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BMJ Case Reports 2009; doi:10.1136/bcr.08.2008.0832
  • Rare disease

Eosinophilic vasculitis in an isolated central nervous system distribution

  1. R Brian Sommerville1,
  2. James M Noble2,
  3. Jean Paul Vonsattel3,
  4. Robert Delapaz4,
  5. Clinton B Wright5
  1. 1
    Washington University in St. Louis, Neurology, 660 S. Euclid, Box 8111, St. Louis, Missouri, 63110, USA
  2. 2
    Columbia University, Neurology, 16th Fl, Harlem Hospital Ctr, 506 Lenox Avenue, New York, 10037, USA
  3. 3
    Columbia University, Pathology, T8, 3959 Broadway, BHC, New York, 10032, USA
  4. 4
    Columbia University, Radiology, MHB3-111, 177 Fort Washington Ave, New York, 10032, USA
  5. 5
    University of Miami, Neurology, CRB 1349, 1120 NW 14th Street, Miami, Florida, 33136, USA
  1. cwright{at}med.miami.edu
  • Published 2 February 2009

Summary

Eosinophilic vasculitis has been described as part of the Churg–Strauss syndrome, but affects the central nervous system (CNS) in <10% of cases. A 39-year-old woman with a history of migraine without aura presented to an institution in an acute confusional state with concurrent headache and left-sided weakness. Laboratory evaluation showed an increased cerebrospinal fluid (CSF) protein level, but otherwise unremarkable serologies. Magnetic resonance imaging showed bifrontal polar gyral-enhancing brain lesions. Her symptoms resolved over two weeks without residual deficits. Eighteen months later the patient presented with similar symptoms and neuroradiological findings showed involvement of territories different from those in her first episode. Brain biopsy showed transmural, predominantly eosinophilic, inflammatory infiltrates and fibrinoid necrosis without granulomas. She improved when treated with corticosteroids. To our knowledge, this is the first case of non-granulomatous eosinophilic vasculitis isolated to the CNS. No aetiology for this patient’s primary CNS eosinophilic vasculitis has yet been identified.

Footnotes

  • Competing interests: None.

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