Skip to main content

Advertisement

SpringerLink
Log in

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

  • ORIGINAL COMMUNICATION
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

Background and Objective

Familial Mediterranean fever (FMF) is an inherited inflammatory disease characterized by recurrent febrile polyserositis. Central nervous system (CNS) involvement in FMF is uncommon, but recently cases with multiple sclerosis (MS) and FMF have been reported. Here we assess patients with both FMF and MS, in order to clarify any relationship between FMF and MS, and to evaluate disease characteristics.

Patients and Methods

Our MS database between 1986–2005 was screened retrospectively, and patients with both FMF and inflammatory/demyelinating CNS disease were evaluated among a total of 2800 patients including definite MS (n = 2268) and other demyelinating disorders.

Results

There were 12 patients with FMF, who developed a CNS disorder with multifocal white matter lesions. Median age at onset of FMF was 7 years, and median age at neurological onset was 26.8 years. Nine patients (including two siblings) had definite MS according to clinical and MRI findings, whereas 3 patients had atypical features suggesting other demyelinating disorders. Disease severity varied among the patients between very mild to a fatal course. All 8 patients evaluated for oligoclonal IgG bands in CSF were positive.

Conclusion

The rate of FMF among our patients with definite MS is almost 4 times the expected prevalence in Turkey. Our series including a sibling pair concordant for FMF and MS may suggest that similar genetic susceptibility and environmental factors might be responsible, although coincidence still remains a possibility. A prospective study on a larger sample seems to be justified.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Babior BM, Matzner Y (1997) The Familial Mediterranean fever genecloned at last. N Engl J Med 337:1548–1549

    Article  PubMed  CAS  Google Scholar 

  2. Celik Y, Birgili O, Kiyat A, Guldiken B, Yilmaz H, Saip S, Yandim–Kuscu D, Sutlas N, Agaoglu J, Utku U, Siva A, and members of the Edirne MS Prevalence Study Group (2003) Prevalence of multiple sclerosis in the metropolitan area of Edirne city, Turkey. Mult Scler P211

  3. Chataway J, Feakes R, Coraddu F, et al. (1998) The genetics of multiple sclerosis: principles, background and updated results of the UK systematic genome screen. Brain 121:1869–1887

    Article  PubMed  Google Scholar 

  4. Dean G, Aksoy H, Akalin T, Middleton L, Kyriallis K (1997) Multiple sclerosis in the Turkish– and Greek speaking communities of Cyprus. A United Nations (UNHCR) Bicommunal Project. J Neurol Sci 145:163–168

    Article  PubMed  CAS  Google Scholar 

  5. Eliakim M, Bental E (1961) Recurrent polyserositis – electroencephalographic changes. Arch Int Med 108:91

    CAS  Google Scholar 

  6. Eraksoy M, Hensiek A, Kurtuncu M, et al. (2003) A genome screen for linkage disequilibrium in Turkish multiple sclerosis. J Neuroimmunol 143:129–132

    Article  PubMed  CAS  Google Scholar 

  7. Eraksoy M, Kurtuncu M, Akman– Demir G, et al. (2003) A whole genome screen for linkage in Turkish multiple sclerosis. J Neuroimmunol 143:17–24

    Article  PubMed  CAS  Google Scholar 

  8. The French FMF consortium (1997) A candidate gene for familial Mediterranean fever. Nat Genet 17:25–31

    Article  Google Scholar 

  9. The International FMF consortium (1997) Ancient missense mutations in a new member of the RoRet gene family are likely to cause familial Mediterranean fever. Cell 90:797–807

    Article  Google Scholar 

  10. Karabudak R, Dogulu CF, Nurlu G, Simsek H, Saatci I (2003) Central nervous system demyelination in familial mediterranean fever: Is it a coincidence? Eur J Neurol 10:327–328

    Article  PubMed  CAS  Google Scholar 

  11. Linetsky E, Ben–Cherit E, Lossos A (1999) Neurologic aspects of Familial Mediterranean Fever. J Neurol 246 (Suppl):I/134

    Google Scholar 

  12. Livneh A, Langevitz P, Zemer D, Zaks N, Kees S, Lidar T, Migdal A, Padeh S, Pras M (1997) Criteria for the diagnosis of Familial Mediterranean Fever. Arthritis Rheum 40:1879–1885

    PubMed  CAS  Google Scholar 

  13. Lossos A, Eliashiv S, Ben–Chatrit E, Reches A (1993) Optic neuritis associated with Familial Mediterranean Fever. J Clin Neuroophthalmol 13:141–143

    PubMed  CAS  Google Scholar 

  14. Mc Donald I, Compston A, Edan G, et al. (2001) Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Ann Neurol 50:121–127

    Article  Google Scholar 

  15. Ozdogan H, Arisoy N, Kasapcopur O, Sever L, Caliskan S, Tuzuner N, Mat C, Yazıcı H (1997) Vasculitis in familial mediterranean fever. J Rheumatol 24:323–327

