cerebral palsy

7/21/2019 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



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



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)



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:



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



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



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?

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cerebral palsy

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