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Behavioral and cognitive distinction between frontotemporal and Alzheimer dementias

Objective: To identify clinical and psychometric indicators that distinguishes Alzheimer’ s dementia (AD) and frontotemporal lobar degeneration (FTLD), confirmed at autopsy, at initial presentation. Background: FTLD is a focal, non-Alzheimer form of dementia clinically characterized as either behavioral or aphasic variants, without amnestic syndrome, at least in the early stage of the disease (1, 2). Compared to individuals with AD, overall survival amongst those affected with FTLD is shorter (3). A proportion of patients who meet clinical criteria for AD have FTLD confirmed at autopsy, with or without neuropathological AD (4). Thus, the clinical phenotypes of the two disorders may overlap. Methods: A retrospective review of 48 neuropathologically confirmed cases of FTLD (27 had completed psychometric testing) yielded clinical and neuropsychological features for comparison with 27 age-, sex-, education-, and severity-matched individuals with AD. Results At first visit, FTLD differed from AD by behavioral abnormalities, particularly disinhibition and impulsivity, less social withdrawal, and progressive nonfluent aphasia. The two diseases had comparable executive dysfunction and memory problems. The FTLD individuals performed better than those with AD on a visual test of episodic memory, but worse on word fluency (performance correlated with aphasic features). Histopathological AD was present in 11 of the 48 FTLD individuals. Conclusions The clinical and cognitive features of FTLD may overlap with AD, particularly for memory and executive function, although behavioral problems and language difficulties distinguish those with FTLD (3, 5). FTLD individuals were more impaired than those with AD on a word fluency task, but less impaired on tests of nonverbal memory. Memory complaints in FTLD may in part reflect word-finding difficulties stemming from language dysfunction (2). Compounding the overlap of FTLD and AD clinical phenotypes is the presence of neuropathological AD in almost one-fourth of FTLD individuals. References: 1. Neary D, et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 1998 ; 51: 1546-1554. 2. Liscic RM, et al. Clinical and psychometric distinction of frontotemporal and Alzheimer dementias. Arch Neurol 2007 ; 64: 535-540. 3. Rosen HJ, et al. Utility of clinical criteria in differentiating frontotemporal lobar degeneration (FTLD) from AD. Neurology 2002 ; 58: 1608-1615. 4. Varma AR, et al. Evaluation of the NINCDS-ADRDA criteria in the differentiation of Alzheimer’ s disease and frontotemporal dementia. J Neurol Neurosurg Psychiatry 1999 ; 66: 184-188. 5. Rascovsky K, et al. Cognitive profiles differ in autopsy-confirmed frontotemporal dementia and AD. Neurology 2002 ; 58: 1801-1808.

Frontotemporal lobar degeneration; Alzheimer's dementia; FTLF-U; TDP-43; Clinical and psychometric distinction

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