Abstract
AbstractBackgroundFrontotemporal dementia (FTD) is heterogeneous and presents with behavioral or language symptoms with frontal and/or temporal atrophy. A variant presenting with anomia and word comprehension deficits, is termed semantic dementia (SD) and is associated with predominant left anterior temporal atrophy. Although the clinical presentation is different on the right side, no unique syndrome to identify right temporal variant FTD (rtvFTD) has been established.MethodsWe retrospectively collected 70 rtvFTD patients from our cohort based on their initial MRI and identified the clinical characteristics based on the case notes and compared them with those reported in Alzheimer’s disease (n=70), behavioral FTD (n=70), and SD (n=70). In a subset of cases we reported the genetic (n=6) and pathological (n=5) features along with a review of cases in the literature (n=49).ResultsBased on our single‐center study, rtvFTD presented a unique combination of cognitive (face recognition and memory impairments), behavioral/psychiatric (disinhibition, apathy, depression, and bizarre preoccupations), and language problems (word‐finding difficulties and anomia). It's genetic/pathological background was heterogeneous, in contrast to SD. The most common genetic mutation was MAPT, followed by GRN and TARDBP. The most common pathological diagnoses were FTLD‐TDP type C and FTLD‐tau, followed by FTLD‐TDP type A and B. Moreover, pyramidal degeneration was observed as a co‐pathology.ConclusionThe definition of a syndrome associated with right temporal atrophy could promote its recognition as a variant of FTD, which is crucial for individual patient management. A multicenter study will shed light on the many unanswered questions about this equivocal syndrome.
Published Version
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