The Pharmaceutical Journal
Vol 277 No 7428 p641-642
25 November 2006

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What evidence there is for the drug treatment of Huntington's disease

Elizabeth Bevan and Carol Paton searched Medline, EMBASE and the Cochrane Library for evidence up to September 2006 and, in this article, they review the literature on the pharmacological management of chorea and the psychiatric co-morbidity in patients with Huntington's disease

SUMMARY

Huntington’s disease (HD) is a hereditary disease that involves slow progressive degeneration of the neurones in the basal ganglia and cerebral cortex. It is an autosomal dominant disease caused by a mutation of a gene on chromosome 4; there is an expansion of a trinucleotide repeat within the part of this gene that encodes the Huntington protein. Neurones are damaged when the mutated protein aggregates and interferes with normal metabolism and functioning but the mechanism is poorly understood, making it difficult to develop drugs that slow or stop progression. In western Europe the prevalence of HD is between three and seven per 100,000.

Symptom onset is usually between the ages of 35 and 50 years, but can occur in early childhood or old age. The greater the number of trinucleotide repeats, the earlier the age of onset. HD usually has a course of 15 to 20 years, although juvenile onset cases often progress more rapidly. The clinical presentation is characterised by increasingly severe involuntary movements (chorea) and cognitive decline. Choreiform movements and dementia are core symptoms of HD and both psychosis and depression are common. The cause of death is usually respiratory infection secondary to failure of the gag reflex and respiratory muscles.

Evidence supporting the pharmacological management of chorea and the psychiatric manifestations of HD is summarised below. Adjuvant psychotherapy, physiotherapy and speech therapy should be applied to provide optimal management. The use of these strategies is outside the scope of this review.

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