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Brain stem infarction associated with familial Mediterranean fever and central nervous system vasculitis

Sebastian Luger1, Patrick N Harter, Michel Mittelbronn

  • 1Department of Neurology, Goethe-University, Frankfurt am Main, Germany. sebastian.luger@kgu.de.

Clinical and Experimental Rheumatology
|May 29, 2013
PubMed

Insights

Familial Mediterranean fever (FMF), a genetic autoinflammatory disease, can rarely cause central nervous system vasculitis. This case study highlights FMF-associated vasculitis leading to brain stem infarction in a young patient.

Area of Science:

  • Genetics
  • Immunology
  • Neurology

Background:

  • Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disorder caused by MEFV gene mutations.
  • FMF typically presents with recurrent fever and polyserositis, with rare neurological manifestations.
  • Systemic vasculitis associations with FMF, including Henoch-Schönlein purpura and Behçet's disease, are documented.

Observation:

  • A young patient with genetically confirmed FMF experienced a brain stem infarction during a typical FMF attack.
  • Diagnostic workup included cerebrospinal fluid analysis, angiography, and leptomeningeal biopsy.
  • Neurological manifestations of FMF are rare and poorly understood.

Findings:

  • The patient's stroke was attributed to FMF-associated central nervous system vasculitis.
  • This represents a rare but serious complication of Familial Mediterranean fever.
  • The study details the diagnostic process for identifying CNS vasculitis in FMF.

Implications:

  • Highlights a rare but critical neurological complication of FMF.
  • Suggests the need for heightened awareness of CNS vasculitis in FMF patients presenting with neurological symptoms.
  • Warrants further research into the pathophysiology and therapeutic strategies for FMF-associated CNS vasculitis.

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