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Eosinophilic vasculitis in an isolated central nervous system distribution
R Brian Sommerville1, James M Noble, Jean Paul Vonsattel
1Washington University in St. Louis, Neurology, 660 S. Euclid, Box 8111, St. Louis, Missouri, 63110, USA.
BMJ Case Reports
|June 21, 2011
Summary
This study reports a rare case of eosinophilic vasculitis affecting only the central nervous system (CNS). The non-granulomatous condition presented with neurological symptoms and brain lesions, responding to corticosteroid treatment.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Eosinophilic vasculitis, often linked to Churg-Strauss syndrome, rarely involves the central nervous system (CNS).
- Central nervous system involvement in eosinophilic vasculitis is less than 10% of reported cases.
Purpose of the Study:
- To present a unique case of non-granulomatous eosinophilic vasculitis isolated to the CNS.
- To describe the clinical presentation, diagnostic findings, and treatment response in this rare neurological condition.
Main Methods:
- Case report of a 39-year-old woman with acute neurological symptoms.
- Diagnostic workup included cerebrospinal fluid analysis, magnetic resonance imaging (MRI), and brain biopsy.
- Treatment involved corticosteroids.
Main Results:
- The patient presented with confusion, headache, and hemiparesis, with MRI revealing bifrontal brain lesions.
- Brain biopsy confirmed transmural inflammation with eosinophils and fibrinoid necrosis, but no granulomas.
- Symptoms resolved with corticosteroid therapy, with recurrence in different brain territories 18 months later.
Conclusions:
- This is the first reported case of primary non-granulomatous eosinophilic vasculitis exclusively affecting the CNS.
- The etiology of this isolated CNS vasculitis remains unidentified.
- Corticosteroids appear effective in managing this rare neurological disorder.
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