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Prognosis for Gross Motor Function in Cerebral Palsy:

Creation of Motor Development Curves


Peter L. Rosenbaum; Stephen D. Walter; Steven E. Hanna; et al.
Online article and related content
current as of February 22, 2009. JAMA. 2002;288(11):1357-1363 (doi:10.1001/jama.288.11.1357)

http://jama.ama-assn.org/cgi/content/full/288/11/1357

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Related Articles published in Predicting Gross Motor Function in Cerebral Palsy


the same issue Stephen L. Kinsman. JAMA. 2002;288(11):1399.

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ORIGINAL CONTRIBUTION

Prognosis for Gross Motor Function


in Cerebral Palsy
Creation of Motor Development Curves
Peter L. Rosenbaum, MD, FRCPC Context Lack of a valid classification of severity of cerebral palsy and the absence of
Stephen D. Walter, PhD longitudinal data on which to base an opinion have made it difficult to consider prog-
nostic issues accurately.
Steven E. Hanna, PhD
Objective To describe patterns of gross motor development of children with cere-
Robert J. Palisano, ScD bral palsy by severity, using longitudinal observations, as a basis for prognostic coun-
Dianne J. Russell, MSc seling with parents and for planning clinical management.
Parminder Raina, PhD Design Longitudinal cohort study of children with cerebral palsy, stratified by age
and severity of motor function and observed serially for up to 4 years during the pe-
Ellen Wood, MD, FRCPC, MSc
riod from 1996 to 2001.
Doreen J. Bartlett, PhD Setting Nineteen publicly funded regional childrens ambulatory rehabilitation pro-
Barbara E. Galuppi, BA grams in Ontario.
Participants A total of 657 children aged 1 to 13 years at study onset, representing

C
EREBRAL PALSY OCCURS IN EV-
the full spectrum of clinical severity of motor impairment in children with cerebral palsy.
ery to 2/1000 to 2.5/1000 live
Main Outcome Measures Severity of cerebral palsy, classified with the 5-level Gross
births.1 It is . . . an umbrella
Motor Function Classification System; function, formally assessed with the Gross Mo-
term covering a group of non- tor Function Measure (GMFM).
progressive, but often changing, motor
impairment syndromes secondary to le- Results Based on a total of 2632 GMFM assessments, 5 distinct motor develop-
ment curves were created; these describe important and significant differences in the
sions or anomalies of the brain arising rates and limits of gross motor development among children with cerebral palsy by
in the early stages of development.2 severity. There is substantial within-stratum variation in gross motor development.
Thus, whatever additional developmen-
Conclusions Evidence-based prognostication about gross motor progress in chil-
tal difficulties individuals with cerebral
dren with cerebral palsy is now possible, providing parents and clinicians with a means
palsy might have as a result of impair- to plan interventions and to judge progress over time. Further work is needed to de-
ment of the developing central nervous scribe motor function of adolescents with cerebral palsy.
system, the hallmark of these condi- JAMA. 2002;288:1357-1363 www.jama.com
tions is a disorder in the development of
gross motor function.
milestones such as sitting between the aided at or after age 5 years vary for
When first told that their child has ce-
ages of 2 and 4 years and walking at a different clinical types of cerebral palsy.6
rebral palsy (generally in the childs first
later age. However, the findings based on These observations derive from clinic
18 months of life), parents usually want
even these simple markers are conflict- samples and are likely not representa-
to know its severity and whether their
ing.4,5 Crude estimates of the probabil- tive of the entire population of children
child will ever be able to walk. The evi-
ity of being able to walk 10 steps un- with cerebral palsy.
dence on which to base answers was, un-
til recently, limited to observations about
Author Affiliations: Departments of Pediatrics (Dr University, Philadelphia, Pa (Dr Palisano); Department
the association between constellations of Rosenbaum) and Clinical Epidemiology and Biostatis- of Pediatrics, Dalhousie University, Halifax, Nova Sco-
reflex and early motor skills at age 2 years tics (Drs Rosenbaum, Walter, Hanna, and Raina), School tia (Dr Wood); and School of Physical Therapy, Univer-
of Rehabilitation Science (Drs Rosenbaum and Hanna, sity of Western Ontario, London, Ontario (Dr Bartlett).
and walking at a later age3; or on motor and Ms Russell), CanChild Centre for Childhood Dis- Corresponding Author and Reprints: Peter L. Rosen-
ability Research (Drs Rosenbaum, Walter, Hanna, Pali- baum, MD, FRCPC, McMaster University, CanChild
sano, Raina, Wood, Bartlett, and Mss Russell and Centre for Childhood Disability Research, IAHS, Room
For editorial comment see p 1399. Galuppi), McMaster University, Hamilton, Ontario; 408, 1400 Main St W, Hamilton, Ontario, Canada,
Department of Rehabilitation Sciences, MCP Hahnemann L8S 1C7 (e-mail: rosenbau@mcmaster.ca).

