Atypical Acute Hemorrhagic Leukoencephalitis (Hurst's disease) Presenting with Focal Hemorrhagic Brainstem Lesion

被引:16
作者
Abou Zeid, Nuhad E. [1 ]
Burns, Joseph D. [1 ]
Wijdicks, Eelco F. M. [1 ]
Giannini, Caterina [2 ]
Keegan, B. Mark [1 ]
机构
[1] Mayo Clin, Coll Med, Dept Neurol, Rochester, MN 55905 USA
[2] Mayo Clin, Dept Lab Med & Pathol, Rochester, MN 55905 USA
关键词
Hurst disease; Acute hemorrhagic leukoencephalitis; CNS demyelinating disease; Plasma exchange;
D O I
10.1007/s12028-009-9293-x
中图分类号
R4 [临床医学];
学科分类号
1002 ; 100602 ;
摘要
Background Acute hemorrhagic leukoencephalitis (AHL; Hurst's disease) is a rare, severe, inflammatory CNS disease that is typically diffuse, multifocal and associated with petechial hemorrhage. The objective of this study is to report the clinical, radiologic, and pathologic findings in a fatal AHL case with focal brainstem involvement and gross hemorrhage. Methods Patient evaluation in a tertiary neurointensive care unit with serial brain magnetic resonance imaging (MRI) and neuropathological examination on autopsy were performed. Results The patient presented with mild, then rapidly worsening, brainstem impairment to a locked-in syndrome. Brain MRI demonstrated an isolated gadolinium enhancing brainstem lesion that enlarged dramatically over weeks and was associated with gross hemorrhage and necrosis. The patient died despite aggressive treatment with intravenous corticosteroids and plasma exchange. Autopsy demonstrated the isolated severe necrotic lesion consistent with AHL. Conclusions AHL may present as a solitary brainstem lesion with gross hemorrhage and should be considered in patients with isolated enhancing brainstem lesions. AHL may be fatal even despite early, aggressive immunomodulatory therapy.
引用
收藏
页码:95 / 97
页数:3
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