Abnormalities of Tone

and Movement in
Children
Rosalina Q. De Sagun, M.D.
Maria Antonia Aurora M. Valencia,M.D.
Department of Pediatrics
Department of Neurology and
Psychiatry

Developmental Delay
Delayed

acquistion of milestones
expected for chronological age.
Important to distinguish from
Progressive Neurological Disorders
which manifests as LOSS of previously
acquired skills.
Delays can involve any developmental
parameter/s: Motor, Language,
Psychosocial

Localization of Weakness
UM
N

LMN

Bulk
Tone
DTRs

Upper Motor
Neuron
Minimal
Atrophy
Increased ;
spastic
Hyperreflexia

Fasciculations Absent
Babinski
Sensory
Deficit

Present
May be
present

Lower Motor
Neuron
Profound
Atrophy
Decreased;
flaccid
Decreased/
Absent
Present/
Absent
Absent
May be
present

Clinical Clues
1. Central nervous system
Upper motor neuron
(spasticity, hyperreflexia); may be
accompanied by cerebral manifestations
(seizures, cognition, language and
sensory problems)
2. Peripheral nervous system
Lower motor neuron
(decreased to absent reflexes, flaccid)

Disorders of the motor system may

be:
1. Acute - stroke/vascular
metabolic disorders
infection
seizures
2. Chronic - cerebral palsy
(static)
congenital CNS lesion
degenerative disorders
(progressive)

CEREBRAL PALSY

Refers to a group of disorders characterized

by motor abnormalities (tone, posture or
movement) which are neither progressive
nor episodic.

The brain lesions are static and result from

disorders of early brain development, usually
insults in the perinatal period.

They are not progressive but the symptoms

may change in time.

Cerebral Palsy (CP)

Impairment in movement and posture

leading to functional deficits and the
inability to perform activities of daily
living
Caused by a broad group of
developmental, genetic, metabolic,
ischemic, infectious and other
etiologies that produce a common
neurologic phenotype

Epidemiology and Etiology

Most common and costly form of
chronic motor disability with a
prevalence of 2/1000
<10% are due to perinatal asphyxia.
80% pointed to antenatal events
causing abnormal CNS development
Intrauterine exposure to maternal
infection ( UTI and chorioamnionitis)
LBW and those < 1,000 g increased
risk for ICH and PVL

CEREBRAL PALSY
Clinical manifestations:
1. Delay in development i.e. poor head
control, delays in gross motor or fine motor
development
2. Motor deficit depending on the area of
the
brain involved and usually the risk
factors present
3. Associated developmental disabilities
mental retardation, epilepsy, visual,
hearing, speech and behavioral
abnormalities

Motor Disorders in CP
Three main criteria in classification:
1. Type of motor disorder
2. Topographical distribution
3. Gross motor function

Types of Cerebral Palsy and the

Major Causes
Physiologic
Spastic
Athetoid
Rigid
Ataxic
Tremor
Atonic
Mixed
Unclassified

Topographic
Monoplegia
Paraplegia
Hemiplegia
Triplegia
Quadriplegi
a
Diplegia
Double
hemiplegia

Etiologic
Prenatal

Functional
Class I

infection,
metabolic,
anoxia, toxic,
genetic,
infarction

no limitation of
activity

Class II

Perinatal

slight to
moderate
limitation

anoxia

Class III

moderate to
great
toxins, trauma,
limitation
infection

Postnatal

Class IV
no useful
physical

Motor Syndrome

Neuropathology

Major Causes

Spastic diplegia

Periventricular
leukomalacia

Prematurity
Ischemia
Infection
Endocrine
/metabolic/ genetic

Spastic
Quadriplegia

PVL/ Multicystic
encephalomalacia,
Malformations

Same as above

Hemiplegia

Stroke in utero or
neonatal

Thrombophilic
disorders Infection
Genetic
Hemorrahgic
Infarction

Extrapyramidal/
Athetoid

Pathology in the
basal ganglia,
putamen, globus
pallidus, thalamus

Asphyxia
Kernicterus
Mitochondrial
Genetic/Metabolic

Quadriplegia

Diplegia

More Affected
Hemiplegia

Triplegia

Monoplegia

Less Affected

Clinical Manifestations of
CP
Movement disorders
Spasticity
Athetosis
Dystonia
Rigidity
Ataxia
Mixed motor problems
2. Associated with a spectrum of
developmental diasabilities: Mental
retardation, epilepsy, hearing and visual
problems, speech, cognitive and behavioral
1.

Physiologic Classification

Hypotonic Cerebral
Palsy

Physiologic classification
Hypotonia

Physiologic Classification

Hypotonic Cerebral
Palsy

Physiologic Classification

Spastic Diplegic
Cerebral Palsy

Spastic
Cerebral Palsy

Spastic Quadriplegic
Cerebral Palsy

Spastic Diplegic
Cerebral Palsy

Spastic Quadriplegia
Most

severe form because of

involvement of ALL four limbs and
the HIGH association of mental
retardation and seizures.
Feeding problems are common due
to supranuclear bulbar palsies.