2002 American Medical Association. All rights reserved. (Reprinted) JAMA, September 18, 2002Vol 288, No. 11 1357

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PROGNOSIS FOR GROSS MOTOR FUNCTION IN CEREBRAL PALSY

Table 1. Gross Motor Function Classification System (GMFCS)


GMFCS Level

I II III IV V Total
(n = 183) (n = 80) (n = 122) (n = 137) (n = 135) (N = 657)
Age, y
1-2 16 13 13 12 14 68
3-4 47 20 22 30 22 141
5-6 30 15 30 30 36 141
7-8 36 14 27 29 32 138
9-10 36 18 20 31 26 131
10 18 0 10 5 5 38
Age, mean (SD) [median], y 6.90 (2.91) [6.82] 6.16 (2.75) [6.39] 6.88 (2.91) [6.85] 6.81 (2.71) [6.71] 6.76 (2.65) [6.62] 6.76 (2.80) [6.62]

Cross-sectional studies of motor be- licly funded, each program serves the sample of 150 children per GMFCS
havior in children with cerebral palsy majority of eligible children in its area. stratum would provide a power of 0.85.
have demonstrated characteristic pat- Of the sampling frame containing 2108
terns of motor development according Sample children, 1304 were stratified by age and
to severity of the condition,7 although The sampling frame was created in early GMFCS level and were randomly se-
the descriptions of severity previously 1996 with 18 of the 19 centers and 1 hos- lected. Our target was 15 children in each
used have been crude and unsystem- pital-based therapy program in a com- combination of birth year and severity
atic. The motor growth curves created munity without a regional center. Each level. An initial sample was drawn from
by Palisano et al,8 which are based on center identified all the children who had children with known severity level. We
cross-sectional population data strati- been diagnosed as having cerebral palsy also drew a second random sample from
fied by severity using the validated Gross and who had been born in or after 1986. those children whose severity level was
Motor Function Classification System Children with neuromotor findings con- initially unknown. Based on the re-
(GMFCS) for cerebral palsy,9 are an im- sistent with cerebral palsy, such as spas- quired quota for each stratum, the cen-
portant improvement. ticity or reflex abnormalities, who had ters established the severity level for a
This article describes patterns of gross not been diagnosed as having cerebral specified set of children. Once the level
motor development of a community- palsy were included in the study. Chil- of a child in this set became known, he/
based sample of children with cerebral dren with other neuromotor disabili- she was added to the study sample for
palsy followed up prospectively. We used ties, such as spina bifida or muscle dis- the appropriate stratum (TABLE 1).
the GMFCS to longitudinally create eases, were excluded. Children were also Each of the sample sizes was calcu-
curves charting the rates and limits of excluded if they had selective dorsal rhi- lated to achieve equal sampling frac-
motor function by severity of motor im- zotomy,10 had received botulinum toxin tions for children with initially known
pairment. These curves increase the injections in the lower limbs for spastic- or unknown severity. We oversampled
prognostic information available to fami- ity management,11,12 or were receiving in- for each age and GMFCS stratum to try
lies and clinicians considerably. trathecal baclofen.13 At the time the study to achieve at least 15 children per pre-
started, it was not yet known how these defined cell. A total of 366 children were
METHODS relatively new interventions might affect ineligible or unavailable for various rea-
Setting gross motor function. None of these in- sons. Of the remaining 938 children, 721
This study was made possible through terventions was readily available in On- (77%) families consented and 682
a partnership between the CanChild tario at the time of the study. To the best (94.5%) provided data; 657 had fully
Centre for Childhood Disability Re- of our knowledge, no children were re- useable data, after excluding children
search at McMaster University and the ceiving hyperbaric oxygen therapy, an in- without cerebral palsy (FIGURE 1). The
19 publicly funded regional ambula- tervention that has since been shown to children ranged in age from 1 to 13 years
tory childrens rehabilitation pro- be of no added value for children with at study entry.
grams in Ontario. These programs pro- cerebral palsy.14 At the first assessment, therapists
vide a range of developmental therapies Sample size calculations were per- reported the distribution of the childs
and services (predominantly physical, formed using data from Scrutton and cerebral palsy as it was reported in the
occupational, speech-language, and rec- Rosenbaum.7 Based on the Gross Mo- childs clinic chart (hemisyndrome, diple-
reational therapies) by professionals tor Function Measure-88 (GMFM-88) gia, triplegia, or quadriplegia). They also
trained and experienced in assess- and estimated score limits for a 10- included any terms that had been used
ment and management of childhood year-old in each GMFCS stratum (98- to describe the diagnosis. When no for-
disability. Because the centers are pub- 100, 90-95, 60-80, 12-50 and 10), a mal diagnosis had been given to the child,
1358 JAMA, September 18, 2002Vol 288, No. 11 (Reprinted) 2002 American Medical Association. All rights reserved.

