What is semantic dementia?: a cohort study of diagnostic features and clinical boundaries.

OBJECTIVES To describe a large, clinically defined cohort of patients with semantic dementia (SD) that highlights important, sometimes overlooked features and to compare it with similar entities. DESIGN Cohort study. SETTING A cognitive neurology clinic. PATIENTS A population of 48 patients clinically diagnosed with SD was contrasted with 52 patients with progressive nonfluent aphasia, 42 patients with a behavioral variety of frontotemporal dementia, and 105 patients with Alzheimer disease on speech output characteristics, comprehension, naming, and repetition subtests of the Western Aphasia Battery, the Frontal Behavioral Inventory, and other cognitive tests. Neuroimaging was visually analyzed, and 6 patients with SD had autopsy. RESULTS Of 37 patients with probable SD, 48.6% had semantic jargon; 21.6%, excessive garrulous output; and 75.7%, some pragmatic disturbance. Semantic substitutions were frequent in SD (54.1%) but phonological errors were absent, in contrast to progressive nonfluent aphasia with the opposite pattern. All but 3 patients with probable SD questioned the meaning of words. Patients with SD had significantly lower naming and comprehension scores, and their fluency was between progressive nonfluent aphasia and Alzheimer disease or behavioral frontotemporal dementia. Behavior was abnormal in 94.6% of patients with probable SD. CONCLUSIONS Semantic dementia is distinguishable from other presentations of frontotemporal dementia and Alzheimer disease, not only by fluent speech and impaired comprehension without loss of episodic memory, syntax, and phonology but also by empty, garrulous speech with thematic perseverations, semantic paraphasias, and poor category fluency. Questioning the meaning of words (eg, "What is steak?") is an important diagnostic clue not seen in other groups, and behavior change is prevalent.