Objective: To test the hypothesis that different neurocognitive networks underlie verbal fluency deficits in frontotemporal lobar degeneration (FTLD).Methods: Letter ("FAS") and semantic ("animal") fluency tests were administered to patients with a behavioral/dysexecutive disorder (bvFTLD; n ϭ 71), semantic dementia (SemD; n ϭ 21), and progressive nonfluent aphasia (PNFA; n ϭ 26). Tests measuring working memory, naming/lexical retrieval, and semantic knowledge were also obtained. MRI voxel-based morphometry (VBM) studies were obtained on a subset of these patients (bvFTLD, n ϭ 51; PNFA, n ϭ 11; SemD, n ϭ 10).
Results:Patients with SemD were disproportionately impaired on the semantic fluency measure.Reduced output on this test was correlated with impaired performance on naming/lexical retrieval tests. VBM analyses related reduced letter and semantic fluency to anterior and inferior left temporal lobe atrophy. Patients with bvFTLD were equally impaired on both fluency tests. Poor performance on both fluency tests was correlated with low scores on working memory and naming/ lexical retrieval measures. In this group, MRI-VBM analyses related letter fluency to bilateral frontal atrophy and semantic fluency to left frontal/temporal atrophy. Patients with PNFA were also equally impaired on fluency tests. Reduced semantic fluency output was correlated with reduced performance on naming/lexical retrieval tests. MRI-VBM analyses related semantic fluency to the right frontal lobe and letter fluency to left temporal atrophy. Frontotemporal lobar degeneration (FTLD) is a progressive neurodegenerative illness associated with frontal and temporal lobe atrophy.
Conclusions:1 Clinical subgroups of patients with FTLD include a decline in behavior, comportment, and executive functioning (bvFTLD) 2,3 ; semantic dementia (SemD) 4 associated with fluent progressive aphasia, impaired word comprehension, and poor object knowledge; and progressive nonfluent aphasia (PNFA) 5 associated with effortful speech and impaired grammatic comprehension. Imaging studies have shown that these patients have different distributions of cortical atrophy.
6Recent research has demonstrated that these FTLD subgroups can present with impaired executive control as seen by reduced performance on tests of letter ("FAS") and semantic ("animals" 7,8 ) fluency. Because autopsy-confirmed patients with FTLD produce differing patterns of impairment on letter as compared with semantic fluency tests, these tests may help differentiate between Alzheimer disease (AD) and FTLD and between FTLD subtypes. [9][10][11][12] Functional neuroimaging studies of healthy adults associate these tasks with partially distinct activation patterns.