A 51 yo male under psychiatric treatment over several years. Relative complaining of his dementia. On neurological examination noticed Chorea. Family history was significant; father had a milder but similar illness.
Marked atrophy of caudate nuclei with ex vacuo dilatation of frontal horns.
An associated diffuse cerebral cortical Atrophy.
Imaging findings and history is consistent with Huntington's Disease.
Syn: Huntington Chorea.
An autosomal dominant neuro degenerative disorder with loss of GABAergic neurons of basal ganglia.
Clinically characterized by triad of Dementia, Choro athetosis and Psychosis.
Imaging wise diagnostic clue is caudate nuclei atrophy.
Age: Adult onset : 35 - 45years, Juvenile onset : < 20 years; M=F