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Neuropediatrics 2017; 48(02): 079-085
DOI: 10.1055/s-0036-1593374
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Neurological Manifestations in Familial Mediterranean Fever: Results of 22 Children from a Reference Center in Kayseri, an Urban Area in Central Anatolia, Turkey

Mehmet Canpolat
1   Division of Pediatric Neurology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey
,
Hakan Gumus
1   Division of Pediatric Neurology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey
,
Zubeyde Gunduz
2   Division of Pediatric Nephrology and Rheumatology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey
,
Ruhan Dusunsel
2   Division of Pediatric Nephrology and Rheumatology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey
,
Sefer Kumandas
1   Division of Pediatric Neurology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey
,
Ayşe Kaçar Bayram
1   Division of Pediatric Neurology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey
,
Sibel Yel
3   Division of Pediatric Nephrology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey
,
Hatice Gamze Poyrazoglu
1   Division of Pediatric Neurology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey
,
Kenan Yilmaz
3   Division of Pediatric Nephrology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey
,
Selim Doganay
4   Department of Radiology and Pediatric Radiology, Erciyes University, Faculty of Medicine, Kayseri, Turkey
,
Ali Yikilmaz
4   Department of Radiology and Pediatric Radiology, Erciyes University, Faculty of Medicine, Kayseri, Turkey
,
Munis Dundar
5   Department of Medical Genetics, Erciyes University, Faculty of Medicine, Kayseri, Turkey
,
Huseyin Per
1   Division of Pediatric Neurology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey
› Author Affiliations
Further Information

Publication History

17 October 2015

02 August 2016

Publication Date:
22 September 2016 (online)

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Erratum: Neurological Manifestations in Familial Mediterranean Fever: Results of 22 Children from a Reference Center in Kayseri, an Urban Area in Central Anatolia, TurkeyNeuropediatrics 2017; 48(05): 402-402
DOI: 10.1055/s-0037-1599783

Abstract

Background Familial Mediterranean fever (FMF) is an inherited inflammatory disorder characterized by attacks of fever with polyserositis.

Objective The purpose of this study was to evaluate pediatric patients with FMF who had central nervous system (CNS) findings.

Materials and Methods Our medical records database for 2003 to 2014 was screened retrospectively. In total, 104 patients with FMF were identified, 22 of whom had undergone neurological examination for CNS symptoms.

Results Neurological findings included headache in 16 patients (72.7%), epilepsy in 6 patients (27.3%), pseudotumor cerebri in 2 patients (9.1%), multiple sclerosis in 1 patient (4.5%), and tremor in 1 patient (4.5%). The most common MEFV gene mutation was homozygous M694V (40.9%).

Conclusions Patients with FMF can present with various CNS manifestations. Further studies that include large populations are needed to elucidate the neurological manifestations of FMF.

Keywords

familial Mediterranean fever - neurological manifestations - M694V mutation - children

Author Contributions

M. C., H. P., H. G., A. Y., R. D., A. K. B., S. K., and S. Y. planned the study and prepared the first draft. M. C., S. Y., K. Y., H. G. P., A. K. B., and S. D. collected the patient data. Statistical analysis was performed by A. K. B. A. K. B., M. C., S. D., Z. G., H. G., S. Y., M. D., and H. P. prepared the article for publication and reviewed the literature. All the authors were involved in and contributed to patient monitoring and treatment.


 