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PROGNOSIS FOR GROSS MOTOR FUNCTION IN CEREBRAL PALSY

therapists were asked whether the childs limb-dominant, 98 (15.3 %) hemisyn- MacMillan Centre (Toronto, Ontario),
motor behavior and patterns looked dromes, and 17 (2.8%) unknown. and the Thames Valley Childrens Cen-
like cerebral palsy. Males (n=369) com- tre (London, Ontario) approved the
prised 56% of the group. Topographical Outcome Measures study. It was centrally managed at Can-
distribution of cerebral palsy included Severity of cerebral palsy was based solely Child, with a site coordinator in each
217 (33.0%) leg-dominant children, 62 on GMFCS level, which is a reliable and center, who was responsible for the day-
(9.4%) 3 limb-dominant, 263 (40.0%) 4 valid system that classifies children with to-day management of data collection.
cerebral palsy by their age-specific gross Before beginning to assess children, all
motor activity.8,9,15 The GMFCS de- therapists were trained on the adminis-
Figure 1. Sample Selection and Recruitment
scribes the major functional character- tration and scoring of the GMFM.21
2108 Children in Sampling Frame istics of children with cerebral palsy in Their reliability was assessed against a
each level within the following age win- criterion tape at the end of training and
1304 Randomly Selected
dows: prior to second birthday; be- reassessed annually over the 4 years of
366 Ineligible or Unavailable tween age 2 years and fourth birthday; data collection to ensure that they con-
to Participate
between age 4 years and sixth birthday; tinued to score the measure reliably.
938 Invited to Participate
and between ages 6 and 12 years. The To track individual gross motor devel-
BOX outlines the main abilities of chil- opment, children younger than 6 years
217 Refused Consent
dren aged 6 to 12 years in each GMFCS were assessed with the GMFM-66
721 Consented to Participate level. Use of the GMFCS requires famil- approximately every 6 months, and older
iarity with the child, but is not a test and children were assessed every 9 to 12
682 Provided Data requires no formal training. months. This timing was based on pre-
25 Excluded Motor function was assessed with the vious observations that led us to expect
24 Ineligible
1 Incomplete (Poor) Data
GMFM.16 The GMFM is a widely used, more rapid change in gross motor devel-
criterion-referenced, clinical observa- opment in the preschool years.8 On each
657 Included in Analyses tion tool with a scale from 0-100 that was occasion, therapists were also asked to
developed and validated for children with classify the childs current GMFCS level.
cerebral palsy or Down syndrome.17 It
has excellent reliability and demon- Analysis
Box. Gross Motor Function strated ability to evaluate meaningful To estimate the parameters of a non-
Classification System Levels change in gross motor function in chil- linear model of motor development,
for Children With Cerebral dren diagnosed as having cerebral nonlinear mixed-effects modelling22 was
Palsy Between the Ages palsy.16,18,19 The GMFM was not de- used for children in each of the 5
of 6 and 12 Years9 signed to compare the function of chil- GMFCS levels. Importantly, in addi-
Level I dren with cerebral palsy to typically de- tion to describing the average pattern
Walks without restrictions; limita- veloping children. It measures gross of development in each level, this analy-
tions in more advanced gross motor motor function in lying and rolling, sis allows for orderly variations in the
skills crawling and kneeling, sitting, stand- patterns of development. The degree of
Level II ing, and walk-run-jump activities. It can individual variations was estimated and
Walks without assistive devices; limi- be used with any child or adolescent di- individual motor development curves
tations in walking outdoors and in agnosed as having cerebral palsy. It fo- were fitted for each child. The model
the community cuses on the extent of achievement of a has 2 parametersthe estimated rate
variety of gross motor activities (mainly and limit of motor development
Level III
mobility skills and activities requiring that have straightforward clinical in-
Walks with assistive mobility de-
vices; limitations in walking out-
postural control such as sitting, kneel- terpretations. The model assumes that
doors and in the community ing, and standing on 1 foot) that a typi- children have GMFM-66 scores near
cally developing 5-year-old could ac- zero at birth. Subsequently, children are
Level IV complish. For data analyses, we used expected to acquire gross motor abili-
Self-mobility with limitations; chil- scores derived from the GMFM-66, a ties rapidly, with the rate of develop-
dren are transported or use power
measure with interval levels that was de- ment slowing as they approach the limit
mobility outdoors and in the com-
munity
veloped by Rasch analysis of the origi- of their potential.7,8 Based on clinical ex-
nal 88-item scale (GMFM-88).18-20 perience, the rate and limit of motor de-
Level V velopment are expected to vary sub-
Self-mobility is severely limited even Procedures and Quality Control stantially. Initial inspection of the data
with the use of assistive technology The ethics review boards of Hamilton suggested that this model might fit these
Health Sciences Corp, the Bloorview children well.
2002 American Medical Association. All rights reserved. (Reprinted) JAMA, September 18, 2002Vol 288, No. 11 1359

