Cerebral Palsy

Description of Medical Condition

A term used to describe a group of patients with a non-progressive but not unchanging disorder of movement or posture that is a result of a central nervous system insult that occurred prenatally; perinatally, or during the first three years of life

System(s) affected: Nervous, Musculoskeletal

Genetics: Rarely inherited. Small percentage of cases with symmetric signs are associated with autosomal recessive transmission. Symmetric and idiopathic spastic CP (4% of cases) has 1:8 recurrence risk.

Incidence/Prevalence in USA: 1 to 2 per 1,000 live births. Up to 10% of infants with birthweight less than 1500 grams.

Predominant age: Causative CNS insult during period of rapid brain growth but effects are life long and evolve with time.

Predominant sex: Male> Female (1.3:1)

Medical Symptoms and Signs of Disease

By classification

1. Spastic 40%

• Spasticity
• Hemiplegia, quadriplegia or diplegia
• Triplegia, monoplegia are rare
• Contra ctures
• Mental retardation with quadriplegia and mixed forms
• Normal intelligence with hemiplegia or paraplegia
• Scissors gate, toe walking
• Tremors with hemiplegia
• Aphonia with quadriplegia
• Seizures

2. Athetotic (dyskinetic) 30%

• Usually normal intelligence
• Choreoathetoid type is the most common with jerky motions of proximal muscle groups and slow writhing of extremities face neck and trunk
• Dystonic type due to simultaneous contraction of opposing muscle groups
• Speech difficulties
• Muscular hypertrophy
• Deafness common with athetosis caused by kernicterus

3. Ataxic10%

• Normal intelligence
• Clumsy disposition with wide based gate and difficulty with fine movements
• Best prognosis for functional improvement

3. Mixed 20%

• Spasticity and choreoathetosis most common
• Athetosis and ataxia can occur
• Mental retardation common.

What Causes Disease?

70% of the time, neither causes nor risk factors can be identified

In utero bacterial infections (chorioamnionitis), viral infections (e.g. rubella), CNS malformations, chromosomal abnormalities, coagulation disorders, kernicterus. CNS trauma and intraventricular hemorrhage

While most cases are due to prenatal events and prematurity, 10% or less of cases are due to intrapartum events. Such cases are almost always of spastic quadriplegic type or dyskinetic type and are associated with evidence of severe metabolic acidosis at birth (pH < 7.00) and early onset neonatal encephalopathy at birth. Criteria which individually are nonspecific but which together suggest intrapartum cause include a sentinel hypoxic event immediately before or during labor (e.g. cord prolapse, abruption or uterine rupture); sudden, rapid and sustained deterioration of the fetal heart rate pattern which was previously normal; Apgar scores of < 6 for greater than five minutes; early evidence of multisystem involvement and early imaging showing acute cerebral abnormality.

Risk Factors

• Prematurity
• Hypoxic ischemia
• Encephalopathy in the perinatal period
• Seizures in the perinatal period
• Germinal matrix and interventricular hemorrhage in the perinatal period in utero infections
• Meningitis/encephalitis postnatally
• Child abuse
• Intrauterine growth restriction
• Breech
• Multiple gestation

Diagnosis of Disease

Differential Diagnosis

Cerebral palsy is a descriptive term based on clinical observation. Chromosomal and metabolic abnormalities must be excluded. Early diagnosis made at 12 months of life may be wrong more than half the time. Evidence of disease progression excludes cerebral palsy. Early warning signs include delay in motor milestones, toe walking, persistent fisting, seizures, irritability, poor suck, established handedness before 2 years of age, and abnormal limb posture.

Laboratory

Laboratory data is not required to make the diagnosis.

Other tests may help exclude Tay-Sachs metachromatic leukodystrophy, mucopolysaccharidosis

Drugs that may alter lab results: None

Disorders that may alter lab results: None

Pathological Findings

Central ner vous system abnormalities: CT and MRI might show abnormalities of the brain including cysts, cerebral atrophy, calcification, tumors, malformation, strokes, etc.

