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


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.


CONCLUSIONS
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.
Akman-Demir
G
G
Istanbul University, Istanbul Faculty of Medicine, Department of Neurology (Nöroloji), Capa 34390, Istanbul, Turkey. akmandem@istanbul.edu.tr
Gul
A
A
Gurol
E
E
Ozdogan
H
H
Bahar
S
S
Oge
A E
AE
Gurvit
H
H
Saruhan-Direskeneli
G
G
Yazici
H
H
Eraksoy
M
M
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002490:Central Nervous System / Q000473:pathology* / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D016208:Databases, Factual; D018450:Disease Progression; D004777:Environment; D010505:Familial Mediterranean Fever / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000453:epidemiology*; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000453:epidemiology*; D009413:Nerve Fibers, Myelinated / Q000473:pathology; D043025:Oligoclonal Bands / Q000134:cerebrospinal fluid; D015995:Prevalence; D012189:Retrospective Studies; D014421:Turkey / Q000453:epidemiology
D043025:Oligoclonal Bands