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PROGNOSIS FOR GROSS MOTOR FUNCTION IN CEREBRAL PALSY

RESULTS have been used to construct intervals data in Table 2 suggest a trend for a faster
Over the course of the study, 657 chil- that are expected to encompass 50% of progression to the limit as severity of im-
dren had a total of 2632 GMFM assess- the limits in the population. These in- pairment increases. However, the 95%
ments, or an average of 4 observations dividual differences in limit are plotted CIs indicate that children in levels III
per child. From these data, 5 distinct and in Figure 2. The 95% CIs are conceptu- through V progress significantly faster
significantly different motor growth ally different from and unrelated to the than children in level I, but children in
curves, which described patterns of gross 50% bands. The 95% CIs provide an es- level II do not progress faster than chil-
motor development by GMFCS level, timate of the precision of the point es- dren in level I. An earlier (younger)
were created (FIGURE 2 and FIGURE 3). timates of the mean limits, while the 50% age-90 does not indicate better devel-
Parameter estimates for the average bands provide clinical information about opmental progressonly that a child is
GMFM-66 curve in each level are re- the degree to which individuals are ex- closer to his/her limit, whatever that limit
ported in TABLE 2. As expected, the pected to vary around that mean. may be. To aid in clinical interpreta-
estimated limit of development de- To enhance interpretation, the rate pa- tion, the variation in age-90 (50% range)
creased as severity of impairment in- rameters from the nonlinear growth is reported as the interval expected to en-
creased. Confidence intervals (95% CIs) models have been transformed to age- compass 50% of age-90 in the popula-
for the limit parameters are tight and 90, the age in years by which children tion. Positive correlations between limit
confirm that each level of severity is sig- are expected to reach 90% of their mo- and age-90 suggest that there is a ten-
nificantly different from the adjacent lev- tor development potential. Smaller val- dency for children with lower motor de-
els. For clinical purposes, the esti- ues (in years) indicate faster progress to- velopment potential to reach their limit
mated variances in limit for each level ward motor development limits. Age-90 more quickly (ie, have a lower age-90)

Figure 2. Observed and Predicted Gross Motor Function Measure-66 (GMFM-66) Scores in Each Level of the Gross Motor Function
Classification System

Level I Level II Level III


100
90
80
70
GMFM-66 Score

60
50
40
30
20
10
0
0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
Age, y Age, y Age, y

Level IV Level V
100
90
80
70
GMFM-66 Score

60
50
40
30
20
10
0
0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
Age, y Age, y

The curved solid lines indicate average performance. The horizontal dotted lines on the right of the figures indicate the band expected to encompass 50% of childrens
limits of development. The solid vertical lines indicate the average age-90. The dotted vertical lines indicate the bands expected to encompass 50% of age-90 values
around the average. The absence of 50% bands in level IV and level V indicates low variation in age-90 values.

1360 JAMA, September 18, 2002Vol 288, No. 11 (Reprinted) 2002 American Medical Association. All rights reserved.

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PROGNOSIS FOR GROSS MOTOR FUNCTION IN CEREBRAL PALSY

than children with higher potentials, is a suggestion in Table 2 that SDs from GMFCS levels I through III, much later
even within GMFCS levels. the model predictions are larger in lev- in children in level IV, and rarely by chil-
The estimates of the average patterns els I and V than the middle levels. The dren in level V. The GMFM-66 item 69
of motor development in each stratum raw residual SDs were plotted against pre- (diamond C) measures a childs ability
and the degree of individual differences dicted values and against childrens ages to walk forward 10 steps unsupported,
around them (Table 2) have straightfor- in each GMFCS stratum to address the a task associated with a mean GMFM-66
ward clinical interpretations when com- adequacy of the model fit. This revealed score of 56, and achievable (50% chance)
bined with knowledge of childrens ini- no tendency for the model errors to be predominantly by children in GMFCS
tial GMFCS level. Thus, for example, the systematically related to predicted value levels I and II. Finally, the task of walk-
model predicts that the expected limit of or age. The residual SDs in Table 2 sug- ing down 4 steps alternating feet with
a childs potential in level III is 54.3 points gest that the size of the expected within-
on the GMFM-66 (ie, the level III mean), child errors may be related to the
with 50% of childrens limits being be- GMFCS, which supports the use of sepa- Figure 3. Predicted Average Development
by the Gross Motor Function
tween 48.5 and 60.0 points. In terms of rate models for each stratum. Classification System Levels
the rate of development, children in level To illustrate the clinical interpreta-
III are expected to have reached about tion of these curves, 4 selected GMFM-66 100
90% of their potential by about age 3.7 items have been identified on the ordi- 90 Level I

years. The positive correlation between nate of the curves (Figure 3). The GMFM 80 D

limit and age-90 for children in level III item 21 (diamond A) assesses whether 70 Level II

