Abstract
Behavioral variant frontotemporal dementia (bvFTD) is characterized by deep alterations in behavior and personality. Although revised diagnostic criteria agree for executive dysfunction as most characteristic, impairments in social cognition are also suggested. The study aimed at identifying those neuropsychological and behavioral parameters best discriminating between bvFTD and healthy controls. Eighty six patients were diagnosed with possible or probable bvFTD according to Rascovsky et al. (2011) and compared with 43 healthy age-matched controls. Neuropsychological performance was assessed with a modified Reading the Mind in the Eyes Test (RMET), Stroop task, Trail Making Test (TMT), Hamasch-Five-Point Test (H5PT), and semantic and phonemic verbal fluency tasks. Behavior was assessed with the Apathy Evaluation Scale, Frontal Systems Behavioral Scale, and Bayer Activities of Daily Living Scale. Each test’s discriminatory power was investigated by Receiver Operating Characteristic curves calculating the area under the curve (AUC). bvFTD patients performed significantly worse than healthy controls in all neuropsychological tests. Discriminatory power (AUC) was highest in behavioral questionnaires, high in verbal fluency tasks and the RMET, and lower in executive function tests such as the Stroop task, TMT and H5PT. As fluency tasks depend on several cognitive functions, not only executive functions, results suggest that the RMET discriminated better between bvFTD and control subjects than other executive tests. Social cognition should be incorporated into diagnostic criteria for bvFTD in the future, such as in the International Classification of Diseases (ICD)-11, as already suggested in the Diagnostic and Statistical Manual for Mental Disorders (DSM)-5.
Highlights
Behavioral variant frontotemporal dementia, the most frequent subtype of frontotemporal lobar degeneration (FTLD), is characterized by deep alterations in behavior and personality (Neary et al, 1998)
Effect sizes were strong for fluency tests, followed by the modified Reading the Mind in the Eyes Test (RMET) (−0.64) and Hamasch-Five-Point Test (H5PT) (−0.50), whereas intermediate effect sizes emerged for the Stroop task and Trail Making Test (TMT) (−0.35 for both)
Dementia severity measures Clinical Dementia Rating Scale (CDR) Frontotemporal Lobar Degeneration (FTLD-)-CDR Neuropsychological tests Stroop task Trail Making Test (TMT) Hamasch Five-Point Test (H5PT) Phonemic fluency Semantic fluency Reading the Mind in the Eyes Test (RMET) Behavioral Questionnaires Apathy Evaluation Scale (AES), self-report Apathy Evaluation Scale, informant-report Bayer Activities of Daily Living Scale (BADL), self-report Bayer Activities of Daily Living Scale, informant-report Frontal Systems Behavioral Scale (FrSBe), frequency, self-report Frontal Systems Behavioral Scale, frequency, informant-report Frontal Systems Behavioral Scale, distress, self-report Frontal Systems Behavioral Scale, distress, informant-report
Summary
Behavioral variant frontotemporal dementia, the most frequent subtype of FTLD, is characterized by deep alterations in behavior and personality (Neary et al, 1998). By focusing on clinical symptoms in histopathologically confirmed cases, an international consortium revised the diagnostic criteria for bvFTD (Piguet et al, 2011; Rascovsky et al, 2011). ‘possible’ bvFTD is defined by at least three of six clinically discriminating features: disinhibition, apathy/inertia, loss of sympathy/empathy, perseverative/stereotyped/compulsive/ritualistic behavior, hyperorality/dietary changes and, neuropsychologically, deficits in executive functioning. Biomarkers were included into Rascovsky et al.’s (2011) disease definition to increase diagnostic validity. ‘Probable’ bvFTD requires specific (frontotemporal) neuroimaging findings, whereas bvFTD ‘with definite FTLD’ has to be supported by histopathological confirmation or a pathogenic mutation. The new revised criteria have a much higher sensitivity in comparison to the earlier ones (Neary et al, 1998)
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