Huntington's Disease: Current Concepts of Therapy*

Abstract

Huntington's disease (Huntington's chorea), a degenerative disorder of the central nervous system, is inherited in an autosomal dominant pattern. Since there is no cure for this genetic disorder, therapy has been focused on pharmacologic manipulation of the involved neurotransmitter systems. In Huntington's disease, there is a functional predominance of striatal dopaminergic activity over antagonist cholinergic and possibly GABA-minergic systems. Many dopaminergic antagonists and cholinergic and GABA-minergic agonists are currently used in treatment. Supportive psychiatric care for affected persons and their families is an important therapeutic adjunct in the management of Huntington's disease. The eventual therapy will depend upon accurate identification of the primary genetic defect.

Deregulated Transcriptome as a Platform for Adrenal Huntington’s Disease-Related Pathology

“…Dopa decarboxylase activity was elevated in the erythrocytes of HD patients compared to the control group [31]. However, Butterfield et al suggested that changes in dopa decarboxylase activity in HD patients could be connected to phenothiazines used often in therapy [31,32].…”