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.