2005NeurologyRequires access

Comparison of family histories in FTLD subtypes and related tauopathies

Jill Goldman, Jennifer Farmer, Elisabeth McCarty Wood, Julene K. Johnson, Adam L. Boxer, John Neuhaus, Catherine Lomen‐Hoerth, K. C. Wilhelmsen, V. M. -Y. Lee, Murray Grossman, Bruce L. Miller

Open publisher page 356 citations

Abstract

Pedigrees from 269 patients with frontotemporal lobar degeneration (FTLD), including frontotemporal dementia (FTD), FTD with ALS (FTD/ALS), progressive nonfluent aphasia, semantic dementia (SD), corticobasal degeneration, and progressive supranuclear palsy were analyzed to determine the degree of heritability of these disorders. FTD/ALS was the most and SD the least heritable subtype. FTLD syndromes appear to have different etiologies and recurrence risks.

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What this paper is about

Pedigrees from 269 patients with frontotemporal lobar degeneration (FTLD), including frontotemporal dementia (FTD), FTD with ALS (FTD/ALS), progressive nonfluent aphasia, semantic dementia (SD), corticobasal degeneration, and progressive supranuclear palsy were analyzed to determine the degree of heritability of these disorders. FTD/ALS was the most and SD the least heritable subtype. FTLD syndromes appear to have different etiologies and recurrence risks.

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Available abstract

Pedigrees from 269 patients with frontotemporal lobar degeneration (FTLD), including frontotemporal dementia (FTD), FTD with ALS (FTD/ALS), progressive nonfluent aphasia, semantic dementia (SD), corticobasal degeneration, and progressive supranuclear palsy were analyzed to determine the degree of heritability of these disorders. FTD/ALS was the most and SD the least heritable subtype. FTLD syndromes appear to have different etiologies and recurrence risks.

Key concepts: Frontotemporal dementia, Progressive supranuclear palsy, Corticobasal degeneration, Semantic dementia, Frontotemporal lobar degeneration, Primary progressive aphasia, Psychology, Dementia

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