GMFM-66 Score
suggests that a young child, who is per- a child can lift and maintain his/her head 60 Level III
C
forming at a higher level than expected in a vertical position with trunk sup- 50
on the basis of the average level III curve, port by a therapist while sitting. A child 40
Level IV

is likely to level off sooner than his/her with a GMFM-66 score of 16 would be 30 B
Level V
peers. A substantial amount of prognos- expected to have a 50% chance of achiev- 20
tic information can thus be derived on ing this task.8 This is something that 10
A

the basis of a single GMFM-66 assess- would be seen relatively early in life 0
ment. The model incorporates possible among children in GMFCS levels I 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
classification errors within the GMFCS through IV, and only (on average) at Age, y
because these findings are based on chil- about age 2 years in children in level V.
The diamonds on the vertical axis identify 4 Gross Mo-
drens initial classification with no ef- The GMFM-66 item 24 (diamond B) as- tor Function Measure-66 (GMFM-66) items that pre-
fort to verify the childs true level if that sesses whether when in a sitting posi- dict when children are expected to have a 50% chance
happens to have changed over time. tion on a mat, a child can maintain sit- of completing that item successfully. The GMFM-66
item 21 (diamond A) assesses whether a child can lift
The residual SDs in Table 2 provide ting unsupported by his/her arms for 3 and maintain his/her head in a vertical position with
an indication of the degree to which the seconds. Children would be expected to trunk support by a therapist while sitting; item 24 (dia-
mond B) assesses whether when in a sitting position
model fits for each GMFCS level, and are have a 50% chance of being successful on a mat, a child can maintain sitting unsupported by
a measure of how much each childs at this task at an average GMFM-66 score his/her arms for 3 seconds; item 69 (diamond C) mea-
sures a childs ability to walk forward 10 steps unsup-
GMFM-66 score can be expected to vary of 32 points. This task would be rela- ported; and item 87 (diamond D) assesses the task of
around their true ability over time. There tively easily achieved by children in walking down 4 steps alternating feet with arms free.

Table 2. Parameters of Motor Development for Gross Motor Function Classification System (GMFCS)*
GMFCS Level

I II III IV V
(n = 183) (n = 80) (n = 122) (n = 137) (n = 135)
Mean No. of observations per child 4.0 4.4 4.1 3.9 3.8
GMFM-66 limit 87.7 68.4 54.3 40.4 22.3
95% CI 86.0-89.3 65.5-71.2 52.6-55.8 39.1-41.7 20.7-24.0
50% range 80.1-92.8 59.6-76.1 48.5-60.0 35.6-45.4 16.6-29.2
Age-90, y 4.8 4.4 3.7 3.5 2.7
95% CI 4.4-5.2 3.8-5.0 3.2-4.3 3.2-4.0 2.0-3.7
50% range 4.0-5.8 3.3-5.8 2.5-5.5 3.5 2.7
GMFM-66 limit/age-90 correlations 0.38 0.75 0.73 NA NA
Residual SDs 3.9 2.8 2.0 2.4 3.1
*GMFM-66 indicates Gross Motor Function Measure-66; CI, confidence interval; and NA, data not available because the variation in age-90 is near zero.
Age-90 is the age in years at which children are expected to achieve 90% of their potential GMFM-66 score.
The variation in age-90 was near zero, so the 50% range is approximately equal to the population average.

