Lewy Body Disease is a Contributor to Logopenic Progressive Aphasia Phenotype

被引：22

作者：

Buciuc, Marina ^{[1
]}

Whitwell, Jennifer L. ^{[2
]}

Kasanuki, Koji ^{[3
,4
]}

Graff-Radford, Jonathan ^{[1
]}

Machulda, Mary M. ^{[5
]}

Duffy, Joseph R. ^{[1
]}

Strand, Edythe A. ^{[1
]}

Lowe, Val J. ^{[2
]}

Graff-Radford, Neill R. ^{[6
]}

Rush, Beth K. ^{[7
]}

Franczak, Malgorzata B. ^{[8
]}

Flanagan, Margaret E. ^{[9
]}

Baker, Matthew C. ^{[3
]}

Rademakers, Rosa ^{[3
]}

Ross, Owen A. ^{[3
]}

Ghetti, Bernardino F. ^{[10
]}

Parisi, Joseph E. ^{[11
]}

Raghunathan, Aditya ^{[11
]}

Reichard, R. Ross ^{[11
]}

Bigio, Eileen H. ^{[9
]}

Dickson, Dennis W. ^{[3
]}

Josephs, Keith A. ^{[1
]}

机构：

[1] Mayo Clin, Dept Neurol, Rochester, MN 55905 USA

[2] Mayo Clin, Dept Radiol, Rochester, MN 55905 USA

[3] Mayo Clin, Dept Neurosci, Jacksonville, FL 32224 USA

[4] St Marianna Univ, Dept Neuropsychiat, Sch Med, Kawasaki, Kanagawa, Japan

[5] Mayo Clin, Dept Psychiat & Psychol, Rochester, MN 55905 USA

[6] Mayo Clin, Dept Neurol, Jacksonville, FL 32224 USA

[7] Mayo Clin, Dept Psychiat & Psychol, Jacksonville, FL 32224 USA

[8] Med Coll Wisconsin, Dept Neurol, Milwaukee, WI 53226 USA

[9] Northwestern Univ, Dept Pathol, Feinberg Sch Med, Cognit Neurol & Alzheimers Dis Ctr, Chicago, IL 60611 USA

[10] Indiana Univ, Dept Pathol & Lab Med, Indianapolis, IN 46204 USA

[11] Mayo Clin, Dept Lab Med & Pathol, Rochester, MN 55905 USA

来源：

ANNALS OF NEUROLOGY | 2021年 / 89卷 / 03期

基金：

美国国家卫生研究院;

关键词：

ALZHEIMERS-DISEASE; NEUROPATHOLOGIC ASSESSMENT; DEMENTIA; PATHOLOGY; ASSOCIATION; BODIES; CLASSIFICATION; HETEROGENEITY; EPSILON-4; DIAGNOSIS;

D O I：

10.1002/ana.25979

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Objective The objective of this study was to describe clinical features, [F-18]-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 alpha-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 epsilon 4 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 alpha-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 alpha-synuclein-associated functional impairment of the dominant parietal lobe. ANN NEUROL 2020

引用

页码：520 / 533

页数：14

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[1] Sleep talking and primary progressive aphasia: case study and autopsy findings in a patient with logopenic primary progressive aphasia and dementia with Lewy bodies [J].