Late onset of Huntington's disease.
Twenty-five patients with late-onset Huntington's disease were studied; motor impairment appeared at age 50 years or later. The average age at onset of chorea was 57.5 years, with an average age at diagnosis of 63.1 years. Approximately 25% of persons affected by Huntington's disease exhibit late onset. A preponderance of maternal transmission was noted in late-onset Huntington's disease. The clinical features resembled those of mid-life onset Huntington's disease but progressed more slowly. Neuropathological evaluation of two cases reveal less severe neuronal atrophy than for mid-life onset disease.
Myers
R H
RH
Sax
D S
DS
Schoenfeld
M
M
Bird
E D
ED
Wolf
P A
PA
Vonsattel
J P
JP
White
R F
RF
Martin
J B
JB
D000367:Age Factors; D000368:Aged; D001284:Atrophy; D001921:Brain / Q000473:pathology; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology; D008297:Male; D008875:Middle Aged