2002 American Medical Association. All rights reserved. (Reprinted) JAMA, September 18, 2002Vol 288, No. 11 1361

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PROGNOSIS FOR GROSS MOTOR FUNCTION IN CEREBRAL PALSY

arms free, which is GMFM-66 item 87 scribed,11,12 the mean measured change tions recent International Classification
(diamond D), will be observed at an av- on the GMFM-88 was in the range of of Functioning, Disability and Health
erage GMFM-66 score of 81 points, and about 3%. Intrathecal baclofen is increas- model.26 Furthermore, the GMFM-66 as-
probably only by children in GMFCS ingly being used for the management of sesses observed independent achieve-
level I. spasticity in individuals with cerebral ment of motor function tasks, but does
palsy. Although individuals have shown not (at least in this study) attempt to
COMMENT improvements in spasticity and pain re- evaluate the ways in which childrens
The patterns of motor development in lief after receiving intrathecal baclofen, function is performed with or might be
children with cerebral palsy are the first its effect on measured change in gross enhanced through the addition of aug-
to be based on longitudinal observa- motor function is limited.24 These com- mentative and technical interventions
tions. They were created using a valid ments are in no way meant to minimize such as aids, orthoses, or the use of pow-
classification system of functional abili- the effectiveness of these interventions ered mobility to increase day-to-day in-
ties and limitations of children with ce- for specific subgroups of children with dependence.27 Children may change and
rebral palsy and a systematic evalua- cerebral palsy, but rather to note that at improve their gross motor performance
tion of gross motor function with an this time these interventions do not, on over the developing years through in-
evaluative clinical instrument (GMFM). average, have a major impact on func- creased balance, stamina, energy effi-
Data were collected from a large strati- tion as assessed with the GMFM. ciency, and quality of motor control
fied random sample of children diag- We expect that the findings from our all features that are important and should
nosed as having cerebral palsy, who were study will help parents understand the be evaluated, but are beyond the scope
receiving a range of accepted medical, outlook for their childs gross motor of the GMFM-66.
orthopedic, and developmental therapy function, because an evidence-based es- Thus, it is extremely important that
services. We believe the sample is rep- timate can now be made about gross mo- parents, physicians, therapists, pro-
resentative of the population of chil- tor prognosis based on age and GMFCS gram managers, third-party payers, and
dren with cerebral palsy in Ontario, with level. The data should prove equally use- other decision makers not assume fur-
results generalizable to populations else- ful to clinicians planning interventions, ther therapy is unhelpful or unneces-
where receiving similar types of mixed enabling clinicians and parents to make sary when the curves appear to level off.
developmental therapies. informed decisions about the most ap- Continuing efforts should be made to ad-
It is not clear whether children who propriate therapy goals for children. The dress ways both to increase indepen-
are currently receiving newer therapeu- curves also provide an effective way to dent activity and to promote participa-
tic modalities (selective dorsal rhi- assess whether a childs motor progress tion of children with disabilities, as well
zotomy,10 botulinum toxin,11,12 intra- is consistent with patterns observed in as to address secondary impairments that
thecal baclofen 1 3 ) might perform children of similar age and severity. may arise. It should also be remem-
substantially better than the children in- Because the GMFM-66 assessments of bered that the children in the present
volved in this study, and if so, whether children reported here were specifically study were receiving a range of contem-
this would limit the generalizability of made without the use of aids, such as porary developmental therapy services
our findings. It should be noted that all walkers or crutches, these patterns of that we believe are representative of the
of these recent therapeutic innovations gross motor development probably rep- therapies provided in the Western world.
are used only with highly selected sub- resent the lower limit of what children It is likely that as new therapies emerge,
groups of children with cerebral palsy, in each level can, on average, accom- patterns of motor development in chil-
and that even the best results apply only plish in gross motor function. Further- dren diagnosed as having cerebral palsy
to those specific groups and not to the more, the curves appear to reach pla- may change and modifications to these
whole population. teaus by about age 7 years. Children, on models will be needed. We believe that
For example, following selective dor- average, reach about 90% of their mo- the motor development curves will have
sal rhizotomy, the reported improve- tor function (as measured by the GMFM- important applications for the evalua-
ments in gross motor function are sta- 66) by around age 5 years or younger, tion of specific interventions by permit-
tistically significantly greater than those depending on their GMFCS level. ting analysis of the extent to which these
seen with physical therapy alone, but the However, the curves reveal nothing interventions improve a childs gross mo-
actual measured GMFM-66 changes are about the quality of motor control used tor function beyond what is predicted
still quite modest (mean measured added to accomplish the activities, which is an based on age and GMFCS level.
benefit in GMFM-66 change scores in the aspect of motor development that ap- Based on our previous work, an as-
[selective dorsal rhizotomy plus therapy] pears to emerge later in childhood.25 Nor sumption of this study was that GMFCS
group was 2.6)23 and are unlikely to be do the curves show how children apply levels are stable over time, making prog-
associated with a change of GMFCS level. their motor function in the context of ac- nostication meaningful. Wood and
Similarly, while the effects of botuli- tivity or participation in daily life, as for- Rosenbaum15 demonstrated an overall
num toxin injections have been well de- mulated in the World Health Organiza- reliability of GMFCS over time (from age
1362 JAMA, September 18, 2002Vol 288, No. 11 (Reprinted) 2002 American Medical Association. All rights reserved.

