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Inflammatory/demyelinating central nervous system involvement in familial Mediterranean fever (FMF): coincidence or

G Akman-Demir1, A Gul, E Gurol

  • 1Istanbul University, Istanbul Faculty of Medicine, Department of Neurology (Nöroloji), Capa 34390, Istanbul, Turkey. akmandem@istanbul.edu.tr

Journal of Neurology
|March 3, 2006
PubMed
Abstract

Insights

Familial Mediterranean fever (FMF) and multiple sclerosis (MS) may share genetic and environmental factors, as indicated by an increased FMF prevalence in MS patients. Further research is warranted to explore this potential link.

Area of Science:

  • Neurology
  • Immunology
  • Genetics

Background:

  • Familial Mediterranean fever (FMF) is an inherited autoinflammatory disorder causing recurrent polyserositis.
  • Central nervous system (CNS) involvement is rare in FMF, but co-occurrence with multiple sclerosis (MS) has been noted.
  • Investigating patients with both FMF and MS is crucial to understand their relationship and disease characteristics.

Purpose of the Study:

  • To assess patients diagnosed with both FMF and MS.
  • To clarify potential links between FMF and MS.
  • To evaluate the specific disease characteristics in individuals with co-occurring FMF and MS.

Main Methods:

  • Retrospective screening of an MS patient database (1986-2005) involving 2800 patients.
  • Evaluation of patients with definite MS (n=2268) and other demyelinating disorders.
  • Identification of 12 patients with FMF who developed CNS inflammatory/demyelinating disease.

Main Results:

  • Twelve FMF patients developed CNS disorders with multifocal white matter lesions.
  • Median age at FMF onset was 7 years; median neurological onset was 26.8 years.
  • Nine patients had definite MS, three had atypical demyelinating disorders; 8/8 tested patients were positive for oligoclonal IgG bands in CSF.

Conclusions:

  • The prevalence of FMF among MS patients in this cohort is nearly four times the expected rate in Turkey.
  • A familial MS and FMF case suggests shared genetic or environmental factors, though coincidence is possible.
  • A larger prospective study is recommended to validate these findings and explore the FMF-MS association.

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