CiteWeb id: 19980000061

CiteWeb score: 6875

DOI: 10.1212/WNL.51.6.1546

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. NEUROLOGY 1998;5 1: 1546-1554 Frontotemporal lobar degeneration (FTLD) is the third most common cause of cortical dementia, following AD and Lewy body disease. In the past few years FTLD has been studied extensively, and substantial progress has been made in understanding its associated clinical syndromes and underlying pathologic changes. This report provides a consensus statement based on a conference of international investigators familiar with the disorder, and provides an update and extension of previously proposed clinical and pathologic diagnostic criteria for frontotemporal dementia (FTD).l FTLD encompasses two major pathologic substrates which affect primarily the frontal or temporal cortex, in some patients asymmetrically. Three prototypic neurobehavioral syndromes can be produced by FTLD. Results of the consensus conference presented here describe these three behavioral conditions. The most common clinical manifestation of FTLD is a profound alteration in personality and social conduct, characterized by inertia and loss of volition or social disinhibition and distractibility, with relative preservation of memory function (FTD).”5 There is emotional blunting and loss of insight. Behavior may be stereotyped and perseverative. Speech output is typically economical, leading ultimately to mutism, commensurate with the patient’s amotivational state, although a press of speech may be present in some overactive, disinhibited

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