Frontotemporal lobar degeneration - A consensus on clinical diagnostic criteria

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 syndrome s-frontotemporal dementia, progressive nonfluent aphasia, and semantic deme ntia-were developed by members of an international workshop on frontotempor al lobar degeneration. These criteria build on earlier published clinical d iagnostic guidelines for frontotemporal dementia produced by some of the wo rkshop members. Results: The consensus criteria specify core and supportive features for each of the three prototypic clinical syndromes and provide b road inclusion and exclusion criteria for the generic entity of frontotempo ral lobar degeneration. The criteria are presented in lists, and operationa l definitions for features are provided in the text. Conclusions: The crite ria ought to provide the foundation for research work into the neuropsychol ogy, 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 .