Abstract Details

Title Case Series: Eosinophilic Vasculitis Isolated to the Central Nervous System

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) P02 - (-)

Poster/Presentation
Number 053

Objective

Background BACKGROUND: Eosinophilic vasculitis has been described as part of the Churg-Strauss syndrome, but affects the CNS in only 6-7% of cases. Presentation in an isolated CNS distribution is rare, with only one case previously reported in the literature by these authors. Here we present a new case of isolated CNS eosinophilic vasculitis, and exhibit it alongside the original case.

Design/Methods

Results RESULTS: A 55 year old woman with no past medical history presented to an outside institution with headache and right visual field changes. Over several days she developed non-fluent aphasia and simple partial seizures followed by right hemiparesis. CSF evaluation showed elevated protein and WBCs; MRI brain showed focal areas of leptomeningeal enhancement, EEG was unremarkable. She was started on levetiracetam and discharged, then was admitted to our institution after several weeks of decline. Two CSF analyses again showed elevated protein and WBCs with atypical lymphocytes. Repeat MRI showed focal leptomeningeal and gyral enhancment in the left hemisphere which had expanded. Brain biopsy showed transmural, predominanly eosinophilic inflammatory infiltrates of medium-sized leptomeningeal arteries without granulomas. Elevated ESR and CRP were the only remarkable serum studies, without elevation of serum eosinophils. There were no systemic manifestations of vasculitis. She was started on high dose corticosteroids with rapid improvement of her symptoms. Clinical course and diagnostic findings are similar to a prior case previously reported by these authors in 2007 which is also detailed here.

Conclusions CONCLUSIONS: To our knowledge, these are the only two cases of non-granulomatous eosinophlic vasculitis isolated to the CNS. No etiology for the vasculitic process has yet been identified. Spontaneous resolution without recurrence with corticosteroid therapy may suggest an environmental epitope with immune reaction as the cause.

Authors/Disclosures
Brenton A. Wright, MD PRESENTER	Dr. Wright has nothing to disclose.
Linda D. Lewis, MD, FAAN (Columbia University)	No disclosure on file
James M. Noble, MD, FAAN (The Neurological Institute of New YorkTaubs Institute)	Dr. Noble has received stock or an ownership interest from NoMo Diagnostics. The institution of Dr. Noble has received research support from National Institute on Aging.
	No disclosure on file
Alexander G. Khandji, MD	No disclosure on file
R B. Sommerville, MD (Washington University School of Medicine)	No disclosure on file
Clinton B. Wright, MD, FAAN (NINDS)	An immediate family member of Dr. Wright has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. Dr. Wright has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Abali Milne Kalil. Dr. Wright has received publishing royalties from a publication relating to health care. An immediate family member of Dr. Wright has received personal compensation in the range of $500-$4,999 for serving as a surveyor with ASTRO.
Johan Van Beek, PhD	No disclosure on file

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