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Across dementia subtypes, definitive diagnosis has historically been reserved for cases with neuropathological confirmation or positive genetic testing. These authors examined the accuracy of prospectively obtained clinical diagnoses compared with autopsy-based diagnoses for 228 patients evaluated at an early-onset dementia clinic (mean age at onset, 57 ±8 years).
Diagnoses were rendered using a standardized approach that used the following information: illness course; the relative prominence of cognitive, behavioral, and motor signs and symptoms; the specific pattern of cognitive deficits elicited by clinical history-taking and neuropsychological testing; and the degree of selectivity of the cognitive deficits. During the period of clinical…