    PubMed  CAS  Google Scholar 

  16. Ozen S, Karaaslan Y, Ozdemir O, et al. (1998) Prevalence of Juvenile and chronic arthritis and Familial Mediterranean Fever in Turkey: a field study. J Rheumatol 25:2445–2449

    PubMed  CAS  Google Scholar 

  17. Ozkan E, Caliskan A, Idil MK, et al. (1978) Electroencephalographic changes in familial mediterranean fever and its relation to abdominal epilepsy. Bulletin of Istanbul Faculty of Medicine 41:70–77

    Google Scholar 

  18. Pras M, Kastner DL (1998) Familial Mediterranean fever. In: Klippel JH, Dieppe PA (eds) Rheumatology. Mosby Year Book Ltd, pp, 5. 23. 1–5. 23. 4

  19. Schwabe AD, Monroe JB (1988) Meningitis in familial mediterranean fever. Am J Med 85:715–717

    Article  PubMed  CAS  Google Scholar 

  20. Shinar Y, Livneh A, Villa Y, Pinhasov A, Zeitoun I, Kogan A, Achiron A (2003) Common mutations in the FMF gene associate with rapid progression to disability in non–Ashkenazi Jewish MS patients. Genes Immun 4:197–203

    Article  PubMed  CAS  Google Scholar 

  21. Thorpe JW, Kidd D, Moseley IF, et al. (1996) Spinal MRI in patients with suspected multiple sclerosis and negative brain MRI. Brain 119:709–714

    PubMed  Google Scholar 

  22. Tinaztepe K, Gucer S, Bakkaloglu A, Tinaztepe B (1997) Familial Mediterranean fever and polyarteritis nodosa: experience of five pediatric cases. A causal relationship or coincidence? Eur J Pediatr 156:505–508

    PubMed  CAS  Google Scholar 

  23. Topaloglu R, Saatci U, Bakkaloglu A, Besbas N, Bassoy V (1992) Evaluation of the hypercoagulable state by measuring protein–C and antithrombin–III levels in nephrotic syndrome and in familial mediterranean fever–related amyloidosis Turk J Pediatr 34:15–20

    CAS  Google Scholar 

  24. Topcuoglu MA, Karabudak R (1997) Familial mediterranean fever and multiple sclerosis. J Neurol 244:510–514

    Article  PubMed  CAS  Google Scholar 

  25. Touitou I, Lesage S, McDermott M, Cuisset L, Hoffman H, Dode C, Shoham N, Aganna E, Hugot JP, Wise C, Waterham H, Pugnere D, Demaille J, Sarrauste de Menthiere C (2004) Infevers: an evolving mutation database for auto–inflammatory syndromes. Hum Mutat 24:194–198

    Article  PubMed  CAS  Google Scholar 

  26. Tunca M, Akar S, Onen F, Ozdogan H, Kasapcopur O, Yalcinkaya F, Tutar E, Ozen S, Topaloglu R, Yilmaz E, Arici M, Bakkaloglu A, Besbas N, Akpolat T, Dinc A, Erken E, Turkish FMF Study Group (2005) Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine 84:1–11

    Article  PubMed  Google Scholar 

  27. Yucesan C, Canyigit A, Turkcapar N (2004) The coexistence of familial Mediterranean fever with multiple sclerosis. Eur J Neurol 11:716–717

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Istanbul University, Istanbul Faculty of Medicine, Department of Neurology (Nöroloji), Çapa 34390, Istanbul, Turkey

    G. Akman-Demir, E. Gurol, S. Bahar, A. E. Oge, H. Gurvit & M. Eraksoy

  2. Istanbul University, Istanbul Faculty of Medicine, Department of Rheumatology, Istanbul, Turkey

    A. Gul

  3. Cerrahpasa Faculty of Medicine, Department of Rheumatology, Istanbul, Turkey

    H. Ozdogan & H. Yazici

  4. Istanbul University, Istanbul Faculty of Medicine, Department of Physiology, Istanbul, Turkey

    G. Saruhan-Direskeneli

Authors
  1. G. Akman-Demir
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. A. Gul
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. E. Gurol
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. H. Ozdogan
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. S. Bahar
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. A. E. Oge
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. H. Gurvit
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. G. Saruhan-Direskeneli
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. H. Yazici
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. M. Eraksoy
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to G. Akman-Demir.

Additional information

Part of this study was presented at the Eighth Meeting of the European Neurological Society (ENS) in Nice, June 1998.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Akman-Demir, G., Gul, A., Gurol, E. et al. Inflammatory/demyelinating central nervous system involvement in familial Mediterranean fever (FMF): coincidence or association?. J Neurol 253, 928–934 (2006). https://doi.org/10.1007/s00415-006-0137-8

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00415-006-0137-8

Key words

Search

Navigation