By means of case
functional abilities and inabilities of children with Proximal studies and imaging of video fluoroscopicswallowstudies the
Focal Femoral Deficiency (PFFD)with and without prostheses. impaired swallowing processwill be explained,providing rele-
Met&&: Amputee Mobility Predictory Scoring Ouestionnaire vant information for cliniciansworking with children with dis-
(AMP) and The Child Amputee Prosthetic and Project abilities.
FunctionalStatus Inventory (CAP-FSI)are used to evaluate the
functional state of the seven children whose ages varied
between 8 and 17 years. Besides the prosthetic assessments,
evaluations were carried out, first, before treatment; second,
three weeks after treatment; and third, six months after dis-
charge,with and without the prostheses.
Results:There was no important differencebetween the first,
second, and third evaluationsofAMP and CAP-FSIperformed
without the prostheses.The results showed that the children

58 EACDAbstractsZOO5
155 VAN MEETEREN type of Cp, up to 50% of the patients with CP have learning
Validity of an arm-hand function disabilities. The aim of this study is to determine which cog
classification system (Manual Ability nitive and communicative functions are important for social
Classification System) in young adults with participation.
cerebral palsy Methods: As part of the Dutch CP Transition study, adoles-
J ~ , E CELEN", A DE G R U N D ~ ,
N M E ROEBROECK~,
~ cents and young adults aged 16 to 20 diagnosed with CP
c NIEUWENHUIJSEN~, HJ ST AM^ without severe learning disabilities (n= 105) participated in
aErasmusMC-UniversityMedical Centre,Rotterdam; this study. General patient characteristics and level and type
bSophiaRehabilitation Centre, l%e HaguelDelf, the of education were measured. Cognitive abilities were mea-
Netherlands sured by means of cognition and communication items of
the Vineland Adaptive Behavior Scale and the Functional
Objective: The transition phase from childhood to adult- Independence Measure. Outcome measure was social par-
hood is considered critical in the development of participa- ticipation (LIFE-HABITS).
tion. In 50% of participants with cerebral palsy (CP), the Results: About 86% of the participants was still studying. The
arm-hand function is limited. This study aimed to assess current level of education was lower vocational or less in
whether the Manual Ability Classification System (MACS) 40% of participants. About 50% followed mainstream educa-
designed for children between 8 and 12 years can be used in tion of whom 5 to 10% needed special assistance. With
young adults with CP in order to investigate arm-hand func- respect to cognition most difficulties were encountered in
tion as a determinant of participation in addition to gross reading, writing, and problem solving. Impaired gross motor
motor function and cognitive level. functioning was related to impaired cognitive functioning
Method: As part of the CP Transition Study in the South-West and low level of education.
Netherlands 90 young adults with CP without severe learn- Cognitive functioning (particularly problem solving,
ing disabilitywere assessed with respect to activitiesand par- r= 0.58) and communicative functioning (particularly read-
ticipation. In this study the arm-hand function was assessed inghwiting, r=O.66) were related to level of education.
using four bimanual items of the Melbourne assessment, the Furthermore, all variables of cognitive and communicative
Abilhand Questionnaire domain self-care according to the functioningwere correlated with social participation (respec-
FIM, and some items of the Life Habits questionnaire. Patients tively r=0.23-0.59 and r=0.37-0.55).Finally, level of educa-
were classified according to the Bimanual Fine Motor tion was related to social participation (r=0.44).
Function (BFMF) and MACS. Interrelationships between Conclusions: In patients with CP without severe learning
these assessments of arm-hand function and relationships disabilities, cognitive and communicative impairments
with other patient characteristics including the GMFCS were were related to level of education and social participation.
analyzed by correlation coefficients (Spearman's rho). For these patients level of education seems a valid indica-
Results: The distribution of the MACS was 72% in level I, 16%in tor of cognitive functioning.
II,5% in 111, and 2%inVThe correlationbetween the MACS and
GMFCSwas 0.47 (p=O.OOOl). Further analysis is performed to
assess the BFMF based on four items of the Melbourne assess-
ment. Comparison with the other measurement outcomes is 157 VERKERK
presented. Reliability and validity of the Canadian
Conclusion:The MACS seems to be a useful classificationsys- Occupational Performance Measure in
tem for the arm-hand function in young adults with CP The parents
results of this study contribute to the evidence on its validity G VERKERK", M-J WOLF^, A LOUWERS~,A ME ESTER^, F NOLLEP
for adult patients. aDepartment of Rehabilitation, Academic Medical Centre,
Amsterdam; bRehabilitation Centre, Pappenberg, Huizen,
the NetherZands

