THU0558 Increased frequency of febrile seizures in two periodic fever syndromes: familial mediterranean fever and pfapa syndrome
Authors: H. Kilic, A. Gurup, K. Barut, E. Pehlivan, S. Sahin, A. Adrovic, O. Kasapcopur, S. Saltik
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Fever and the fear of febrile seizure risk in parents are a common reason that lead parents to seek care in childhood emergency departments. The most frequently seen periodic fever syndromes in Turkey are periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome and familial Mediterranean fever (FMF). There is lack of data regarding the frequency of febrile seizures in periodic fever syndromes. <h3>Objectives</h3> To document the frequency of febrile seizure in patients with Familial Mediterranean Fever and PFAPA syndrome. <h3>Methods</h3> Patients with FMF and PFAPA, who were diagnosed according to Turkish paediatric FMF diagnostic criteria and Marshall criteria, were enrolled to the study. Past medical history of all subjects from postnatal six months to 6 years of age were assessed in terms of febrile seizure. Clinical, demographic and laboratory data of both patient groups were obtained from patient files. The frequency of febrile seizure in both disease groups was compared with the prevalence in healthy children of a previous Turkish study. <h3>Results</h3> A total of 417 FMF and 157 PFAPA subjects were recruited to the study with a female frequency of 49.9% and 42.8%, respectively. The mean age of the FMF and PFAPA patients at study time was 12.4±4.5 years and 5.3±2.1 years, respectively. The mean age at disease onset was 5.1±3.8 (IQR: 2–7) years in FMF group, whereas it was 21±16.5 months (IQR: 10–30) in PFAPA group. The frequency of febrile seizure in FMF and PFAPA syndrome was similar (8.4% <i>versus</i> 8.6%, p&gt;0.05). Among the subjects with febrile seizure, 42.9% (n=15) in FMF group and 30.8% (n=4) in PFAPA group underwent electroencephalography. The EEG result of all subjects were reported to be normal. While 8 subjects with febrile seizure (22.9%) in FMF group required anticonvulsant therapy, 2 patients (15.4%) in PFAPA group used anticonvulsant treatment. Among the PFAPA subjects with febrile seizure (n=13), 53.9% (n=7) underwent tonsillectomy. Thereafter, 85.7% (n=6) of the patients with tonsillectomy never experienced a febrile seizure. Both the FMF and PFAPA syndrome group were found to have a higher frequency of febrile seizure compared with the healthy subjects of the previous Turkish study (FMF <i>versus</i> healthy children: 8.4% <i>versus</i> 3.2%) (PFAPA syndrome <i>versus</i> healthy children: 8.6% <i>versus</i> 3.2%); and this was statistically significant (p&lt;0.001). However, the frequency of febrile seizure in FMF did not differ from the patients with PFAPA syndrome (8.4%<i> versus</i> 8.6%, p&gt;0.05). <h3>Conclusions</h3> Compared to the healthy children, the frequency of febrile seizure in FMF and PFAPA syndrome was significantly increased. The recurrent fever, which is the main shared manifestation of these two diseases, is possibly a trigger for febrile seizure. This increased frequency of febrile seizure in both periodic syndrome seems to be a result of recurrent fever, other than a neurologic involvement of the FMF itself. <h3>References</h3> [1] Barut K, Sahin S, Adrovic A, Sinoplu AB, Yucel G, Pamuk G, et al. Familial Mediterranean fever in childhood: a single-center experience. Rheumatology international2018;38(1):67–74. [2] Yakinci C, Kutlu NO, Durmaz Y, Karabiber H, Egri M. Prevalence of febrile seizure in 3637 children of primary school age in the province of Malatya. Turk J Trop Pediatr2000;46:249–50. <h3>Disclosure of Interest</h3> None declared