Logopenic aphasia due to Lewy body disease dramatically improved with donepezil

Kakinuma, Kazuo; Baba, Toru; Ezura, Michinori; Endo, Keiko; Saito, Yumiko; Narita, Wataru; Iizuka, Osamu; Nishio, Yoshiyuki; Kikuchi, Akio; Hasegawa, Takafumi; Akiyama, Masashi; Suzuki, Kyoko

doi:10.1016/j.ensci.2020.100241

Cited by 8 publications

(11 citation statements)

References 12 publications

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“…Co‐occurrence of LPA with any of the clinical core features of DLB significantly impaired performance on executive function tasks, as well as rapid improvement of cognitive performance upon initiation of cholinesterase inhibitor therapy should prompt a consideration of DLB. Noteworthy, cholinesterase inhibitor therapy in such patients might improve aphasia 9,40 …”

Section: Discussionmentioning

confidence: 99%

“…The findings add to the heterogeneity and complexity of the LPA phenotype. They also have important clinical significance as certain therapies, such as choline esterase inhibitors can be beneficial for this group of patients with LPA 9,40 . Additionally, caution should be exercised when considering neuroleptic treatment for patients with LPA who develop behavioral dyscontrol, as severe adverse effects might occur in those with DLBD 50 .…”

Section: Discussionmentioning

confidence: 99%

“…Clinicopathologic studies of patients with LPA found the most common underlying pathology to be Alzheimer's disease (AD) with the remaining minority being due to frontotemporal lobar degeneration (FTLD) 3,4 . In rare instances, other pathologies, such as Lewy body disease (LBD), have been identified often co‐occurring with intermediate‐to‐high likelihood of AD 5–10 raising the question of whether any other pathologies could be playing a role in the LPA syndrome.…”

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confidence: 99%

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Lewy Body Disease is a Contributor to Logopenic Progressive Aphasia Phenotype

Buciuc

Whitwell

Kasanuki

et al. 2020

Annals of Neurology

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Objective The objective of this study was to describe clinical features, [18F]‐fluorodeoxyglucose (FDG)‐positron emission tomography (PET) metabolism and digital pathology in patients with logopenic progressive aphasia (LPA) and pathologic diagnosis of diffuse Lewy body disease (DLBD) and compare to patients with LPA with other pathologies, as well as patients with classical features of probable dementia with Lewy bodies (pDLB). Methods This is a clinicopathologic case‐control study of 45 patients, including 20 prospectively recruited patients with LPA among whom 6 were diagnosed with LPA‐DLBD. We analyzed clinical features and compared FDG‐PET metabolism in LPA‐DLBD to an independent group of patients with clinical pDLB and regional α‐synuclein burden on digital pathology to a second independent group of autopsied patients with DLBD pathology and antemortem pDLB (DLB‐DLBD). Results All patients with LPA‐DLBD were men. Neurological, speech, and neuropsychological characteristics were similar across LPA‐DLBD, LPA‐Alzheimer's disease (LPA‐AD), and LPA‐frontotemporal lobar degeneration (LPA‐FTLD). Genetic screening of AD, DLBD, and FTLD linked genes were negative with the exception of APOE ε4 allele present in 83% of LPA‐DLBD patients. Seventy‐five percent of the patients with LPA‐DLBD showed a parietal‐dominant pattern of hy pometabolism; LPA‐FTLD – temporal‐dominant pattern, whereas LPA‐AD showed heterogeneous patterns of hypometabolism. LPA‐DLBD had more asymmetrical hypometabolism affecting frontal lobes, with relatively spared occipital lobe in the nondominantly affected hemisphere, compared to pDLB. LPA‐DLBD had minimal atrophy on gross brain examination, higher cortical Lewy body counts, and higher α‐synuclein burden in the middle frontal and inferior parietal cortices compared to DLB‐DLBD. Interpretation Whereas AD is the most frequent underlying pathology of LPA, DLBD can also be present and may contribute to the LPA phenotype possibly due to α‐synuclein‐associated functional impairment of the dominant parietal lobe. ANN NEUROL 2021;89:520–533

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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Lewy Body Disease is a Contributor to Logopenic Progressive Aphasia Phenotype

Buciuc

Whitwell

Kasanuki

et al. 2020

Annals of Neurology

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“…In summary, we report a patient with svPPA associated with DLB whose language dysfunctions were resolved following the administration of donepezil. We have previously reported that donepezil improved language symptoms in a case of DLB and Alzheimer's disease with lvPPA [ 3 ]. These cases suggest that donepezil is a promising treatment option for PPA associated with DLB and associated pathologies.…”

Section: Discussionmentioning

confidence: 99%

“…One rare cause of PPA is dementia with Lewy bodies (DLB), and little is known about the characteristics of PPA associated with DLB. To date, almost all published cases of PPA associated with DLB are lvPPA [ [3] , [4] , [5] , [6] , [7] , [8] ]. In this report, we describe the novel case of a patient with DLB presenting with the clinical features of svPPA.…”

Section: Introductionmentioning

confidence: 99%