Special Tests

• Urine amino acid screening
• High resolution karyotype

Diagnostic Procedures

History and careful physical exam EEG, MRLCT

Treatment (Medical Therapy)

Appropriate Health Care

The goal of therapy is to improve function

General Measures

• Early intervention services
• Physical therapy, occupational therapy, orthosis, adaptive equipment
• Oral medications should be carefully titrated upward to avoid side effects
• Alter muscle tone, assuming that the abnormal tone is adversely affecting function, by:

– Injection of botulinum toxin into the abnormal muscles

– Rhizotomy

– Continuous infusion of intrathecal baclofen

• Strabismus, refractive errors and visual field defects are common and should be addressed early to prevent deterioration
• Anti-convulsants for seizures
• Alternative therapies

– Hyperbaric oxygen therapy and therapeutic electrical stimulation were shown to have no benefit in randomized clinical trials

– Conductive Education (The Peto Method) was as good as, but not better than standard care in a randomized trial

– Constraint-induced movement or forced use therapy may be effective, but there are no large controlled studies

– Adeli Suit which is based on a design for Soviet cosmonauts is available only in Poland as part of intensive physio-therapy program. Little information is available.

Surgical Measures

• Tendon transfers and contracture release to prevent bone deformation
• Open reduction of hip subluxation and dislocation
• Dorsal rhizotomy to decrease spasticity
• Implantation of reservoir for intrathecal baclofen
• Spinal fusion and rods for scoliosis

Activity

Full activity depending upon the patient’s dysfunction

Diet

Normal diet, although constipation is frequent and stool softeners might be considered Poor suck, dysphagia and reflux often lead to nutritional difficulties. Nasogastrictube (short term) orgastros-tomy tube (long term) may be necessary.

Patient Education

It is very important to educate the patient and parents about the child’s disabilities as well as prognosis; mental retardation seen in 20-25% of patients with cerebral palsy.

United Cerebral Palsy, 1660 L Street, NW, Suite 700. Washington, DC 20036, (800)872-5827/(202)776-0406: TTY (202)973-7197; Fax (202)776-0414

Disabled children are at higher risk of physical abuse, neglect and sexual abuse. The usual adolescent issues such as sexuality must be addressed.

Patients and their families should be made aware of their rights under the Americans with Disabilities Act

Medications (Drugs, Medicines)

Drug(s) of Choice

1. Drugs to decrease spasticity:

• Baclofen is most commonly used
• Diazepam, clonazepam and clorazepate also are used and may also help with sleep disturbances and irritability
• Dantrolene sodium is sometimes used, but can be hepatoxic
• Tizanidine and clonidine, also available as transdermal patch, are alpha-2 adrenergic agents

2. Intrathecal baclofen via an implantable reservoir minimizes systemic side effects

3. Anti-seizure medications as needed

Contraindications: Refer to manufacturer’s literature

Precautions: Refer to manufacturer’s literature

Significant possible interactions: Refer to manufacturer’s literature

Alternative Drugs

– ther centrally acting muscle relaxants

Patient Monitoring

Followup visits are important to determine the development of contrac-tures and the presence of associated problems (e.g., epilepsy, learning disabilities, strabismus, hearing loss and mental retardation).

Prevention / Avoidance

In theory measures which decrease premature birth and fetal growth restriction would be of benefit. These include good prenatal care, smoking cessation, avoidance of substance abuse and care in infertility treatment to decrease the risk of multiple pregnancies. Antenatal corticosteroids (betamethasone or dexamethasone) should be given to the mother when premature birth is anticipated between 24 and 34 weeks gestation. While given mainly to decrease the risk of respiratory distress syndrome, their use has been associated with a decreased incidence of neonatal intraventricular hemorrhage.

Possible Complications

Chronicitywith permanent disability

Bladder dysfunction may lead to frequent urinary tract infections

Expected Course / Prognosis

The patient should improve in function with time

Muscle tone may change for the worse during adolescence (does not mean that the disease is progressive)

Miscellaneous

Associated Conditions

• Epilepsy
• Learning disabilities
• Mental retardation
• Behavioral problems
• Strabismus
• Hearing loss

Age-Related Factors

Pediatric:

Contractures will increase as a result of growth associated with asymmetrical muscle tone and strength. Scoliosis may develop as a result.

Geriatric:

N/A

Pregnancy

Reproductive function should not be affected. Contractures may make positioning for vaginal delivery difficult but cesarean is usually reserved for usual obstetric indications

For patients with seizures the risks of poor seizure control outweigh the teratogenic potential of almost all anticonvulsants. Periconceptional folate supplementation (1 to 4 mg per day) may decrease the risk of anticonvulsant teratogenesis.

There is little information on baclofen and dantrolene use in pregnancy although no adverse fetal or newborn effects have been reported

Synonyms

• Little disease

• Cerebral diplegia

• Infantile cerebral paralysis

International Classification of Diseases

343.9 Infantile cerebral palsy, unspecified