title: Neurological Manifestations in Familial Mediterranean Fever: Results of 22 Children from a Reference Center in Kayseri, an Urban Area in Central Anatolia, Turkey.
abstract: BackgroundFamilial Mediterranean fever (FMF) is an inherited inflammatory disorder characterized by attacks of fever with polyserositis.ObjectiveThe purpose of this study was to evaluate pediatric patients with FMF who had central nervous system (CNS) findings.Materials and MethodsOur 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.ResultsNeurological findings included headache in 16 patients (72.7%), epilepsy in 6 patients (27.3%), pseudotumor cerebri in 2 patients (9.1%), tremor in 2 patients (9.1%), and multiple sclerosis in 1 patient (4.5%). The most commonMEFVgene mutation was homozygous M694V (40.9%).ConclusionsPatients with FMF can present with various CNS manifestations. Further studies that include large populations are needed to elucidate the neurological manifestations of FMF.
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
authors.forename: Mehmet
authors.initials: M
authors.lastname: Canpolat
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
authors.forename: Hakan
authors.initials: H
authors.lastname: Gumus
authors.affiliation: Division of Pediatric Nephrology and Rheumatology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
authors.forename: Zubeyde
authors.initials: Z
authors.lastname: Gunduz
authors.affiliation: Division of Pediatric Nephrology and Rheumatology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
authors.forename: Ruhan
authors.initials: R
authors.lastname: Dusunsel
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
authors.forename: Sefer
authors.initials: S
authors.lastname: Kumandas
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
authors.forename: Ayşe Kaçar
authors.initials: AK
authors.lastname: Bayram
authors.affiliation: Division of Pediatric Nephrology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
authors.forename: Sibel
authors.initials: S
authors.lastname: Yel
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
authors.forename: Hatice Gamze
authors.initials: HG
authors.lastname: Poyrazoglu
authors.affiliation: Division of Pediatric Nephrology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
authors.forename: Kenan
authors.initials: K
authors.lastname: Yilmaz
authors.affiliation: Department of Radiology and Pediatric Radiology, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
authors.forename: Selim
authors.initials: S
authors.lastname: Doganay
authors.affiliation: Department of Radiology and Pediatric Radiology, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
authors.forename: Ali
authors.initials: A
authors.lastname: Yikilmaz
authors.affiliation: Department of Medical Genetics, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
authors.forename: Munis
authors.initials: M
authors.lastname: Dundar
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
authors.forename: Huseyin
authors.initials: H
authors.lastname: Per
mesh_terms: D000293:Adolescent; D002493:Central Nervous System Diseases / Q000453:epidemiology / Q000209:etiology / Q000235:genetics / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D004252:DNA Mutational Analysis; D010505:Familial Mediterranean Fever / Q000150:complications / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006261:Headache / Q000453:epidemiology / Q000209:etiology / Q000235:genetics / Q000503:physiopathology; D006801:Humans; D007223:Infant; D008297:Male; D009154:Mutation; D000071198:Pyrin / Q000235:genetics; D012189:Retrospective Studies; D014421:Turkey / Q000453:epidemiology; D014505:Urban Population
chemical_list: C000604192:MEFV protein, human; D000071198:Pyrin