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Journal ArticleDOI

Frontotemporal lobar degeneration A consensus on clinical diagnostic criteria

TLDR
Consensus criteria for the three prototypic syndromes-frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia-were developed by members of an international workshop on frontotem temporal lobar degeneration and ought to provide the foundation for research work into the neuropsychology, neuropathology, genetics, molecular biology, and epidemiology of these important clinical disorders.
Abstract
Objective: To improve clinical recognition and provide research diagnostic criteria for three clinical syndromes associated with frontotemporal lobar degeneration. Methods: Consensus criteria for the three prototypic syndromes-frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia-were developed by members of an international workshop on frontotemporal lobar degeneration. These criteria build on earlier published clinical diagnostic guidelines for frontotemporal dementia produced by some of the workshop members. Results: The consensus criteria specify core and supportive features for each of the three prototypic clinical syndromes and provide broad inclusion and exclusion criteria for the generic entity of frontotemporal lobar degeneration. The criteria are presented in lists, and operational definitions for features are provided in the text. Conclusions: The criteria ought to provide the foundation for research work into the neuropsychology, neuropathology, genetics, molecular biology, and epidemiology of these important clinical disorders that account for a substantial proportion of cases of primary degenerative dementia occurring before the age of 65 years.

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Citations
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Journal ArticleDOI

Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia.

TL;DR: The revised criteria for behavioural variant frontotemporal dementia improve diagnostic accuracy compared with previously established criteria in a sample with known frontotmporal lobar degeneration and reflect the optimized diagnostic features, less restrictive exclusion features and a flexible structure that accommodates different initial clinical presentations.
References
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Journal ArticleDOI

Association of missense and 5′-splice-site mutations in tau with the inherited dementia FTDP-17

TL;DR: In this paper, the authors sequenced tau in FTDP-17 families and identified three missense mutations (G272V, P301L and R406W) and three mutations in the 5' splice site of exon in
Journal ArticleDOI

Tau is a candidate gene for chromosome 17 frontotemporal dementia.

TL;DR: Three lines of evidence indicate that the Val279Met change is an FTDP‐17 causative mutation, and normal valine is found at this position in all three tau interrepeat sequences and in other microtubule associated protein tau homologues.
Journal ArticleDOI

Slowly progressive aphasia without generalized dementia

TL;DR: Six right‐handed patients experienced a slowly progressing aphasic disorder without the additional intellectual and behavioral disturbances of dementia, and this condition may constitute a syndrome of relatively focal cerebral degeneration with a predilection for the left perisylvian region.
Journal ArticleDOI

Semantic dementia: A form of circumscribed cerebral atrophy

TL;DR: In this article, a 67-yr-old man and 2 women (aged 60 and 66 yrs) with primary cerebral atrophy in whom progressive breakdown in language and visual perception are attributed to loss of semantic information.
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