Cerebral Palsy

Overview

• Definition

• Pathogenesis

• Clinical features

• Associated problems

• Differential diagnosis

• Management

• Prognosis

Cerebral palsy (CP)

Definition

• It is a heterogeneous group of disorder with persistent disorder of movement and posture caused by non progressive defects or lesions of immature brain.

• Non-specific term

• Non-progressive and may include perceptual problems, language deficits, and intellectual involvement.

CP history

• Dr William John Little detected this disorder first in 1860.

• Sometimes known as Little’s disease.

• Incidence has increased since the 1960 ’s, maybe due to improved survival of VLBW infants.

Incidence

• Most common physical disability of childhood.

• Incidence has increased since the 60’s, maybe due to improved survival of VLBW infants.

• Difficult to estimate accurate incidence.

• Approximately 1-2 /100 live births in India.

Etiology

• Variety of perinatal, prenatal, and postnatal factors contribute, either singly or multifactorily to CP.

• Commonly thought to be due to birth asphyxia; now known to be due to existing prenatal brain abnormalities.

• Premature delivery is the single most important determinant of CP.

• In 24% of cases, no cause is found.

TABLE 40-1 Causes of CP

• Time (% of cases)

• Prenatal (44%)

– First trimester

– Second trimester

• Causes

• Teratogens, chromosomal abnormalities, genetic syndromes, brain malformations

• Intrauterine infections, problems in fetal/placental functioning,coagulation & thrombosis

Causes of CP• Time (% of cases)

• Labor and delivery (19%)

• Perinatal (8%)

• Childhood (5%)

• Not obvious (24%)

• Causes

• Preeclampsia, complications of labor and delivery, multiple births.

• Sepsis/CNS infection, asphyxia, prematurity, acid base imbalance, indirect hyperbilirubinemia,

• Meningitis, traumatic brain injury, toxins

Pathogenesis

• Selective neuronal Necrosis

• Parasagittal brain injury

• Status marmatosus

• Cerebellar nuclei invovlvement

• Periventricular leucomalacia

Clinical Classification of CP

• Spastic-hypertonicity with poor posture control

• Hypotonic – despite pyramidal features infants are hypotonic.

• Dyskinetic/athetoid- abnormal involuntary movement/slow wormlike writhing

• Ataxic- wide-based gait

• Mixed-type- combination of spasticity and athetosis

Clinical manifestations

• Delayed gross motor development

– A universal manifestation of CP

– The discrepancy between motor ability and expected achievement tends to increase as growth advances.

– Delayed development of ability to balance slows milestones

– Delay in all motor accomplishments

Clinical Manifestations

• Abnormal motor performance– Preferential unilateral hand use may be apparent at 6

months.

– Hemiplegia, abnormal crawling or asymmetrical crawl; spasticity may cause child to walk and stand on toes

– dyskinetic CP or uncoordinated or involuntary movements (writhing tongue, fingers, and toes; facial grimacing), poor sucking and feeding, persistent tongue thrust; head staggering, tremor on reaching, truncal ataxia.

Alterations in muscle tone

• Increased or decreased resistance to passive movement (abnormal muscle tone).

• Opisthotonic postures or exaggerated back arching, feel stiff on dressing.

• Difficulty diapering due to spastic hip adductor muscles and lower extremities

• When pulled to a sitting position, child may extend the entire body and be rigid at hip and knee. This is an early sign of spasticity.

Abnormal postures

• Children with spastic CP have abnormal posture at rest or when position is changed

• Infantile lying prone may have hip higher than trunk with legs and arms drawn in.

• Persistent infantile resting and sleeping position is a sign of spasticity.

• Hemiparetic child may rest with affected arm adducted and held against torso, with the elbow pronated and slightly flexed and the hand closed.

Reflex Abnormalities

• Persistence of primitive infantile reflexes (one of the earliest signs of CP)

– Tonic neck reflex

– Hyperactivity or moro, plantar, palmar grasp

Hyperreflexia, ankle clonus, stretch reflexes can be elicited from any muscle group.

Associated disabilities and problems

• Intellectual impairment

– 70% w/in normal limits; wide range

– Tests should be carried out over a period of time.

– Children with athetosis and ataxia more intelligent.

Speech difficulties (not a sign or MR)- child has motor and sensory defects

ADHD- (may occur)-poor attention span, marked distractibility, hyperactive behavior

ASSOCIATED DISABILITIES

Seizures- generalized tonic-clonic;more in postnatally acquired hemiplegia

Drooling- may occur and lead to wet clothing/skin irritation

Feeding- alterations in muscle tone lead to difficulties chewing, swallowing, talking, etc.

Address nutritional concerns.

Coughing, choking may lead to aspiration.

Altered respiratory patterns may lead to inadequate gas exchange.

Motor Impairment

• Orthopedic complications– Unilateral or bilateral hip dislocations, scoliosis, joint contractures due

to unbalanced muscle tone.

Decreased Mobility

– difficulties with toileting may lead to constipation

– Difficult chewing bulky foods may lead to constipation

– May need stool softeners or laxatives

Associated Problems

• Dental carries– Improper dental hygiene

– congenital enamel defects (hyperplasia of primary teeth)

– high carbohydrate intake and retention

– Dietary balance with poor nutritional intake

– Inadequate fluoride

– Difficulty in mouth closure and drooling

– Spastic or clonic movements cause gagging or biting on toothbrush

Associated Problems

• Malocclusion in 90% of children

• Oral hypersensitivity causes resistance to good hygiene

• Gingivitis is secondary to poor hygiene

• Dental health further complicated by anti-seizure meds

Associated problems

• Nystagmus and amblyopia common

– May need surgery or corrective lenses

– May be due to sensoneural involvement

– Infants lying flat too long may have otitis media which may leads to conductive hearing loss