Cerebral Palsy
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Cerebral Palsy
Basics
DESCRIPTION
Cerebral palsy (CP) describes a group of disorders of movement and posture, limiting
activity, attributed to nonprogressive underlying brain pathology. The motor
disorders of CP are often accompanied by disturbances of sensation, cognition,
communication, perception, and/or behavior, or by a seizure disorder.
Spastic (pyramidal; 40%): Increased deep tendon reflexes, sustained clonus,
hypertonia, and the clasp-knife response:
1. Spastic diplegia –lower extremity involvement
2. Spastic hemiplegia –1 side of the body involved
3. Spastic quadriplegia –total body involvement
Dyskinetic (30%): Fluctuating tone, rigid total body involvement by definition.Persistent primitive reflex patterns (asymmetric tonic neck reflex,
labyrinthine)
1. Athetoid: Slow writhing movements (or chorea; rapid, random, jerky
movements)
2. Dystonic: Posturing of the head, trunk, and extremities
Ataxic (<10%): Characterized by cerebellar signs (ataxia, dysmetria, past
pointing, tremor, nystagmus) and abnormalities of voluntary movement
Mixed (10%): 2 or more types codominant, most often spastic and dyskinetic Other (10%): Criteria for CP met, but specific subtype cannot be defined
Extrapyramidal: Sometimes applied to nonspastic types of CP as a group
EPIDEMIOLOGY
~50% of cases are associated with prematurity.
Increased concordance among monozygotic versus dizygotic twins in some
studies (not in others)
Intrauterine growth retardation (IUGR) more common in CP than controls,
especially for full-term infants in whom CP develops
Male > Female (1.3:1)
Inconsistent relationship to maternal age, socioeconomic status, and parity
Prenatal factors are more strongly associated with subsequent CP than are
perinatal or postnatal factors; however, individual risk factors are poorly
predictive of subsequent CP in the individual child.
Diagnosis of perinatal asphyxia requires evidence of hypoxic –ischemic insult,
severe encephalopathy (e.g., neonatal seizures, severe hypotonia), consistent
laboratory/radiological findings; accounts for only ~9% of cases of CP.
OTHER
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Increased with multiple gestation (10% were twins in 1 study)
Prevalence ~2/1000
ETIOLOGY
Not apparent in most cases. A more recently recognized perinatal factor is the
presence of chorioamnionitis; mild or even subclinical cases may have
increased association with CP.
Epidemiologic studies indicate 2 types of vulnerability to CP:
1. Prematurity: Vulnerability of the periventricular white matter
between 28 and 32 weeks of gestation results in periventricular
leukomalacia.
2. IUGR: Fetal growth retardation associated with CNS dysgenesis,
non-CNS malformation, teratogens, growth retardation, evidence of
hypoxic-ischemic encephalopathy more often seenBASICS-ASSOCIATED-CONDITIONS
Sensory:
1. Sensorineural and conductive hearing loss
2. Impaired visual acuity
3. Oculomotor dysfunction
4. Strabismus
5. Cortical visual impairment
6.
Somatosensory impairments Cognitive and linguistic:
1. Mental retardation (MR) in ~50%, especially in spastic quadriparesis
2. High incidence of language and learning disabilities
3. Dysarthria
4. Attention deficit hyperactivity disorder
5. Sleep and behavioral disturbances
Neurologic:
1. Seizures
2. Hydrocephalus
Musculoskeletal:
1. Contractures
2. Hip subluxation/dislocation
3. Scoliosis
Cardiorespiratory:
1. Upper airway obstruction
2. Aspiration pneumonitis
3.
Restrictive lung disease/thoracic deformity
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4. Reactive airway disease
GI/nutritional:
1. Poor growth
2. Gastroesophageal reflux
3.
Constipation
4. Oral motor dysfunction/dysphagia
Urinary: Neurogenic bladder
Skin: Decubitus ulcers
Dental:
1. Malocclusions
2. Caries
3. Gingival hyperplasia
4.
Abnormalities of enamel (congenital)Diagnosis
SIGNS AND SYMPTOMS
History
Prenatal:
1. Exposure to toxins/drugs
2. Infections or fever
3. HIV/STD risk
4.
Vaginal bleeding5. Abnormal fetal movement
6. Pre-eclampsia (especially proteinuria)
7. Breech position
8. Poor maternal weight gain
9. Premature labor
10. Fetal distress
11. IUGR
12. Prenatal testing
13. Placental disorders
Perinatal:
1. Premature delivery
2. Neonatal resuscitation
3. Low Apgar scores (<5 at 5 minutes)
4. Birth trauma
5. Evidence of neonatal encephalopathy (seizures, severe hypotonia)
6. Complicated neonatal course (intraventricular hemorrhage, prolonged
respiratory support, meningitis, sepsis, hyperbilirubinemia)
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Postnatal:
1. Hospitalization for severe infection or trauma
2. Periodic or persistent deterioration in function (suggests
neurodegenerative/metabolic disease)
Development:
1. Severe delay in motor milestones (e.g., not rolling at 7 months, not
sitting at 8 months, not walking at 15 months) associated with
persistent primitive reflexes (e.g., prominent tonic neck and
labyrinthine responses at 1 year of age) and delayed or absent
development of protective reactions (e.g., lateral prop at 7 months,
parachute at 13 months)
2. Associated delays in language, play, social, and adaptive behavior
Physical Exam
Respiratory pattern: Obstruction, aspiration risk, evidence of
dysmorphism/pigmentary skin changes, and growth abnormalities contribute
to assessment of etiology.
