Neuropathological Findings in Susac Syndrome: An Autopsy Report

被引：32

作者：

Agamanolis, Dimitri P. ^{[1
,2
]}

Klonk, Collin ^{[2
]}

Bigley, Kim ^{[3
]}

Rennebohm, Robert M. ^{[4
]}

机构：

[1] Akron Childrens Hosp, Dept Pathol, One Perkins Sq, Akron, OH 44308 USA

[2] Northeast Ohio Med Univ NEOMED, Akron, OH USA

[3] Renoun Reg Med Ctr, Dept Neurol, Reno, NV USA

[4] Cleveland Clin Fdn, Dept Pediat Rheumatol, 9500 Euclid Ave, Cleveland, OH 44195 USA

来源：

JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY | 2019年 / 78卷 / 06期

关键词：

Cerebral vasculitis; Deafness; Encephalopathy; Susac syndrome; Visual loss; HEARING-LOSS; MICROANGIOPATHY; ENCEPHALOPATHY; BRAIN;

D O I：

10.1093/jnen/nlz031

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

A 24-year-old woman developed encephalopathy, branch retinal artery occlusion, hearing loss, and had "snowball" lesions in the corpus callosum, classic findings of Susac syndrome (SuS). Despite intensive immunosuppressive therapy, she lapsed into a coma, and died 7 months after the onset of her illness. Neuropathological examination, revealed perivascular inflammation and vasculitis involving small vessels, associated with vascular narrowing and occlusion, and numerous microinfarcts diffusely throughout the brain. The findings establish SuS as a neuroinflammatory condition that can include vasculitis. This represents the most comprehensive report of the neuropathological findings in SuS.

引用

页码：515 / 519

页数：5

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