156 VANSTIPHOUT Objecttve:To study the reliability and validity of the Canadian
Cognitive abilities and social participation in Occupational Performance Measure (COPM) in parents of
adolescents and young adults with cerebral Dutch children referred for occupational therapy.
palsy without severe learning disabilities Methods: The COPM is a semi-structured interview to detect
C G H VANSTIPHOU?~~, M DONKERVOORT~,R PANGALILA M D ~ , occupational performance problems and was administered
M E ROEBROECK".~,H J ST AM^ twice with a mean interval of 7 days (SD 2.8) by two occupa-
aErasmusMC- UniversityMedical Centre, Rotterdam; tional therapists independently On both occasions the par-
bRijndamRehabilitation Centre, Rotterdam; Cl+ansition ents prioritised five problems with their child. The problems
Research Group,Rehabilitation Centres in region South- reported in the COPM were compared with an open-ended
WestNetherlands;dErasmusMC- UntversityMedical Centre, question, the Pediatric Evaluation of Disability Inventory
Department of Rehabilitation Medicine, the Netherlands (PEDI-NL), and the Preschool children's Quality of Life ques-
tionnaire (TAPQOL) in children below six years of age and
Objective: Research in young adults with cerebral palsy (CP) the Children's Quality of Life questionnaire (TACQOL) in
shows that they are confronted with limitations in daily activ- older children.
ities and social participation. In a previous study we found Results: Complete data of 80 parents ofchildren between 1-7%
gross motor functioning and level of education to be impor- years (mean age 3y 8mo) were obtained. Of the five problems
tant determinants of social participation. Depending on the prioritized in the first interview, 74% were also prioritized in

59
the second interview. Seventy-one per cent of the problems this moment. The first results are promising. At the congress
mentioned in the open-ended questionnaire could be iden- the tests will be visually demonstrated and data on reliability
tified in the prioritized problems of the COPM. and feasibilitywill be presented.
Flfty per cent of the problems identified by the COPM, how-
ever, could not be detected in the PEDI-NL. These problems
included preschool skills,independent play, outdoor activities,
reading, and writing which are activitiesthat require adequate 159 WALSH
sensory integration. Sixtyone per cent of the problems identi- Mobile gait analysis - a low-cost solution to
fied by the COPM could not be detected by the TAP(C)QOL. provision of this specialized service to a large
Conclusions:Results suggest that the reliability of the con- geographical area
tent of the COPM problems is sufficient and that the COPM M WALSH, R O'SULLIVAN, D BENNETT, A JENKINSON, C DUNLEVY,
provides unique information that cannot be obtained with T O'BRIEN, 0 HENSEY
existing standardized tests. Central Remedial Clinic,Dublin, Ireland
The COPM is, therefore, a valuable tool to identifyoccupa-
tional performance problems of young children from the Introduction: One of the specialized services offered at the
perspective of their parents using a client-centred approach. Central Remedial Clinic in Ireland is clinical gait analysis.
Children from all over Ireland travel to Dublin to access this
service. This often involves an arduous and expensive jour-
ney for children and their families which poses problems.
158 VERSCHUREN Establishing permanent gait laboratories in remote regions
Development of two running-based anaerobic to make this specialized service more accessible would be
field exercise tests for children and adolescents extremely difficult and economically unjustifiable. The pur-
with cerebral palsy pose of this project was to develop a mobile gait laboratory.
OLAF VERSCHIJREN", MARJOLIJN KETELAAR~,TIM TAKKEN~, Materials and methods: Three Coda CX1 motion analyzers,
PAUL J M HELDERS, JAN WILLEM GORTER", two portable AMTI force plates, and a Noraxon 16-channel
aRehabiNtationCentre,De Hoogstraat, Utrecht;bUniversity wireless EMG system were integrated to capture simultane-
Children'sHospital andMedica1 Centet;Department of ous kinematic, kinetic, and EMG data respectively. Video
Pediatric Physiotherapy and Exercise Physiology, Utrecht, equipment was purchased and a 15m fibreglass walkway was
the Netherlands designed to provide a flush walking surface. Video-con-
ferencing equipment was purchased to allow live communi-
Objective:As many childhood activities consist of short-term cation of data between the mobile clinic and the permanent
bursts of intense activity, anaerobic performance is an impor- gait laboratory in Dublin.
tant measure. For children with cerebral palsy (CP) classi- Results: Following successful pilot clinics, the mobile gait
fied as level I or I1 on the GMFCS, anaerobic performance has analysis service is now firmly established in the Mid-Westem
predominantly been measured using the Wingate Anaerobic and South-EasternHealth Executive regions of Ireland. So far,
30s cycling Test (WAnT). The WAnT, however, is more specifi- nine outreach clinics have been conducted, which has resulted
cally geared to cycling, not running and the necessary equip- in 37 families and their children not having to travel to Dublin
ment is expensive, may require modification,and may not be for their assessment. The staff of the mobile gait laboratory
readily accessible. have linked-in live with the orthopaedic surgeon's clinic in
The purpose of this study was to develop two running- Dublin through video-conferencing technology to present
based exercise tests for children with CI! their patients from the remote regions and receive their med-
Methods: Literature on theories of exercise and exercise tests ical recommendations. Feedback from service-usersand local
were studied. Based on the WAnT and the effort children clinicians has been extremely positive.
have to perform in it, two running-based tests for anaerobic Conclusions:The mobile gait analysis service provides a low-
performance were developed. There were several try-outs cost solution to the Irish government in providing this special-
with children with CP, varying for age and GMFCS-levels I and ized service locally to more remote parts of Ireland. The core
11. Running-distances ofboth tests were modified until mean medical expertize and established knowledge base of the gait
total test-timewas around 30 seconds. laboratory remains centralized while the technical acquisition
Results: N o running-based exercise tests were developed: (1) expertize has been mobilized.
The 10x5m sprint test and (2) The Cerebral Palsy-Anaerobic
Running Test (CP-ART).
For the 10x5111 sprint test, the participant is asked to
complete 10 runs of 5m at maximum pace. There is no rest
in between the runs. This test measures the anaerobic per-
formance and, in some way, the coordination of speedy
movements.
In the CP-ART the participant is asked to complete six 15m
runs at maximum pace. Between each run the participant was
allowed 10 seconds rest. Power output for each sprint can be
calculated from the collected data. Both tests were easy to per-
form and children enjoy doing it.
Conclusions: Reliability and feasibility are being studied at

