Huntingdon’s chorea

The spiralling cost of drag development also created problems with drugs for rare diseases. About 4000 rare diseases are described in the literature. Many are known by the names of those who first described them—Huntingdon s chorea, Paget s disease, Tourette s syndrome and so on. Many might or do respond to chemotherapy. It is in the public interest that such diseases should be treated, but the problem is who should pay. 

Huntingdon s chorea in the Community, which means 3250 per person. Add in the other costs of drug production and the patient is being asked to pay at least 10 000 annually. 

Tetrabenazine, a benzoquinolizine derivative, inhibits vesicular monoamine transporter 2, leading to depletion of dopamine and other monoamines in the central nervous system. It was licensed in 2008 by the US Food and Drug Administration for use in the treatment of chorea associated with Huntingdon s disease. It is also used in the treatment of hemibaUismus, tardive dyskinesia, and Tourette syndrome. 

Consider rarer dementias if unusual (particularly neurological) symptoms frontotemporal, dementia in Parkinson s disease, Huntingdon s disease (family histoiy, chorea), Creutzfeldt Jakob Disease, AIDs-related encephalopathy, neurosyphilis, progressive supranuclear palsy. 

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