CEREBRAL PALSY
CATALINA RUIZ, MD
12/06/12
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DEFINITION
ETIOLOGY
CLASSIFICATION AND CLINICAL FEATURES
ASSOCIATED SYNDROMES
DIAGNOSIS
TREATMENT
PROGNOSIS
DEFINITION
• Difficulty in motor control
• Non-progressive brain lesion
• Injury to the brain before it was fully mature
(prenatally, perinatally, postnatally)
• Abnormalities to motor activity and posture
• Voluntary movements are supposed to be
complex, coordinated and varied.
• In CP they are uncoordinated, stereotypic and
limited (is a spectrum of symptoms, mild to
severe)
• Diagnosis is difficult before 6 months of age,
most of the movements are reflex in origin
and don’t involve the voluntary motor cortex
ETIOLOGY
• Prematurity
• Intrauterine growth
restriction
• Intrauterine infection
• Antepartum
hemorrhage
• Severe placental
pathology
• Multiple pregnancy
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Perinatal asphyxia
Intracranial hemorrhage
Kernicterus
Near drowning
Trauma
Stroke
CLASSIFICATION
• Based upon the type and distribution of
motor abnormalities.
• Spastic (diplegia, hemiplegia, quadriplegia)
• Dyskinetic (dystonic, athetoid)
• Ataxia (simply ataxia, ataxic diplegia)
TO REMEMBER…
• TONE: is the continuous and passive partial
contraction of the muscles.
• Maneuvers to test muscle tone and normal
responses include:
• Holding the infant under the arms – he/she
will assume a sitting position; truncal
resistance allows the infant to be easily
supported without slipping through the
examiner's hands
• Passive extension of the legs at the knees –
resistance is met at approximately 90º.
• Pulling the infant from the supine to sitting
position – only a slight head lag is expected; the
head should wobble in the midline for a few
seconds when the sitting position is reached.
• Holding the infant in horizontal suspension –
he/she flexes the limbs, straightens the back, and
maintains the head in the midline for a few
seconds.
CLASSIFICATION
1. SPASTIC SYNDROMES
• Spastic hypertonia (clasp-knife)
• Hyperreflexia caused by hyperexcitability of the
stretch reflex
• Extensor plantar responses
• Clonus
• Slow effortful voluntary movements
• Impaired fine-motor function
• Difficulty in isolating individual movements
• Fatigability
SPASTIC
SPASTIC DIPLEGIA
• Affects both term and preterm infants
• In preterm infants often is associated with
periventricular leukomalacia
• The lower limbs are affected predominantly
• Atrophy below the waist occurs in many
patients
SPASTIC HEMIPLEGIA
• Typically affects term infants of normal birth
weight
• The presentation of congenital spastic
hemiplegia is variable
• It is usually caused by a cortical lesion.
• The arm typically is more affected than the leg
SPASTIC HEMIPLEGIA
• Suspect when: early hand dominance,
reduced movement, or abnormal posturing on
one side
• movement and tone on the affected side
typically decrease before tone and tendon
reflexes abnormally increase.
SPASTIC QUADRAPLEGIA
• Most severe form of spastic CP.
• It usually affects term infants who are small
for gestational age.
• Affected infants typically are severely
handicapped. In addition to spasticity, they
may have dystonia and feeding and
respiratory difficulties because of
pseudobulbar palsy
SPASTIC QUADRAPLEGIA
• Associated findings hip subluxation,
contractures, and scoliosis, intellectual
disabilities, visual impairment.
2. DYSKINETIC SYNDROMES
• Neuronal necrosis in the hippocampus,
thalamus, basal ganglia, reticular formation,
and Purkinje cells of the cerebellum.
• The neonatal presentation includes
encephalopathy characterized by lethargy,
decreased spontaneous movement,
hypotonia, and suppressed primitive reflexes.
DYSKINETIC SYNDROMES
DYSKINETIC SYNDROMES
• There are 2 types:
1. Athetoid: movements are athetoid,
choreiform, or a combination of both.
1. Dystonic
DYSKINETIC SYNDROMES
• ATHETOID MOVEMENTS: slow, smooth,
writhing movements that involve distal
muscles athetosis – YouTube
• Athetosis is most apparent during reaching, as
the fingers extend and abduct.
DYSKINETIC SYNDROMES
• CHOREA: rapid, irregular, unpredictable
contractions of individual muscles or small
muscle groups that involve the face, bulbar
muscles, proximal extremities, and fingers and
toes.
• Stress, excitement, or fever may exacerbate
the chorea CHOREA
DYSKINETIC SYNDROMES
• DYSTONIC: repetitive, patterned, twisting, and
sustained movements of the trunk and limbs
that may be either slow or rapid. DYSTONIA
• The muscle tone typically is normal or
hypotonic, especially in early childhood.
• Infants tend to have retention of primitive
reflexes, involuntary grimacing, a tendency to
drool, and delayed psychomotor
development.
