Cerebral Palsy

– A broad classification encompassing nonprogressive motor disorders caused by prenatal, perinatal, or postnatal central nervous system damage and characterized by impaired voluntary movement and possibly perceptual, intellectual, and language deficits and convulsive seizures. The four major categories of cerebral palsy (CP) are spastic, dyskinetic, ataxic, and mixed CP.

Causes and Incidence

A variety of prenatal, perinatal, and postnatal factors, alone or together, contribute to the development of CP. Prenatal causes, which are implicated in 44% of cases, include genetic factors, chromosomal abnormalities, teratogens, malformations in the brain, intrauterine infection, and fetal or placental transport malfunction. Perinatal causes (27% of cases) include prematurity, preeclampsia, sepsis, birth trauma, and asphyxia. Postnatal causes (5% of cases) include meningitis, head injury, and toxins. The cause is unknown or not readily apparent in 24% of diagnosed cases.
Among infants and children in the United States, the incidence of CP is about 2 per 1,000. CP is the most common cause of permanent disability in children, and the incidence is rising.

Disease Process

CP has no characteristic pathologic picture. Neurologic damage varies widely and is manifested in a number of ways. Anoxia seems to play a major role, but that role is ill defined. Spastic syndromes (70% of cases) involve the upper motor neurons. Dyskinetic syndromes (20% of cases) involve the basal ganglia. Ataxic syndromes (10% of cases) involve the cerebellum or cerebellar pathways, or both.

Symptoms

Clinical signs and symptoms evolve as the damaged nervous system matures; the signs and symptoms vary according to the location and severity of the neurologic damage.

General
Gross motor delays, abnormal motor performance, alteration of muscle tone, poor head and trunk control, poor balance, excessive docility or irritability, persistence of primitive reflexes.

Spastic type
Weakness, paralysis, muscle wasting, and spasticity involving one to four limbs; scissors gait; toe walking; weakness of oral and lingual muscles with dysarthria, tongue thrusting, difficulty swallowing; abnormal posture; sudden rigid extension of body while sitting; opisthotonic posturing.

Dyskinetic type
Slow, writhing, involuntary movements of trunk, mouth, and limbs that increase with stress and cease during sleep; abrupt, jerky movements of distal extremities; severe dysarthria; drooling; choking and coughing while feeding.

Ataxic type
Weakness; incoordination; intention tremor; widebased, ataxic gait; rapid, repetitive movements; disintegration of movement in upper extremities when reaching.

Associated problems
Intellectual impairment, hearing and visual impairments, language deficits, seizures, hyperactivity, short attention span.

Potential Complications

Aspiration and aspiration pneumonia may occur because of swallowing and choking with feeding. Hip dislocations, scoliosis, and joint contractures may occur because of hypertonicity or tone imbalance. Dental problems (e.g., malocclusion, gingivitis, caries, enamel defects) occur secondary to drooling, spasticity, and inadequate oral hygiene. Constipation is common. Skin breakdown and pathologic fractures can result from disuse syndrome.

Diagnostic Tests

Because the clinical signs of CP emerge as the neurologic system matures, it is rarely possible to detect CP in early infancy. Specific categorization of the syndrome may be impossible before 2 years of age, and other progressive neurologic disorders must be ruled out. A thorough neurologic examination and history are the primary diagnostic tools in CP. Gross motor delays; motor abnormalities; and altered muscle size, function, and tone are crucial diagnostic indicators. Moro reflex persisting after 4 months of age and tonic neck reflex persisting after 6 months of age are also useful indicators. High-risk children should be monitored closely.

Treatments

Surgery
Correction of hip displacement; release of contractures; lengthening of heel cord to prevent tiptoe stance; spinal instrumentation for scoliosis; dorsal rhizotomy to control spasticity.

Drugs
Muscle relaxants and antianxiety agents to reduce spasticity and facilitate movement; anticonvulsants for seizure activity; stool softeners, suppositories for constipation.

General
Long-term medical monitoring by neurologists, orthopedists, and pediatricians; Also, Physical therapy to reduce spasticity, maintain range of motion, prevent contractures, promote head and trunk control, and improve balance, sitting, standing, and ambulation Occupational therapy to improve oral motor skills (sucking, chewing, swallowing) and to facilitate activities of daily living (ADLs) Speech therapy to improve language and communication skills Adaptive equipment to assist mobility (wheelchairs, braces, scooter boards, standers), communication (communication boards, computers, switch controllers, power pads), recreation (adapted toys), ADLs (bath aids, environmental control units, feeding aids) Counseling for child and family to adapt to disability Education under disabilities act provisions Case management to provide support and to coordinate efforts by health care team Respite care to aid primary caregiver.

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