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PROGNOSIS FOR GROSS MOTOR FUNCTION IN CEREBRAL PALSY

2 years to 12 years) of 0.79, with about the impact of puberty and the de- 10. Steinbok P. Outcomes after selective dorsal rhi-
zotomy for spastic cerebral palsy. Childs Nerv Syst.
higher values when one tracked the con- mands of secondary school on motor 2001;17:1-18.
sistency of GMFCS levels from age 2 to function and activity of adolescents with 11. Mall V, Heinen F, Kirschner J, et al. Evaluation of
botulinum toxin. J Child Neurol. 2000;15:214-217.
4 years to age 12 years (0.82) or from cerebral palsy. Finally, we expect that re- 12. Kirschner J, Linder M, Berweck S, et al. Treat-
age 4 to 6 years to age 12 years (0.87). search by our group and others will pro- ment of pes equines in cerebral palsy with botulinum
vide validation of the accuracy and util- toxin A and functional benefit according to gross mo-
Children in our study were allocated to tor function measure. Eur J Neurol. In press.
a GMFCS level at entry to the study, and ity of these curves. 13. Almeida GL, Campbell SK, Girolami G, Penn R,
data were analyzed according to that ini- Corcos DM. Multidimensional assessment of motor
Author Contributions: Study concept and design: function in a child with cerebral palsy following intra-
tial assignment. This was done to re- Rosenbaum, Walter, Palisano, Russell, Wood. thecal administration of baclofen. Phys Ther. 1997;
flect the clinical reality that parents seek Acquisition of data: Rosenbaum, Walter, Russell, Raina, 77:751-764.
Galuppi. 14. Collet JP, Vanasse M, Marois P, et al. Hyperbaric
prognostication about their childs out- Analysis and interpretation of data: Rosenbaum, oxygen for children with cerebral palsy. Lancet. 2001;
look from the time the disability is first Walter, Hanna, Palisano, Raina, Bartlett. 357:582-586.
Drafting of the manuscript: Rosenbaum, Walter, 15. Wood EP, Rosenbaum PL. The Gross Motor Func-
diagnosed. As development progresses, Hanna, Palisano, Raina. tion Classification System for cerebral palsy. Dev Med
children may be reclassified. If this oc- Critical revision of the manuscript for important in- Child Neurol. 2000;42:292-296.
tellectual content: Rosenbaum, Walter, Hanna, 16. Russell DJ, Rosenbaum PL, Cadman DT, et al. The
curs, it is important for clinicians to re- Palisano, Russell, Raina, Wood, Bartlett, Galuppi. gross motor function measure. Dev Med Child Neu-
formulate the prognosis, based on the Statistical expertise: Walter, Hanna, Raina. rol. 1989;31:341-352.
Obtained funding: Rosenbaum, Walter, Palisano, 17. Russell D, Palisano R, Walter S, et al. Evaluating
most recent assessment of a childs mo- Russell. motor function in children with Down syndrome. Dev
tor activities and GMFCS stratum. Administrative, technical, or material support: Med Child Neurol. 1998;40:693-701.
Rosenbaum, Palisano, Raina, Bartlett, Galuppi. 18. Russell DJ, Avery L, Rosenbaum PR, Raina P, Wal-
The present data can be used to ex- Study supervision: Rosenbaum, Russell. ter SD, Palisano RJ. Improved scaling of the gross mo-
plore the creation of motor growth Funding/Support: This study was made possible by tor function measure for children with cerebral palsy.
curves for children with different dis- grant MT-13476 from the Canadian Institutes of Health Phys Ther. 2000;80:873-885.
Research (CIHR) (formerly the Medical Research Coun- 19. Russell DJ, Rosenbaum PL, Avery L, Lane M. Gross
tributions of cerebral palsy, as has been cil of Canada) and grant R01-HD-34947 from the Na- Motor Function Measure (GMFM-66 and GMFM-
done by others.28 These analyses are tional Center for Medical Rehabilitation Research of 88) Users Manual: Clinics in Developmental Medi-
the National Institute of Child Health and Human De- cine, No. 159. London, England: Mac Keith Press; 2002.
currently under way. However, there is velopment. 20. Avery L, Russell D, Raina P, Rosenbaum P, Wal-
reason to believe that in the absence of Previous Presentation: A preliminary report of the find- ter S. Rasch analysis of the gross motor function mea-
ings of this study was presented at the European Acad- sure. Arch Phys Med Rehabil. In press.
a systematic, protocol-driven classifi- emy for Childhood Disability, Go teborg, Sweden, 21. Russell DJ, Rosenbaum PL, Lane M, et al. Train-
cation of the topographical compo- October 13, 2001. ing users in the gross motor function measure. Phys
Acknowledgment: We thank the children and fami- Ther. 1994;74:630-636.