DYSKINETIC SYNDROMES
• The involuntary movements evolve with time and
may not be apparent until the child is two to
three years of age. Unlike spastic CP, contractures
usually do not develop, unless they are
positional.
• Patients often have more than one form of
involuntary movement, and the types may
overlap in some cases. The extent of dysarthria
and motor and intellectual disability are variable.
3. ATAXIA
• Although ataxia indicates an incoordination of
cerebellar or sensory origin, ataxic CP
represents a widespread disorder of motor
function. Ataxia
• The etiology of ataxic CP is heterogeneous.
Most cases are caused by early prenatal
events.
ATAXIA
• Most patients have congenital hypotonia.
• Motor milestones and language skills typically
are delayed. Ataxia usually improves with
time.
• Speech, which is related to intellectual ability,
typically is slow, jerky, and explosive.
• The diagnosis of ataxic CP is made by
exclusion.
ASSOCIATED CONDITIONS
INTELLECTUAL DISSABILITY: 52% of patients.
Severity correlates with the extent of motor
handicap.
Children with spastic quadriplegia are typically
the most severely affected
PSYCHIATRIC DISORDERS: emotional lability,
poor attention and vigilance, and obsessivecompulsive traits
ASSOCIATED CONDITIONS
EPILEPSY: 45% of patients.
Most common in patients with spastic quadriplegia
and acquired hemiplegia.
The onset of seizures is typically during the first two
years of life.
Partial seizures with secondary generalization are
the most common type.
Intellectual disability is more common in CP
patients with seizures than in those without
seizures
ASSOCIATED CONDITIONS
VISUAL DISORDERS: strabismus and refraction
errors are the most common ones. Ambliopia
and visual fields defects can also be present.
Low visual acuity may be due to cortical
impairment.
ASSOCIATED CONDITIONS
SPEECH IMPAIRMENT : aphasia and dysarthria
(38%).
Abnormal function of oropharyngeal muscles
and lack of coordination of breathing patterns
contribute.
ASSOCIATED CONDITIONS
HEARING IMPAIRMENT: 10-20%.
It is most common in those with very low
birthweight or severe hypoxic-ischemic insults.
Sensorineural hearing loss is also a feature of CP
caused by kernicterus.
ASSOCIATED CONDITIONS
GROWTH FAILURE: mostly due to poor nutrition.
Poor nutritional status is caused by both
inadequate intake and
gastrointestinal abnormalities
ASSOCIATED CONDITIONS
PULMONARY DISEASE: leading cause of death.
Poor coordination of respiratory muscles and
chest deformity.
Recurrent aspiration (reflux), restrictive disease
(scoliosis).
ASSOCIATED CONDITIONS
ORTHOPEDIC DISORDERS: subluxation,
dislocation, and progressive dysplasia of the
hip, foot deformities, and scoliosis.
Osteopenia.
ASSOCIATED CONDITIONS
URINARY DISORDERS: dysfunctional voiding
symptoms, including enuresis, frequency,
urgency, and stress incontinence.
Neurogenic bladder and incontinence.
DIAGNOSIS
• Combination of findings, including motor
delay, neurologic signs, persistence of
primitive reflexes, and abnormal postural
reactions.
• Infants with an abnormal obstetric or
perinatal history may be at increased risk to
develop CP, and should be monitored closely.
DIAGNOSIS
• CLUES:
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Psychomotor delay
Abnormal oromotor or oculomotor patterns
Hypotonia/hypertonia
Hyperreflexia, clonus
DIAGNOSIS
DIFERENTIAL DIAGNOSIS
CP is a diagnosis of exclusion.
• Neurodegenerative diseases
• Inborn errors of metabolism
• Neuromuscular or movement disorders
• Neoplasm
TREATMENT
• Multidisciplinary treatment
• Psychological development, communication,
and education are priorities in the
management .
• Interventions should be directed to maximize
the quality of life.
TREATMENT
• PT/OT
• Botulism toxin
• Antispastic drugs (Dantrolene, benzos,
Baclofen)
• Surgery (muscle-tendon, hip)
• Constraint-induced movement therapy (CIMT)
• Gastrostomy feeding
TREATMENT
• Motorized wheelchairs, switching devices
used to activate communication systems,
voice-activated computers
• Braces
• Psychological support
PROGNOSIS
• The majority of children with CP survive to
adulthood.
• Survival of adults with CP also depends upon
the degree of disability.
• The prognosis for survival and motor function
in children with CP is highly variable, and
depends on the severity and type.
PROGNOSIS
• The prognosis for walking is poor in children
who do not achieve head balance by 20
months, retain primitive reflexes or have no
postural reactions by 24 months, or do not
crawl by approximately five years of age. The
prognosis for walking is good in children who
sit by two years and crawl before 30 months
of age.
REFERENCES
• UP TO DATE
• NELSON
• PEDS IN REVIEW