Atypical acute hemorrhagic leukoencephalitis (Hurst's disease) presenting with focal hemorrhagic brainstem lesion

Nuhad E. Abou Zeid, Joseph D. Burns, Eelco F.M. Wijdicks, Caterina Giannini, B. Mark Keegan

Research output: Contribution to journalArticle

10 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)95-97
Number of pages3
JournalNeurocritical care
Volume12
Issue number1
DOIs
StatePublished - Feb 1 2010

Keywords

  • Acute hemorrhagic leukoencephalitis
  • CNS demyelinating disease
  • Hurst disease
  • Plasma exchange

ASJC Scopus subject areas

  • Clinical Neurology
  • Critical Care and Intensive Care Medicine

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