nents of cerebral palsy, reliability of lies, whose ongoing participation made this study pos- 22. Pinheiro JC, Bates DM. Mixed Effects Models in
such categorizations is relatively poor.29 sible. We also acknowledge the considerable contri- S and S-PLUS. New York, NY: Springer; 2000.
For the same reason, we have not yet butions of the coordinators of the study at each center, 23. McLaughlin J, Bjornson K, Temkin N, et al. Se-
and the tireless work of more than 125 therapists who lective dorsal rhizotomy: meta-analysis of three ran-
analyzed the findings according to the assessed children during the course of this study. domized controlled trials. Dev Med Child Neurol. 2002;
form of motor impairment (spastic, dys- 44:17-25.
24. Butler C, Campbell S. Evidence of the effects of
tonic, ataxic, or mixed), which has been REFERENCES
intrathecal baclofen for spastic and dystonic cerebral
described elsewhere.30 In fact, approxi- 1. Stanley FJ, Blair E, Alberman E. Cerebral Palsies: palsy: AACPDM Treatment Outcomes Committee Re-
Epidemiology & Causal Pathways. London, England: view Panel. Dev Med Child Neurol. 2000;42:634-
mately 76% of the children in this study Mac Keith Press; 2000:29. 645.
were described as having spastic cere- 2. Mutch LW, Alberman E, Hagberg B, Kodama K, 25. Boyce W, Gowland C, Rosenbaum P, et al. The
Velickovic MV. Cerebral palsy epidemiology. Dev Med gross motor performance measure: validity and re-
bral palsy, with much smaller num- Child Neurol. 1992;34:547-555. sponsiveness of a measure of quality of movement.
bers in the other subgroups. 3. Bleck EE. Locomotor prognosis in cerebral palsy. Phys Ther. 1995;75:603-613.
The curves describe patterns for Dev Med Child Neurol. 1975;17:18-25. 26. World Health Organization. International Classi-
4. Molnar GE, Gordon SV. Predictive value of clinical fication of Functioning, Disability and Health. Geneva,
groups of children. There is within- signs for early prognostication of motor function in cere- Switzerland: World Health Organization; 2001. Avail-
stratum variation in motor develop- bral palsy. Arch Phys Med Rehabil. 1974;57:153-158. able at: http://www3.who.int/icf/icftemplate.cfm. Ac-
5. Watt JM, Robertson CM, Grace MG. Early prog- cessibility verified August 26, 2002.
ment, which is based on other aspects nosis for ambulation of neonatal intensive care sur- 27. Butler C. Augmentative mobility: why do it? Phys
of each childs functional status.31,32 More vivors with cerebral palsy. Dev Med Child Neurol. Med Rehabil Clin North Am. 1991;2:801-816.
1989;31:766-773. 28. Beckung E, Carlsson G, Carlsdottor, Uvebrant P.
research is needed to understand, and 6. Crothers B, Paine RS. Natural History of Cerebral The natural history of gross motor function in chil-
to be able to measure accurately, the im- Palsy. Cambridge, Mass: Harvard University Press; 1959. dren with cerebral palsy in western Sweden. Dev Med
7. Scrutton D, Rosenbaum PL. The locomotor devel- Child Neurol. 2001;43(suppl 89):27.
pact of factors such as a childs visual opment of children with cerebral palsy. In: Connolly 29. Blair E, Stanley F. Interobserver agreement in the
ability, cognitive capacity, motivation, K, Forssberg H, eds. Neurophysiology and Neuropsy- classsification of cerebral palsy. Dev Med Child Neu-
parental encouragement, and the con- chology of Motor Development. London: Mac Keith rol. 1985;27:615-622.
Press; 1997:101-123. 30. Cans C. Surveillance of cerebral palsy in Europe:
tribution of therapies that might be as- 8. Palisano RJ, Hanna SE, Rosenbaum PL, et al. Vali- a collaboration of cerebral palsy surveys and regis-
sociated with individual variation in dation of a model of gross motor function for children ters. Dev Med Child Neurol. 2000;42:816-824.
with cerebral palsy. Phys Ther. 2000;80:974-985. 31. Kennes J, Rosenbaum PL, Hanna SE, et al. Health
progress. It will also be important to con- 9. Palisano RJ, Rosenbaum PL, Walter SD, Russell DJ, status of school-aged children with cerebral palsy. Dev
tinue to follow the motor development Wood EP, Galuppi BE. Development and reliability of Med Child Neurol. 2002;44:240-247.
a system to classify gross motor function in children 32. Bartlett DJ, Palisano RJ. A multivariate model of
of these children through adolescence, with cerebral palsy. Dev Med Child Neurol. 1997;39: determinants of motor change for children with ce-
because much remains to be learned 214-223. rebral palsy. Phys Ther. 2000;80:598-614.

2002 American Medical Association. All rights reserved. (Reprinted) JAMA, September 18, 2002Vol 288, No. 11 1363

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