Head circumference: To evaluate for
microcephaly/macrocephaly/hydrocephaly; growth velocity is important.
Strabismus/cataracts/iris or retinal abnormalities: Eye exam: Cranial nerve
damage, muscle imbalance, metabolic disease, or congenital infection
Musculoskeletal:1. Decreased range with contractures
2. Leg-length discrepancy: Hip dislocation
3. Spinal curvature/scoliosis
Neurologic:
1. Documentation of best level of visual motor/manipulative skills (e.g.,
able to run, transfer, hold a cup): To follow course of motor
impairment
2. Cranial nerves: Strabismus, speech and swallowing, vision and hearing
3. Tone: Spasticity versus rigidity versus hypotonia
4. Strength: Often decreased
5. Hyperactive deep tendon reflexes and clonus in spasticity; Babinski
reflex (extensor response to plantar stimulation)
6. Persistent primitive reflexes
7. Protective reactions: Head and trunk righting, prop reactions,
parachute; cerebellar signs
8. Balance, stability
CLINICAL:
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Pitfalls:
Overdiagnosis of CP in infants with spastic hypertonia; normalization of
tone/function may take up to 2 years.
False or premature assumption of cognitive deficit in children with severe
dysarthria. May take years of augmentative communication supports to
determine true potential.
Slowly progressive neurogenerative disease may masquerade as CP.
Cervical cord lesions may masquerade as quadriparetic spastic CP.
Determination of ideal body weight may be complex in CP; growth standards
according to CP type are under development.
TESTS
Hearing and vision: All in 1st year, with regular follow-up exams
Audiologic evaluation: Required for children with CP in accordance withregular health care and educational guidelines
Urodynamic studies: Spastic bladder in those with recurrent UTIs or voiding
dysfunction
Sleep study: May disclose treatable obstructive sleep apnea in those with
somnolence or abnormal sleep-wake cycles
Pulmonary function studies: Document progressive restrictive pulmonary
dysfunction (e.g., in severe scoliosis)
Brain wave (EEG): If seizure suspectedLABORATORY
Genetic and metabolic studies: If history or physical suggests a progressive or
hereditary disorder
Blood chemistries, liver function studies, cell counts: Evaluate
nutritional/metabolic status, anticonvulsant levels
IMAGING
Brain imaging: Perform when hydrocephalus is suspected; can help determine
etiology
Radiography: Should be done routinely in spastic diparesis for hip dislocation;
consider scoliosis films
Radionuclide studies to evaluate gastroesophageal reflux, gastric emptying,
aspiration
DIFFERENTIAL DIAGNOSIS
Motor syndromes related to spinal cord, lower motor neuron, peripheral
nerve, primary muscular disease, or progressive disorders of the basal ganglia
(dopa-responsive dystonia)
Connective tissue disorders (primary and secondary) resulting in
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musculoskeletal abnormalities (e.g., arthrogryposis multiplex, skeletal
dysplasias)
Inborn errors of metabolism and CP: Protean manifestations, dyskinesia,
ataxia, postnatal growth failure, neurologic deterioration, recurrent vomiting
Treatment
GENERAL MEASURES
Family-centered care is directed toward optimizing function and minimizing
handicap.
Interdisciplinary clinics: Services (medical, surgical, therapy) coordinated with
primary physician
Therapy with IM injections of botulinum toxin used increasingly, although the
therapy may need to be repeated every 3 –
5 months. Establish specific goalsfor therapy.
Education services: Recent emphasis on inclusion/mainstreaming; for many,
special education services are still required.
Augmentative communication supports especially for nonverbal/dysarthric
children
Physical, occupational, speech/language therapy, other allied health
professionals: Therapy provided in home, school, and hospital settings;
directed primarily at improved mobility, self-care, and communication;orthodontists for braces
Counseling support for children coping with chronic disability
Social services: Provided in a variety of contexts to aid in the coordination of
care
Vocational counseling and employment options, assistance with transition to
adulthood, self advocacy, self determination
Transition to adult health care system
DIET
Nutritional assessment and support for those with dysphagia or poor growth
Follow-up Recommendations
Requirements for follow-up vary greatly with the degree of disability and
impairment. An interdisciplinary clinic setting may be more appropriate for a
child with severe CP.
Early referral to a pediatric orthopedist is indicated, especially for monitoring
of early hip subluxation, which is best managed before progression to
dislocation.
Early referral for developmental assessment: Need for early intervention, to
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optimize development and promote family coping.
Patient Teaching Medication
Online information for parents: United Cerebral Palsy Association, www.ucp.org.
Frequently Asked Questions
Q: Is severe clumsiness a form of CP?
A: Mild spastic diplegia or hemiplegia may present this way, but tone
abnormalities distinguish these from developmental coordination disorders.
Q: Do children with CP also have MR?