It is an autosomal dominant degeneration of the basal ganglia, cerebral cortex especially the frontal lobe. There is decreased levels of GABA, acetyl choline, angiotensin II in the striatum. It is an uncommon condition affecting patients 30-50 years old.
The presence of progressive chorea (basal ganglia) in the presence of behavioral changes and dementia (frontal lobe).
CT / MRI in fully developed cases shows atrophy especially of basal ganglia.
The treatment aims mainly at symptomatic relief of patients. Serpasil 1-2 mg tds and chlorpromazine 100 mg tds may offer some benefit.
Are you a Doctor, Pharmacist, PA or a Nurse?
Join the Doctors Lounge online medical community
Editorial activities: Publish, peer review, edit online articles.
Ask a Doctor Teams: Respond to patient questions and discuss challenging presentations with other members.