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Inheritance of frontotemporal dementia.
T W Chow1, B L Miller, V N Hayashi
1Department of Neurology, Reed Neurological Research Center, University of California, Los Angeles, School of Medicine, 90095-1769, USA.
Archives of Neurology
|July 15, 1999
Summary
Fronto-temporal dementia (FTD) is often inherited in an autosomal dominant pattern. Early symptoms include behavioral changes, frequently misdiagnosed, highlighting the need for awareness in presenile dementia cases.
Area of Science:
- Neurology
- Genetics
- Dementia Research
Background:
- Previous studies on fronto-temporal dementia (FTD) suggest autosomal dominant inheritance but often focus on families with multiple affected individuals.
- This study investigates familial presentation and inheritance in a larger, unselected group of FTD index cases.
Purpose of the Study:
- To determine the frequency of familial FTD and related disorders in unselected FTD cases.
- To identify the most likely mode of inheritance for familial FTD.
Main Methods:
- Interviews with family members of FTD index cases.
- Review of medical records and autopsy reports.
- Analysis of clinical findings, neuroimaging, and inheritance patterns.
Main Results:
- Fifty percent of the 42 FTD index cases had familial involvement, with 89% showing dominant inheritance.
- Initial presentations varied, commonly including personality/behavioral changes (43%) or psychiatric illness (33%).
- Average age of onset was 56.1 years, with few cases starting after 65.
Conclusions:
- Familial FTD typically follows an autosomal dominant inheritance pattern.
- Insidious onset with mood and behavioral changes in presenile years is common and often misattributed.
- FTD is a prevalent form of presenile dementia in North America.