RT Journal Article
SR Electronic
T1 Late onset of Huntington's disease.
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP 530
OP 534
DO 10.1136/jnnp.48.6.530
VO 48
IS 6
A1 R H Myers
A1 D S Sax
A1 M Schoenfeld
A1 E D Bird
A1 P A Wolf
A1 J P Vonsattel
A1 R F White
A1 J B Martin
YR 1985
UL http://jnnp.bmj.com/content/48/6/530.abstract
AB 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.