"Semantic variant primary progressive aphasia" due to comorbidity of Lewy body disease and a previous cerebral venous infarction in the left anterior temporal lobe: A case report

Kazuo Kakinuma; Wataru Narita; Toru Baba; Osamu Iizuka; Yoshiyuki Nishio; Kyoko Suzuki

doi:10.1016/j.ensci.2021.100318

"Semantic variant primary progressive aphasia" due to comorbidity of Lewy body disease and a previous cerebral venous infarction in the left anterior temporal lobe: A case report

eNeurologicalSci. 2021 Jan 19:22:100318. doi: 10.1016/j.ensci.2021.100318. eCollection 2021 Mar.

Authors

Kazuo Kakinuma¹, Wataru Narita¹, Toru Baba^{1

2}, Osamu Iizuka¹, Yoshiyuki Nishio³, Kyoko Suzuki¹

Affiliations

¹ Department of Behavioral Neurology and Cognitive Neuroscience, Tohoku University Graduate School of Medicine, Sendai, Japan.
² Department of Neurology, National Hospital Organization Sendai-Nishitaga Hospital, Sendai, Japan.
³ Department of Psychiatry & Neurology, Tokyo Metropolitan Matsuzawa Hospital, Tokyo, Japan.

Abstract

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. ¹²³I-ioflupane single-photon emission computed tomography and ¹²³I-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.

Keywords: 123I-IMP SPECT, 123I-isopropyl-iodoamphetamine single photon emission computed tomography; 123I-MIBG, 123I-meta-iodobenzylguanidine; DLB, dementia with Lewy bodies; DaT, 123I-ioflupane single photon emission computed tomography; Dementia with Lewy bodies; Donepezil; MMSE-J, Mini-Mental State Examination Japanese version; MRI, Magnetic Resonance Imaging; MoCA-J, Montreal Cognitive Assessment Japanese version; PPA, primary progressive aphasia; Primary progressive aphasia; Semantic dementia; Stroke; TLPA, Test of Lexical Processing in Aphasia; Treatment; WAB, Western Aphasia Battery; lvPPA, logopenic variant PPA; svPPA, semantic variant PPA.

Publication types

Case Reports