There is a widely held belief that most patients presenting with senil
e chorea have late-onset Huntington's disease (HD) with an unknown fam
ily history. We measured CAG trinucleotide repeat expansion in the HD
gene in four patients with a clinical presentation of senile chorea an
d found that CAG repetition lengths were normal. These findings suppor
t senile chorea as being a distinct clinical entity that is nosologica
lly separate from late-onset HD.