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Primary progressive aphasia (PPA) is a neurological syndrome characterized by progressive language impairment. Various neurodegenerative disorders cause PPA. Dementia with Lewy bodies (DLB) is one known cause of PPA, and little is known about this association. Almost all published cases of PPA associated with DLB are the logopenic variant of PPA. Here, we describe the novel case of a patient with DLB presenting clinical features of the semantic variant PPA (svPPA). A 75-year-old woman was referred to our hospital with a 2-year history of progressive anomia and amnesia. Two months before admission, she had been experiencing visual hallucinations, and at the age of 60 years, she had venous infarction in the left temporal lobe, which she recovered from without any residual symptoms. Upon admission to our hospital, she displayed anomia, impaired single-word comprehension, and surface dyslexia with preserved repetition and speech production. These symptoms met the criteria for the diagnosis of svPPA. 123 I-ioflupane single-photon emission computed tomography and 123 I-meta-iodobenzylguanidine myocardial scintigraphy indicated DLB. Thus, she was administered donepezil, and this dramatically improved her symptoms. We hypothesize that the combination of DLB with the previous asymptomatic venous thrombosis in the left temporal lobe may have contributed to the "svPPA" in this patient. In conclusion, we show that PPA associated with DLB could be treated with donepezil, and we suggest that donepezil should be pursued as a treatment option for PPA., Competing Interests: None., (© 2021 The Authors.)

•Pathological basis of primary progressive aphasia is heterogeneous.•Logopenic primary progressive aphasia can precede dementia with Lewy bodies (DLB).•Cholinesterase inhibitor can improve logopenic aphasia with DLB., (© 2020 The Author(s).)