[Frontotemporal lobar degeneration].
نویسندگان
چکیده
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 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.
منابع مشابه
Pharmacological treatment of frontotemporal lobar degeneration: systematic review.
OBJECTIVE To identify the therapeutic options available for treatment of cognitive and behavioral symptoms in frontotemporal lobar degeneration. METHOD Systematic review using the descriptors "frontotemporal lobar degeneration" OR "frontotemporal dementia" OR "fronto-temporal dementia" OR "fronto-temporal degeneration" OR "Pick's disease" OR "Pick's atrophy" OR "semantic dementia" OR "progres...
متن کاملElevated TMEM106B levels exaggerate lipofuscin accumulation and lysosomal dysfunction in aged mice with progranulin deficiency
Mutations resulting in haploinsufficiency of progranulin (PGRN) cause frontotemporal lobar degeneration with TDP-43-positive inclusions (FTLD-TDP), a devastating neurodegenerative disease. Accumulating evidence suggest a crucial role of progranulin in maintaining proper lysosomal function during aging. TMEM106B has been identified as a risk factor for frontotemporal lobar degeneration with pr...
متن کاملAnterior opercular syndrome in frontotemporal lobar degeneration with ubiquitin-only immunoreactive neuronal changes.
This paper presents for the first time an anterior opercular syndrome in association with a gait disorder, dropped head syndrome, dysphagia, and sialorrhea in a neuropathologically proven case of frontotemporal lobar degeneration with ubiquitin inclusions.
متن کاملDelusion of Pregnancy in Frontotemporal Lobar Degeneration with Motor Neurone Disease (FTLD/MND)
Psychotic phenomena such as delusions and hallucinations are rare in frontotemporal dementia syndromes but have recently been recognised as an early feature in some cases of frontotemporal lobar degeneration with motor neurone disease (FTLD/MND). A patient with delusion of pregnancy as an early feature of FTLD/MND is presented to illustrate the need to consider neurodegenerative disease as well...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Fortschritte der Neurologie-Psychiatrie
دوره 77 5 شماره
صفحات -
تاریخ انتشار 2009