Febrile seizures in children with familial Mediterranean fever: Coincidence or association?

Abstract Background Familial Mediterranean fever (FMF) is an inherited disease characterized by recurrent bouts of fever and polyserositis and caused by MEditerranean FeVer gene (MEFV) mutations. Given the febrile characteristics of the disease one would expect higher frequency of febrile seizure in this group of pediatric patients. Objectives To evaluate the frequency of febrile seizure and related factors in patients with FMF. Methods The children with the diagnosis of FMF were enrolled in the study. Information including clinical features, type of mutation and the history of febrile seizure were all noted. Results A total of 97 patients, 43 (44.3%) girls with a median age of 7.93 ± 4.05 years (2–16) and a median follow-up period of 20.65 ± 24.33 months (6–135) were included in the study. The frequency of febrile seizure in children with FMF was found as 13.4%, which is higher than the general population [ p = 0.04, OR: 2.9 (95% CI: 1.0–8.5)]. The allele frequency of exon 2 mutations in MEFV genes was higher in the patients with febrile seizure ( p = 0.03). Frequency of FMF related clinical findings (fever, abdominal pain, arthralgia/myalgia, arthritis, chest pain and erysipelas-like erythema) was similar between the two groups. However, frequency of headache was higher in the patients with febrile seizure ( p = 0.014). Conclusion The frequency of febrile seizure in children with FMF was found to be higher than the general population. Although this finding may be related to high fever during FMF attacks in individuals with genetic propensity of febrile seizure, it may also be a neurologic complication of FMF.