Eosinophilic vasculitis in an isolated central nervous system distribution

R. B. Sommerville, J. M. Noble, J. P. Vonsattel, R. Delapaz, Clinton B Wright

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Background: Eosinophilic vasculitis has been described as part of the Churg-Strauss syndrome, but affects the central nervous system (CNS) in <10% of cases; presentation in an isolated CNS distribution is rare. We present a case of eosinophilic vasculitis isolated to the CNS. Case report: A 39-year-old woman with a history of migraine without aura presented to an institution (located in the borough of Queens, New York, USA; no academic affiliation) in an acute confusional state with concurrent headache and left-sided weakness and numbness. Laboratory evaluation showed increased cerebrospinal fluid (CSF) protein level, but an otherwise unremarkable serological investigation. Magnetic resonance imaging showed bifrontal polar gyral-enhancing brain lesions. Her symptoms resolved over 2 weeks without residual deficit. After 18 months, later the patient presented with similar symptoms and neuroradiological findings involving territories different from those in her first episode. Again, the CSF protein level was high. She had a raised C reactive protein level and erythrocyte sedimentation rate. Brain biopsy showed transmural, predominantly eosinophilic, inflammatory infiltrates of medium-sized leptomeningeal arteries without granulomas. She improved, without recurrence, when treated with a prolonged course of corticosteroids. Conclusions: 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. Spontaneous resolution and recurrence of her syndrome is an unusual feature of the typical CNS vasculitis and may suggest an environmental epitope with immune reaction as the cause.

Original languageEnglish
Pages (from-to)85-88
Number of pages4
JournalJournal of Neurology, Neurosurgery and Psychiatry
Volume78
Issue number1
DOIs
StatePublished - Jan 1 2007
Externally publishedYes

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Vasculitis
Central Nervous System
Central Nervous System Vasculitis
Cerebrospinal Fluid Proteins
Churg-Strauss Syndrome
Migraine without Aura
Recurrence
Confusion
Hypesthesia
Blood Sedimentation
Brain
Granuloma
C-Reactive Protein
Headache
Epitopes
Adrenal Cortex Hormones
Arteries
Magnetic Resonance Imaging
Biopsy

ASJC Scopus subject areas

  • Neuropsychology and Physiological Psychology
  • Neuroscience(all)
  • Psychiatry and Mental health

Cite this

Eosinophilic vasculitis in an isolated central nervous system distribution. / Sommerville, R. B.; Noble, J. M.; Vonsattel, J. P.; Delapaz, R.; Wright, Clinton B.

In: Journal of Neurology, Neurosurgery and Psychiatry, Vol. 78, No. 1, 01.01.2007, p. 85-88.

Research output: Contribution to journalArticle

Sommerville, R. B. ; Noble, J. M. ; Vonsattel, J. P. ; Delapaz, R. ; Wright, Clinton B. / Eosinophilic vasculitis in an isolated central nervous system distribution. In: Journal of Neurology, Neurosurgery and Psychiatry. 2007 ; Vol. 78, No. 1. pp. 85-88.
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