Geriatrics Research Today is a free monthly online journal that collates and summarizes the latest research about Geriatrics, including details on old age, psychiatry, depression, medicine.

Geriatric neurogenetics: oxymoron or reality?

Bird TD, Lipe HP, Steinbart EJ

Geriatric Research Education Clinical Center, VA Puget Sound Health Care System, 1660 S Columbian Way, S-182-GRECC, Seattle, WA 98108. tomnroz@u.washington.edu.

BACKGROUND: Primary genetic diseases are generally associated with pediatric and young adult populations. Little information is available about the occurrence of single-gene mendelian diseases in elderly populations. OBJECTIVE: To describe the occurrence of single-gene neurogenetic disorders in a group of elderly patients. DESIGN: Retrospective review of neurogenetic cases in an academic medical center. SETTING: Academic university and Veterans Affairs medical centers. Patients Eight elderly patients with single-gene neurogenetic diseases were studied. These patients included an 87-year-old man and an 85-year-old man with Huntington disease, an 84-year-old woman with limb-girdle muscular dystrophy type 2A, a 78-year-old man with spinocerebellar ataxia type 14, an 86-year-old man with spinocerebellar ataxia type 5, an 85-year-old man with a presenilin 1 familial Alzheimer disease mutation, an 87-year-old man with autosomal dominant hereditary neuropathy, and a 78-year-old man with spinocerebellar ataxia type 6. Three patients had no family history of neurologic disease. MAIN OUTCOME MEASURES: Medical histories, physical examination results, and genetic testing results. CONCLUSIONS: Single-gene mendelian neurogenetic diseases can be found in the oldest old population (> 85 years). Such cases are currently underrecognized and will become more commonly observed in the future. This phenomenon is a result of (1) the aging of the general population, (2) better recognition of the highly variable ages at onset of genetic diseases, and (3) the availability of specific DNA-based genetic testing.

Published 16 April 2008 in Arch Neurol, 65(4): 537-9.
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