Athetoid
Cerebral Palsy

Athetoid CP
Less

common than the spastic type

This is the type most likely
associated with birth asphyxia
Hypotonic infants who do not have
UMN signs but later on develop
increased variable tone (rigidity) and
dystonia and other dyskinesias
Intellect is preserved in many cases

Athetoid
Cerebral Palsy

Ataxic
Cerebral Palsy

Diagnosis

Cerebral Palsy is a clinical diagnosis

Neuroimaging will document the

extent of the structural pathology,
aid in diagnosis and prognostication
and rule out treatable causes and
slowly progressive neurological
disorders.

Diagnosis
Thorough history to identify risk factors,
developmental assessment, physical and
neurological examinations
Hearing and vision screening
EEG if with seizures
If no possible etiology or risk factors for CP,
do diagnostic tests as:
Neuroimaging CT/MRI
Metabolic screening,
Chromosomal studies

Differential Diagnosis
1. Motor delays from congenital structural
malformations
2. Progressive disorders of the brain
white
matter diseases
3. Muscle disorders- myopathies,
dystrophies.
4. Anterior horn cell disease- spinal
muscular atrophy (SMA)

Differentials
1.

Dysmyelinating / demyelinating disorders

- Abnormal myelin formation as in some
metabolic disorders
- Abnormal myelin secondary to brain
insults from infection, trauma, autoimmune.
Clinically: - White matter involvement with
progressive neurologic deficits ( spasticity,
hyperreflexia, hearing and vision may be
affected.)

2. Spinal Muscular atrophy

- disorders of the anterior horn cell
- usually progressive
- of 3 varieties depending on the
age of onset of symptoms
Infantile type Werdnig-Hoffman
disease
Intermediate
Juvenile - Kugelberg Welander disease
Present with LMN signs: fasciculations,
floppy, areflexic

Differentials
Muscle - Congenital myopathy
4. Nerve disorders
- manifestations of lower motor
disease, decreased reflexes, tone
(e.x. Hereditary Motor-Sensory
Neuropathy)
3.

CP
Effective

management requires
1. Understanding of the
pathophysiology
of the disorder
2. Careful assessment of the patient s
capabilities and limitations
3. Knowledge of available treatment
regimens, their applications and
limitations

Management
Multidisciplinary
1.
2.

3.
4.
5.
6.
7.

Pediatrician
Neurologist- management of seizures,
botulinum toxin injections
Rehabilitation specialists
Physical and occupational therapists
Developmental psychologists
Education specialists
Orthopedic surgeons
Social workers

Autism Spectrum Disorder

Autism

is a neurodevelopmental
disorder of unknown etiology but with
a strong genetic basis.
Behavioral phenotype:
Qualitative impairment in language/
communication
Impaired Social interactions and
reciprocity
Lack of Imaginative play

Signs and Symptoms

No

pathognomonic symptom or behavior

Most will present with:
Impairment in joint attention ( use of eye
contact and pointing to share experiences
with others) Normally develops at 18 months
Lack of protoimperative pointing ( to get an
object of desire)
Lack of protodeclarative pointing ( to share
an object of interest/ naming)
Lack of imaginative play

Manifestations
Poor

eye contact
Verbal abilities vary: non-verbal to
advanced speech
But speech may have odd prosody or
intonation, echolalia, pronoun
reversal, non-sense rhyming
IQ from MR to superior functioning
Stereotypical/ ritualistic behaviors
Marked need for sameness

Diagnosis
DSM

IV Criteria for Autism

Modified Checklist for Autism in
Toddlers (M-CHAT)
Pervasive Developmental Disorder
Screening Test (PDDST)

Treatment
Multidisciplinary
Developmental

pediatrician, Pediatric
Neurologist/ Child Psychiatrist
Psychologist
Occupational/ Speech therapist
Special Education Teacher (if necessary)
Medications depending on co-morbid
conditions

Attention Deficit
Hyperativity Disorder
(ADHD)
Most common neurobehavioral disorder in
childhood
Characterized by (DSM IV):

(1) Inattention
(2) Poor impulse control
(3) Motor overactivity / restlessness
Symptoms should be present for more than 6
months in at least 2 settings ans significantly
affects social, academic or occupational
functioning.

Etiology
Multifactorial
Genetic

susceptobility
Maternal complications during pregnance
Maternal smoking amnd alcohol intake
during pregnancy
Abnormal brain structures
Psychosocial family stressors exacerbate
and/ or contribute to the symptoms.

Pathogenesis
Smaller

prefrontal cortex and basal

ganglia with 5-10% less blood flow in
these areas.
These areas are rich in dopamine
receptors led to the dopamine
hypothesis disturbances in
dopamine system are related to the
onset of ADHD

Diagnosis
Clinical

interview and history

Fulfills criteria
Family Hx of ADHD
Family discord, situational stress
Behavior

rating scales ( e.x. Conners

rating scale), helps establish the
magnitude and pervasiveness of
symptoms and can be used for monitoring
improvement with intervention

Treatment
Psychosocial

treatments
Behaviorally oriented treatments
Medications : Methylphenidate and
Atomoxetine

Prognosis
Persists

throughout the life span

Reduction in hyperactive behavior with
age
Other symptoms: Impulsivity,
disorganization and inattention become
prominent affecting relationships and
occupational functioning
High risk for risk taking behaviors
( substance abuse) and psychiatric dso.

Thank
you!