Speech and Language Impairments in Behavioral Variant Frontotemporal Dementia: A Systematic Review

Although behavioral variant frontotemporal dementia (bvFTD) is classically defined by behavioral and socio-emotional changes, impairments often extend to other cognitive functions. These include early speech and language deficits related to the disease s core neural disruptions. Yet, their scope and clinical relevance remains poorly understood. This systematic review characterizes such disturbances in bvFTD, considering clinically, neuroanatomically, genetically, and neuropathologically defined subgroups. We included 181 experimental studies, with at least 5 bvFTD patients diagnosed using accepted criteria, comparing speech and language outcomes between bvFTD patients and healthy controls or between bvFTD subgroups. Results reveal extensive and heterogeneous deficits across cohorts, with (a) consistent lexico-semantic, reading & writing, and prosodic impairments; (b) inconsistent deficits in motor speech and grammar; and (c) relative preservation of phonological skills. Also, preliminary findings suggest that the severity of speech and language deficits might be associated with global cognitive impairment, predominantly temporal or fronto-temporal atrophy and MAPT mutations (vs C9orf72). Although under-recognized, these impairments contribute to patient characterization and phenotyping, while potentially informing diagnosis and management. © 2021 Elsevier Ltd