S106 EUROPEAN JOURNAL O F PAEDIATRIC NEUROLOGY 19s (2015) S1 – S152
P42 - 2808 was 7, he presented shock-like episodes with absences. Pro-
Neuroimaging evaluation of first afebrile seizures in pediatric longed video-EEG showed 2Hz generalized spikes and waves, age group – Qatar experience atypical absences and tonic seizures. Brain MRI revealed BFPP2. DNA analysis revealed compound heterozygoty in GPR56, par- A.M. Khair, R. Al Shami, K. Mohamed, K. Ibrahim, M. Elseid, K. Al ents where heterozygous for GPR56. Conclusion: This case is, Yefei, A. Elsetouhy, H. Kamal. Department of Pediatrics, Hamad to our knowledge, the first case of BFPP2 phenotype presenting Medical Corporation, Doha, Qatar initially as a specific learning disorder and harbouring compound Objective: To determine the prevalence of Neuro Imaging ab- heterozigoty in GPR56. normality in Children (1 month to 14 years) with first afebrile seizure. To collect evidence sufficient to make a recommenda- tion for the use of routine neuro-imaging in children with first P44 - 2863 episode of non-febrile seizure. Methods: We have reviewed charts Clinical and radiological factors associated with later occurence of all children who have presented to the Pediatric Emergency of epilepsy and epileptic encephalopathy after hypoxic ischemic Centers (PECs) in Qatar with their first afebrile seizure during insult 2012–2013 years. Labeling and definition of seizure was up to N.E. Hacıfazlıoğlu, G. Ekici, O. Ünver, D. Türkdoğan. Department of the international league against epilepsy (ILAE) booklet. From Pediatric Neurology, University of Marmara, Istanbul,Turkiye those we have found that 96 of them underwent neuroimag- ing either on urgent or non-urgent basis. Patient demographics Objective: Epileptic encephalopathy is a devastating type of and neuroimaging were reviewed in details thereafter. Results: epilepsy in early life causing severe cognitive and behavioral In our study neuroimaging abnormalities were found in (33%) of impairments. Epilepsy and epileptic encephalopathies may be children presented with their first Afberile seizure. Around ¼ of complications of hypoxic ischemic encephalopathy during the patients (27%) were below 2 years of age, who have demonstrated neonatal period. Methods: In this retrospective study 60 patients significantly higher percentage of abnormal imaging (59%). This who suffered hypoxic ischemic encephalopathy in the neonatal risk has showed decreased as children get older. In regard to period; aged 6.75±5.34 (0.30–12.09) years, 28 girls (46.7%) and 32 seizure semiology abnormal results were detected in a higher boys (53.3%) were invastigated to describe clinical and radio- rates in focal seizures compared to generalized ones (35% Vs logical factors associated with later occurence of epilepsy and 31%). Also interesting higher rates of having abnormal neu- epileptic encephalopathy were divided in 3 groups. Group 1: roimaging results were noticed in those with who presented Patients with epileptic encephalopathy (n: 27 (%45) Group 2: Pa- with status epilepticus (58%) as compared to (25%) in patients tients with epilepsy without encephalopathy (n: 18 (%30). Group with short seizures. Conclusion: Neuro-imaging should be con- 3: Patient without epilepsy and epileptic encephalopathy (n:15 sidered in any and child with first afebrile seizure with those (%25). Groups were compared for prenatal, natal and postnatal with underlying risk factors and in area having high prevalence insults, neonatal seizures, cranial USG, electroencephalography of NCC, tuberculosis and it is highly recommended in younger (EEG) in neonatal period and cranial Magnetic Resonance Imagi- infants and those with focal or prolonged seizures. Change in nation (MRI). Results: Gestational ages and birth weights of group medical management depending on neuroimaging finding may 3 were lower then Group 1 and Group 2 (p<0.05), asphyxia grades be required in about 6–8% of all case with first afebrile seizures. of Group 1 were higher then Group 3 (p<0.01). asphyxia grades of Group 2 were higher then Group 3 (p<0.05). The rate of neona- tal menengitis and sepsis of Group 1 and 2 were higher then P43 - 2841 Group 3 (p<0.05). Cortical impairment in cranial MRI in Group 1 Development coordination disorder (DCD) and attention deficit was significantly higher then Group 2 and Group 3 (p<0.05). The hyperactivity disorder (ADHD) as the initial presentation of rate of neonatal seizures were higher in all of the patients with bilateral fronto-parietal polymicrogyria type 2 (BFPP2) secondary epilepsy and epileptic encephalopathy (Group 1+Group 2) were to GPR56 mutation significantly higher then Group 3 (p<0.05). Conclusion: Higher asphyxia grades, neonatal menengitis and sepsis, cortical im- A. Aeby, P. David, S. Coppens, P. Van Bogaert. Department of pairment in cranial MRI, seizures in the neonatal period are high Pediatric Neurology, Erasme Hospital-ULB, Brussels, Belgium risk factors for evaluation of epilepsy and epileptic encephalopa- Introduction: Bilateral Fronto-Parietal Polymicrogyria type 1 (BF- thy in children suffered from HIE in neonatal period. PP1; OMIM 606854) is an autosomic recessive affection usually linked to GPR56. Clinical (mental retardation, cerebellar signs, se- vere psychomotor delay and symptomatic generalized epilepsy) P45 - 2870 and radiological (bilateral fronto-parietal polymicrogyria with an Perampanel adjunctive in pharmacoresistent epilepsy in anterior to posterior gradient with brainstem and cerebellar hy- adolescents: Efficacy and tolerability analysis poplasia) signs are specific. Patients with BFPP without cerebellar P.M. Smeyers Durá, G. Aznar Laín. Pediatric Neurology, Department or brainstem abnormalities (BFPP2) have a milder phenotype and of Pediatrics, Epilepsy Unit, Hospital Universitario y Politécnico La Fe are not linked to GPR56 mutation (Piao et al., 2005; Bahi-Buisson and Hospital del Mar, Universitat Autónoma et al., 2010). We report here a patient with specific learning disorder (Development Coordination Disorder-DCD and attention Objective: To evaluate the efficacy, safety and tolerability of deficit hyperactivity disorder-ADHD) that presented the radiolog- perampanel as add-on therapy in adolescents with focal onset ical characteristics of BFPP2 and harbor a GPR56 mutation. Case seizures aged 12 years and older in a real-life setting. Methods: report: 4-year-old boy with a normal psychomotor and speech We analyse retrospectively the electronic charts of 25 adoles- development, presenting awkwardness, learning difficulties and cent with refractory epilepsy aged 12–19 years. We collected ADHD. Familial and personal history was unremarkable. Neuro- data of epilepsy history, pharmacological previous treatment, logical examination was normal. Neuropsychological tests per- neuroimaging studies, comorbid conditions and any previous formed at 5.5 years showed DCD (Mouvement assessment Battery reaction to antiepileptic drugs. Results: 12 were males and 13 for Children-M-ABC scale −2SD and WPPSI-III: ICV=100, IRP=71, females. The etiology of epilepsy was unknown in 16% of cases IVT=71 with severe dysgraphia) and ADHD. EEG was normal. He and symptomatic in 84%. These include isquemic lesions, mal- received a multidisciplinary rehabilitation and methylphenidate formative complex lesions, cortical dysplasia, polymicrogiria and with an improvement of his learning disabilities even though others. The follow up period was from 12–3 months. 80% of he needed special school when he left kindergarten. When he patients are still on treatment. 20% (5 patients) withdraw, 4 of