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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(More)
Frontotemporal dementia is a dementia syndrome with diverse clinical characteristics. Based upon clinical parameters and single photon emission computed tomography, we identified 47 frontotemporal dementia subjects. In 10 of these 47 the primary site of brain dysfunction was anterior temporal and orbital-frontal with other frontal regions relatively spared.(More)
Recognition of non-verbal environmental sounds was investigated in 52 subjects with unilateral cerebro-vascular accidents and 18 age-matched normal controls. Impaired performance was most consistently found following cortical damage of homologous areas in either the left or the right hemisphere. Lesions involved the superior temporal gyrus (including the(More)
Three cases of slowly progressive speech and language disturbance were studied at various points post onset (three, five and 15 years respectively). Language, neuropsychological and brain imaging (computer tomography and positron emission tomography) evaluations were completed on all three patients. The data suggest that the syndrome of "progressive(More)
Sixteen patients manifested the syndrome of loss of environmental familiarity. The syndrome is characterized by an inability to recognize familiar surroundings in spite of relatively intact verbal memory, cognition, and perception. In addition to the loss of environmental familiarity, other clinical disturbances, including central achromatopsia,(More)
Confabulation consequent to organic amnesia is a well-described clinical finding. A number of plausible theories of confabulation have been proposed, but the various claims and counterclaims have not been systematically tested. A standardized test battery that included four different kinds of questions plus measures of suggestibility and the tendency to(More)
Subcortical dementia is a clinical syndrome characterized by slowness of mental processing, forgetfulness, impaired cognition, apathy, and depression. First recognized in progressive supranuclear palsy and Huntington's disease, the concept has been extended to account for the intellectual impairment of Parkinson's disease, Wilson's disease, spinocerebellar(More)