60 EACDAbstracts2005
160 WHITE-KONING 161 WIEGERINK
Assessment of response competence before Social and sexual relationships of adolescents
administering self-reported quality of life and young adults with cerebral palsy in the
instruments to children with cerebral palsy Netherlands
and intellectual impairment DONKERVOORP,
D I H G W I E G E R I N K ~ +M, E ROEBROECK~,
M
c ARNAUD",~,D FENIEYS", A C O L V E R ~ ,
M L WHITE-KONING".~, HJ ST AM^, P T COHEN-KETTENIS~
H GRANDJEAN~J' ON BEHALF OF SPARCLE GROUP aErasmusMC UniversityMedical Centet;Rotterdam;
aINSERM U558, Toulouse; bUniversit4Paul Sabatier, bRijndamRehabilitation Centet;Rotterdam; W University
Toulouse,France; CUniversityof Newcastle upon Vne, Medical Centet;Amsterdam, the Netherlands
Newcastle upon Vne, UK
Objective:The relationship between quality of life (QoL) par- Objective: Transition into adulthood involves participation
ticipation and environment is being studied in the Study of in different domains. Social and sexual relationships is one
Participation of Children with Cerebral Palsy Living in of these domains in which many changes take place.
Europe (SPARCLE) project involving 8 to 12 year-old chil- This study provides a detailed description and improved
dren with cerebral palsy (CP) in eight centres across Europe. understanding of the problems encountered by young per-
One aim is to assess subjective QoL in these children, includ- sons with cerebral palsy (CP) and to detect potential determi-
ing the subgroup with intellectual impairment which raises nants ofparticipation in social and sexual relationships.
specific conceptual and methodological issues. This presen- Methods:A prospective cohort study on the determinants and
tation examines whether response competence can be course of functional status and participation in 103 adoles-
assessed in children with CP and intellectual impairment. centshoung adults with CP without severe learning disabilities
Methods: We adapted a pre-test procedure developed by aged 16-20 years; and a systematic review of the literature on
Cummins' in which it is established in a graded manner how social and sexual relationships in young adults with CP This
well the child understands the concept of a scale, an essential study is performed as part of the research program Pediatric
pre-requisite to answering questions about QoL. Rehabilitation Research in the Netherlands (PERRIN).
The procedure was adopted by all the SPARCLE interview- Results: In adolescence, contacts with peers, social activities,
ers to aid their assessment of child competence. This proce- and developing intimate and sexual relationships, are impor-
dure has not previously been used in a paediatric population. tant aspects of daily life and normal development of young
Results: The procedure was used mainly with children with sters. Leisure activitiesand social relationships of the youngs
mild or moderate intellectual impairment but four centres people with CP in our study are comparable to Dutch age
also found it useful for assessing non-impaired children. The mates. From those with C€! 52% has experience with dating.
interviewers reported that the procedure successfullyassessed Only 20%of the young people with CP had a steady boyfriend
the ability to use a scale and some used it throughout the while 32% of their Dutch age mates did. The sexual experi-
questionnaire to illustrate the range of possible answers. The ences of this group are delayed compared with their Dutch
main difficultyfor the interviewerswas knowing whether the age mates. The role of self-esteem as a potential determinant
intellectually impaired or young children understood the of sexual relationships is evaluated.
wording of the questions rather than whether they could use a Conclusions: In the transition phase, young people with CP
scaled response. participate well in social relationships but they encounter
Conclusions: Subjective QoL is difficult or impossible to problems in developing intimate and sexual relationships.
assess in childrenwith severe intellectual impairment but for
the less severely impaired it is important and possible to
obtain such an assessment. The competency procedure sug-
gested is adequate for assessing scale comprehension but 162 WILLIAMS
should be combined with some form of evaluation of the Static bicycle training for non-ambulant
child's vocabulary. adolescents with cerebral palsy: effect on
muscle strength and functional ability
Reference HA WILLIAMS, T E POUNTNEY
1. Cummins RA.(1993) ComprehensiveQualityOfLijeScale,
Valence School,Kent, UK
Intellectual Disability. 4th edn. Melbourne:Psychology
Research Centre.
Objective: To investigate the effect of muscle strengthening
Acknowledgement: Supported by European Commission Research exercise on the motor function of adolescents with cerebral
Framework 5, Grant number QLG5-CT-2002-00636. palsy (CP) using an adapted static bicycle.
Methods: The study is an AI3A design with participants acting
as their own controls with a 6-week baseline, 6-week exercise
period (3 sessions a week), and a 6-week follow-up period.
Outcomes were assessed using the Gross Motor Function
Measure (GMFM) 66 and 88,and the Chailey Levels ofAbility.
A 'Graded Exercise Test' determined the pedalling resistance
and muscle 'overload' was ensured by increasing the dura-
tion and speed of pedalling.
Preliminary results: Ten participants (aged 11-15y mean 12y
6mo) were recruited: (1) Significant improvements were
found in 'cycling' ability for duration of pedalling (p<O.OoOl),

