Frontotemporal lobar degeneration through loss of progranulin function

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Frontotemporal lobar degeneration through loss of progranulin function.

The striking clinical and pathological features of frontotemporal lobar degeneration (FTLD) have attracted much attention ever since Arnold Pick reported the first disease cases and Alois Alzheimer described the characteristic nerve cell inclusions of Pick’s disease. We now know that FTLD is a common cause of dementia, especially in the population below the age of 65 (Neary et al., 2005). Clini...

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Genetic and clinical features of progranulin-associated frontotemporal lobar degeneration.

OBJECTIVE To assess the relative frequency of unique mutations and their associated characteristics in 97 individuals with mutations in progranulin (GRN), an important cause of frontotemporal lobar degeneration (FTLD). PARTICIPANTS AND DESIGN A 46-site International Frontotemporal Lobar Degeneration Collaboration was formed to collect cases of FTLD with TAR DNA-binding protein of 43-kDa (TDP-...

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[Frontotemporal lobar degeneration].

Article abstract-Objectiue: 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 lo...

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Unpicking frontotemporal lobar degeneration.

Chou TT, et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 2006; 314: 130–3. Piguet O, Hornberger M, Shelley BP, Kipps CM, Hodges JR. Sensitivity of current criteria for the diagnosis of behavioral variant frontotemporal dementia. Neurology 2009; 72: 732–7. Rascovsky K, Hodges JR, Knopman D, Mendez MF, Kramer JH, Neuhaus J, et al. Sensit...

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Frontotemporal Lobar Degeneration

Frontotemporal lobar degeneration (FTLD) comprises diseases with a very diverging spectrum in regards to clinical presentation, genetics, and neuropathology. In 1892 Arnold Pick published the case of 71-year old male with progressive symptoms of aphasia, apathy, and dementia (Pick 1892). The pathological examination revealed cortical atrophy with emphasis on the left temporal lobe (Pick 1892). ...

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ژورنال

عنوان ژورنال: Brain

سال: 2006

ISSN: 0006-8950,1460-2156

DOI: 10.1093/brain/awl291