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August 1991

Factors Associated With Slow Progression in Huntington's Disease

Author Affiliations

From the Departments of Neurology, Boston (Mass) University School of Medicine (Drs Myers and Sax, Mr Kiely, and Ms Pettengill), and Massachusetts General Hospital, Harvard Medical School, Boston (Drs Myers, Koroshetz, and Bird and Ms Mastromauro); the School of Public Health, Boston University, (Dr Cupples); and the Mailman Research Center, McLean Hospital, Belmont, Mass (Dr Bird).

Arch Neurol. 1991;48(8):800-804. doi:10.1001/archneur.1991.00530200036015

• The rate of disease progression was assessed for 42 persons affected by Huntington's disease who had been neurologically examined at least six times and followed up for at least 3 years. Disease progression was assessed by a disability rating scale administered at each examination. Slow progression was associated with older age at onset of disease and with heavier weight (body mass index) at the first examination. Men tended to have a slower disease progression than did women, and this was particularly evident among men inheriting Huntington's disease from affected mothers. Neither the butyrophenone haloperidol nor the tricyclic antidepressant imipramine were related to rate of progression. Assessments of depression, hostility, and tobacco use were also unrelated to rate of progression. Clinical trials in Huntington's disease should consider these factors when designing therapeutic studies.

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