61
speed (p=0.006), and resistance (p=0.005). (2) Sigmficant Department, Hospital Santa Maria, University of Lisbon,
improvements were found in GMFM-66 (p=0.028) and Portugal, between January and November 2004. The diagno-
GMFM-88 dimensions, D ‘standing’ @=0.027), E ‘walking’ sis of Asperger syndrome was based on a clinical interview,
(p=0.026, but not ‘sitting’),over the intervention period. (3) informal observation,and Gillberg criteria. The ‘Phonological
N o significant change was found in any functional tests over Assessment Battery’ (Bateria de Provas Fonologicas) was used
the baseline or follow-up periods. (4) No significant change to evaluate phonological awareness.
was found in the Chailey Levels of Ability overall scores or Results were compared with those of a conveniently
individual attributes. Full results were available in July 2005 selected control group which included the same number of
based on eight participants. children of the same age attending primary schools in
Conclusions:Muscle strengthening exercise on an adapted greater Lisbon and with a conventional global develop-
static exercise bicycle can improve standing and walking ment.
motor function in older children with severe CP These Statistical analysis of results was performed using non-
improvements are ofvalue to wheelchair users and their car- parametric tests (Mann-WhitneyU and Spearman) from SPSS
ers as they are skills often lost with increasing age and size. forwindows (version 11.0.).
The static bicycle provided a safe enjoyable age-appropriate Results; In general this study revealed significantly lower
means of exercise to a population with very limited opportu- results in children with Asperger syndrome when compared
nities for activity. with the control group. The syllabic analysis test was the only
one in which results were similarin both groups. The tests with
greater statistical significancewere those of initial syllabic and
initial phonemic suppression in the oldest group. The experi-
163 XIMENES mental group presented lower scores because these tasks
Phonological awareness in children with demand more specialized skills and the children had not yet
Asperger syndrome achieved these goals.
M I X I M E N U , R GOUVEIA, M BAPTISTA Conclusions: Children with Asperger syndrome had lower
Child Development Department, Hospital Santa Maria, results in phonological awareness than the control group.
University oflisbon, Lisbon, Portugal These results show the need to design an evaluation and inter-
vention programme in order to stimulate early phonological
Objective: The purpose of this study was to verify the differ- awareness in children with Asperger syndrome.
ences between the level of phonological awareness in child-
ren with Asperger syndrome and children with a conven-
tional development.
Methods: Nineteen children with the diagnosis of Asperger
syndrome were included in this study with ages over 5 years
and 6 months and followed at the Child Development DOI: 10.1017/S0012162205001660

62 EACDAbstracts2005

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