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

• Published in: Journal of neurology Vol. 253; no. 7; pp. 928 - 934
• Main Authors: AKMAN-DEMIR, Gulsen, GUL, Ahmet, GUROL, Edip, OZDOGAN, Huri, BAHAR, Sara, OGE, A. Emre, GURVIT, Hakan, SARUHAN-DIRESKENELI, Guher, YAZICI, Hasan, ERAKSOY, Mefkure
• Format: Journal Article
• Language: English
• Published: Berlin Springer 01-07-2006; Springer Nature B.V
• Subjects: Adolescent; Adult; Age of Onset; Biological and medical sciences; Central Nervous System - pathology; Central Nervous System - physiopathology; Child; Child, Preschool; Comorbidity; Databases, Factual; Disease Progression; Environment; Familial Mediterranean Fever - cerebrospinal fluid; Familial Mediterranean Fever - diagnosis; Familial Mediterranean Fever - epidemiology; Genetic Predisposition to Disease - genetics; Human viral diseases; Humans; Infectious diseases; Medical sciences; Middle Aged; Multiple Sclerosis - cerebrospinal fluid; Multiple Sclerosis - diagnosis; Multiple Sclerosis - epidemiology; Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis; Nerve Fibers, Myelinated - pathology; Neurology; Oligoclonal Bands - cerebrospinal fluid; Prevalence; Retrospective Studies; Turkey - epidemiology; Viral diseases; Viral diseases of the lymphoid tissue and the blood. Aids; Turkey; inflammatory/ demyelinating CNS disease; Multiple sclerosis; Nervous system diseases; magnetic resonance imaging; central nervous system involvement; Diseases of the osteoarticular system; Central nervous system; Nuclear magnetic resonance imaging; Genetic disease; Inflammatory disease; Central nervous system disease; Familial recurrent polyseritis; familial Mediterranean fever
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-006-0137-8

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.
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.
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.
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.