Brief Summary of Cerebral Palsy

The most common cause of crippling in children, cerebral palsy is an umbrella term for a group of neuromuscular disorders resulting from prenatal, perinatal, or postnatal central nervous system (CNS) damage. Although nonprogressive, these disorders may become more obvious as an affected infant ages.

Types:
Three major types of cerebral palsy occur—

1. spastic, 
2. athetoid, and 
3. ataxic

—sometimes in mixed forms. Motor impairment may be minimal (sometimes apparent only during physical activities such as running) or severely disabling. Associated defects—such as seizures, speech disorders, and mental retardation—are common.
The prognosis varies. With mild impairment, proper treatment may make a near-normal life possible.

Cerebral palsy is most common in premature infants (anoxia plays the greatest role in contributing to cerebral palsy) and in those who are small for their gestational age. Cerebral palsy is common in whites and is slightly more common in males than in females.

Causes
Conditions that result in cerebral anoxia, hemorrhage, or other CNS damage are probably responsible for cerebral palsy.

Prenatal causes
Prenatal causes include maternal infection (especially rubella), radiation, anoxia, toxemia, maternal diabetes, abnormal placental attachment, vaginal bleeding, malnutrition, and isoimmunization.

Perinatal and birth difficulties
Examples of perinatal and birth difficulties include inadequate oxygenation of the brain, forceps delivery, breech presentation, placenta previa, abruptio placentae, depressed maternal vital signs from general or spinal anesthetic, and prolapsed cord with delay in the delivery of the head. Premature birth, prolonged or unusually rapid labor, and multiple birth (especially infants born last in a multiple birth) may also cause cerebral palsy as well as bleeding into the brain.

Infection or trauma during infancy
Cerebral palsy may follow kernicterus resulting from erythroblastosis fetalis, brain infection, head trauma, prolonged anoxia, brain tumor, cerebral circulatory anomalies causing blood vessel rupture, and systemic disease resulting in cerebral thrombosis or embolus.

Signs and symptoms
Each type of cerebral palsy typically produces a distinctive set of symptoms, although some children display a mixed form of the disease.

Spastic cerebral palsy
Spastic cerebral palsy is the predominant form, affecting about 70% of patients. This form of the disease is characterized by hyperactive deep tendon reflexes, increased stretch reflexes, rapid alternating muscle contraction and relaxation, muscle weakness, underdevelopment of affected limbs, muscle contraction in response to manipulation, and a tendency toward contractures. A child with spastic cerebral palsy typically walks on his toes with a scissors gait, crossing one foot in front of the other.

Athetoid cerebral palsy
Affecting about 20% of patients, athetoid cerebral palsy causes involuntary movements—grimacing, wormlike writhing, dystonia, and sharp jerks—that impair voluntary movement. Usually, these involuntary movements affect the arms more severely than the legs; involuntary facial movements may make speech difficult. These athetoid movements become more severe during stress, decrease with relaxation, and disappear entirely during sleep.

Ataxic cerebral palsy
Roughly 10% of patients have ataxic cerebral palsy. It’s characterized by disturbed balance, incoordination (especially of the arms), hypoactive reflexes, nystagmus, muscle weakness, tremor (also intention tremor), lack of leg movement during infancy, and a wide gait as the child begins to walk. Ataxia makes sudden or fine movements almost impossible.

Mixed form
Some children with cerebral palsy display a combination of signs and symptoms. In most, impaired motor function makes eating, especially swallowing, difficult and retards growth and development. Up to 40% of these children are mentally retarded, about 25% have seizure disorders, and about 80% have impaired speech. Many also have dental abnormalities, vision and hearing defects, and reading disabilities.

Diagnosis
An early diagnosis is essential for effective treatment and requires careful clinical observation during infancy and precise neurologic assessment. Suspect cerebral palsy whenever an infant:

• has difficulty sucking or keeping the nipple or food in his mouth 
• seldom moves voluntarily or has arm or leg tremors with voluntary movement
• crosses his legs when lifted from behind rather than pulling them up or “bicycling” like a normal infant
• has legs that are hard to separate, making diaper changing difficult
• persistently uses only one hand or, as he gets older, uses his hands well but not his legs.

Infants at particular risk include those with low birth weight, breech presentation, low Apgar scores at 5 minutes, seizures, and metabolic disturbances (such as maternal infection). However, all infants should be screened for cerebral palsy as a regular part of their 6-month checkup.
A computed tomography scan and magnetic resonance imaging may help rule out other problems.

Management

Cerebral palsy can’t be cured, but proper treatment can help affected children reach their full potential within the limits set by this disorder. Such treatment requires a comprehensive and cooperative effort involving physicians, nurses, teachers, psychologists, the child’s family, and occupational, physical, and speech therapists. Home care is usually possible. Treatment usually includes:

• braces or splints and special appliances, such as adapted eating utensils and a low toilet seat with arms, to help these children perform activities independently
• an artificial urinary sphincter for the incontinent child who can use hand controls
• range-of-motion exercises to minimize contractures
• orthopedic surgery to correct contractures
• phenytoin, phenobarbital, or another anticonvulsant to control seizures
• a muscle relaxant or neurosurgery to decrease spasticity.

Children with milder forms of cerebral palsy should attend regular school; severely afflicted children need special education classes.