Juvenile HD
Part 9

The symptoms and characteristics of juvenile Huntington’s disease.

What treatments are recommended specifically for juvenile HD?

Anticonvulsant drugs are usually prescribed to help prevent and control the seizures that occur in children with juvenile HD. Finding the right combination and amount of drugs is not an easy process, and often the optimal treatment varies over time and between individuals. In many cases, caregivers know the most about the child’s reactions to specific drugs, making it very important for the doctor and caregivers to communicate frequently about which drugs and doses are working and which are not.

Usually, physicians attempt to minimize the number of generalized tonic-clonic seizures. Carbamazepine (Tegretol), phenobarbital and phenytoin (Dilantin), and other medications commonly prescribed to control non-HD seizures are not effective in many individuals with HD. Many individuals with juvenile HD have responded more favorably to other seizure drugs, such as valproic acid and benzodiazepines like clonazepam. Myoclonus and jerking motions are usually not treated unless they are very severe (for example, if they cause the child to fall frequently or reduce the child's ability to take in food). Antimyoclonic drugs such as valproate are sometimes prescribed to treat myoclonic jerks.

Side effects of seizure drugs can include drooling, sleepiness, and a general sense of confusion. However, the most significant concern of seizure medications is their potential to aggravate other juvenile HD symptoms. Some drugs may cause increased swallowing problems, drowsiness, and coordination difficulties. Many children with HD also have a poor tolerance of anticonvulsant drugs. Generally, physicians attempt to minimize the seizures as much as they can without lowering the quality of life in other areas. Achieving this ideal balance often requires trying many different drugs and prescribing less than the maximum dosage of each particular drug. Although the children may still have occasional seizures, many parents consider this treatment more acceptable than the alternative: prescribing a higher dosage to eliminate seizures but worsening the child’s other symptoms.

Physical therapy is recommended to ease rigidity and to prevent degeneration (atrophy) of unused muscle. For some individuals, pool therapy especially helps to loosen tight muscles. Pool therapy involves exercises that are done while the individual is submerged in warm water. The warm temperature is soothing for muscles, and the buoyancy of the water makes motion require less effort, enabling patients to strengthen muscles gradually.

Drugs are sometimes prescribed to control other symptoms, such as rigidity and difficulty sleeping. Counseling and medication sometimes help with behavioral and psychological symptoms. Many times individuals with juvenile HD respond poorly to drugs that are commonly prescribed for adult-onset HD. Hence, with each new drug or dosage, the child should be monitored carefully for side effects, such as increased drowsiness or poorer performance in school. The most effective combination of treatments is different for every individual with juvenile HD, and this optimal care can be achieved only when the doctor and caregivers work together to discover what is best for the child.

Table E-1 gives an abbreviated summary of juvenile HD.

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A. Hsu, 2-25-02

For further reading:

1. “Juvenile Huntington Disease,” Huntington Society of Canada. Online.
   An excellent resource for juvenile HD; one of the few souces that focuses only on juvenile HD.
2. Byers, R. K., and J. A. Dodge (1967) “Huntington's chorea in children. Report of four cases.” Neurology 17: 587-96.
   A paper describing the symptoms, treatment, and diagnoses of four children with juvenile HD.
3. Byers, R. K., F. H. Gilles, and C. Fung (1973) “Huntington's disease in children. Neuropathologic study of four cases.” Neurology 23: 561-9.
   A very detailed paper that analyzes the brains of four individuals who died from juvenile HD and compares them to similar neuropathologic studies.
4. “Case 117 -- Progressive Movement Disorder,” University of Pittsburgh School of Medicine, Department of Pathology. Online.
   A case study of the diagnosis of a girl with juvenile HD.

Last Modified: 9-16-02

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