  • References

  • 1 Saatçi U, Bakkaloglu A, Ozen S, Besbas N. Familial Mediterranean fever and amyloidosis in children. Acta Paediatr 1993; 82 (8) 705-706
    CrossrefPubMedGoogle Scholar
  • 2 Gershoni-Baruch R, Shinawi M, Leah K, Badarnah K, Brik R. Familial Mediterranean fever: prevalence, penetrance and genetic drift. Eur J Hum Genet 2001; 9 (8) 634-637
    CrossrefPubMedGoogle Scholar
  • 3 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 2006; 253 (7) 928-934
    CrossrefPubMedGoogle Scholar
  • 4 Yahalom G, Kivity S, Lidar M , et al. Familial Mediterranean fever (FMF) and multiple sclerosis: an association study in one of the world's largest FMF cohorts. Eur J Neurol 2011; 18 (9) 1146-1150
    CrossrefPubMedGoogle Scholar
  • 5 Pauwels I, Cosemans L, Boonen S, Dubois B, Goris A. Rare MEFV variants are not associated with risk to develop multiple sclerosis and severity of disease. Mult Scler 2013; 19 (9) 1132-1136
    CrossrefPubMedGoogle Scholar
  • 6 Booth DR, Gillmore JD, Lachmann HJ , et al. The genetic basis of autosomal dominant familial Mediterranean fever. QJM 2000; 93 (4) 217-221
    CrossrefPubMedGoogle Scholar
  • 7 Bakkaloğlu A. Familial Mediterranean fever. Pediatr Nephrol 2003; 18 (9) 853-859
    CrossrefPubMedGoogle Scholar
  • 8 Topçuoğlu MA, Karabudak R. Familial Mediterranean fever and multiple sclerosis. J Neurol 1997; 244 (8) 510-514
    CrossrefPubMedGoogle Scholar
  • 9 Alpayci M, Bozan N, Erdem S, Gunes M, Erden M. The possible underlying pathophysiological mechanisms for development of multiple sclerosis in familial Mediterranean fever. Med Hypotheses 2012; 78 (6) 717-720
    CrossrefPubMedGoogle Scholar
  • 10 Ugurlu S, Bolayir E, Candan F, Gumus C. Familial mediterranean fever and multiple sclerosis—a case report. Acta Reumatol Port 2009; 34 (1) 117-119
    PubMedGoogle Scholar
  • 11 Shinar Y, Livneh A, Villa Y , et al. Common mutations in the familial Mediterranean fever gene associate with rapid progression to disability in non-Ashkenazi Jewish multiple sclerosis patients. Genes Immun 2003; 4 (3) 197-203
    CrossrefPubMedGoogle Scholar
  • 12 Sayin R, Alpayci M, Soyoral YU. A case with multiple sclerosis and familial Mediterranean fever. Genet Couns 2011; 22 (3) 309-312
    PubMedGoogle Scholar
  • 13 Kalyoncu U, Eker A, Oguz KK , et al. Familial Mediterranean fever and central nervous system involvement: a case series. Medicine (Baltimore) 2010; 89 (2) 75-84
    CrossrefPubMedGoogle Scholar
  • 14 Per H, Canpolat M, Gümüş H , et al. Clinical spectrum of the pseudotumor cerebri in children: etiological, clinical features, treatment and prognosis. Brain Dev 2013; 35 (6) 561-568
    CrossrefPubMedGoogle Scholar
  • 15 Livneh A, Langevitz P, Zemer D , et al. Criteria for the diagnosis of familial Mediterranean fever. Arthritis Rheum 1997; 40 (10) 1879-1885
    CrossrefPubMedGoogle Scholar
  • 16 Lewis DW. Headaches in children and adolescents. Am Fam Physician 2002; 65 (4) 625-632
    PubMedGoogle Scholar
  • 17 Yigit S, Karakus N, Kurt SG, Ates O. Association of missense mutations of Mediterranean fever (MEFV) gene with multiple sclerosis in Turkish population. J Mol Neurosci 2013; 50 (2) 275-279
    CrossrefPubMedGoogle Scholar
  • 18 Unal A, Dursun A, Emre U, Tascilar NF, Ankarali H. Evaluation of common mutations in the Mediterranean fever gene in Multiple Sclerosis patients: is it a susceptibility gene?. J Neurol Sci 2010; 294 (1–2) 38-42
    CrossrefPubMedGoogle Scholar
  • 19 Ben-Chetrit E, Backenroth R. Amyloidosis induced, end stage renal disease in patients with familial Mediterranean fever is highly associated with point mutations in the MEFV gene. Ann Rheum Dis 2001; 60 (2) 146-149
    CrossrefPubMedGoogle Scholar
  • 20 Tunca M, Akar S, Onen F , et al; Turkish FMF Study Group. Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore) 2005; 84 (1) 1-11
    CrossrefPubMedGoogle Scholar
  • 21 Canpolat M, Kumandas S, Poyrazoglu HG, Gumus H, Elmali F, Per H. Prevalence and risk factors of epilepsy among school children in Kayseri City Center, an urban area in Central Anatolia, Turkey. Seizure 2014; 23 (9) 708-716
    CrossrefPubMedGoogle Scholar
  • 22 Feld O, Yahalom G, Livneh A. Neurologic and other systemic manifestations in FMF: published and own experience. Best Pract Res Clin Rheumatol 2012; 26 (1) 119-133
    CrossrefPubMedGoogle Scholar
  • 23 Gentiloni N, Gabbrielli N, Caradonna P , et al. Familial Mediterranean fever and electroencephalographic changes. A clinical case [in Italian]. Minerva Med 1992; 83 (5) 307-310
    PubMedGoogle Scholar
  • 24 Fedorova ML. Neurologic manifestations of periodic disease [in Russian]. Zh Nevropatol Psikhiatr Im S S Korsakova 1979; 79 (7) 912-917
    PubMedGoogle Scholar
  • 25 Eliakim M, Bental E. Recurrent polyserositis (“periodic disease”). Electroencephalographic changes. Arch Intern Med 1961; 108: 91-99
    CrossrefPubMedGoogle Scholar
  • 26 Görkem SB, Doğanay S, Canpolat M , et al. MR imaging findings in children with pseudotumor cerebri and comparison with healthy controls. Childs Nerv Syst 2015; 31 (3) 373-380
    CrossrefPubMedGoogle Scholar
  • 27 Gökalp HZ, Başkaya MK, Aydin V. Pseudotumor cerebri with familial Mediterranean fever. Clin Neurol Neurosurg 1992; 94 (3) 261-263
    CrossrefPubMedGoogle Scholar
  • 28 Ozkaya O, Bek K, Alaca N, Ceyhan M, Açikgöz Y, Taşdemir HA. Cerebral vasculitis in a child with Henoch-Schönlein purpura and familial Mediterranean fever. Clin Rheumatol 2007; 26 (10) 1729-1732
    CrossrefPubMedGoogle Scholar
  • 29 Gedalia A, Zamir S. Neurologic manifestations in familial Mediterranean fever. Pediatr Neurol 1993; 9 (4) 301-302
    CrossrefPubMedGoogle Scholar
  • 30 Cantarini L, Volpi N, Galeazzi M , et al. Colchicine myopathy and neuromyopathy: two cases with different characteristics. J Clin Rheumatol 2010; 16 (5) 229-232
    CrossrefPubMedGoogle Scholar