Huntingdon’s disease

Beal MF, Ferrante RJ, Swartz KJ, Kowall NW (1990) Chronic quinolinic acid lesions in rates closely resemble Huntingdon s disease. J Neurosci 11 1649-1659... 

Several neurodegenerative disorders, such as Alzheimer s and Huntingdon s diseases, are associated with increased production of F -isoprostanes... 

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. 

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. 

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