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Familial Alzheimer Dementia: a prevalent disorder with specific clinical features

Published online by Cambridge University Press:  09 July 2009

John C. S. Breitner  and
Marshal F. Folstein
John C. S. Breitner*
Affiliation:
Department of Psychiatry and Behavioral Science, The Johns Hopkins University, Baltimore, Maryland, USA
Marshal F. Folstein
Affiliation:
Department of Psychiatry and Behavioral Science, The Johns Hopkins University, Baltimore, Maryland, USA
*
1Address for correspondence: Dr John C. S. Breitner Department of Psychiatry, Baltimore City Hospitals, Baltimore, MD 21224, USA.
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Synopsis

The early literature on Alzheimer Dementia (AD) describes the clinical features aphasia, apraxia and agraphia as characteristic. We investigated the hypothesis that these features would specifically identify the familial form of AD (FAD). Since pedigree studies had suggested that FAD is an autosomal dominant genetic disorder, we hypothesized that the first-degree relatives of language-disordered or apractic AD probands would show at least 50% lifetime risks of dementia. Using standardized methods, we screened 3500 nursing home beds for stringently defined AD cases and controls, tested for agraphia, and obtained probands' clinical and family histories from multiple informants. Language disorder and apraxia were found in 78% of AD cases. They strongly predicted familial aggregation of dementia, with a 90-year lifetime incidence among relatives exceeding 50%, or 7 times the control values. The results suggest that language disorder and apraxia specifically identify a distinct clinical entity, Familial Alzheimer Dementia, that is among the commonest forms of senile dementia.

Type
Research Article
Information
Psychological Medicine , Volume 14 , Issue 1 , February 1984 , pp. 63 - 80
Copyright
Copyright © Cambridge University Press 1984

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