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epilepsy, higher than quoted internationally; 50% of thesehave avoidable aetiologies. The presence of co-morbidities issignificant for the large cohort of patients.

P02.8 Epilepsy, convulsions and epileptiform abnormalitieson EEG in children with cystic periventricularleukomalacia

Lj. Popovic Miocinovic1 *, K. Bosnjak Nadj1, M. Mladin Cikara1.1Special hospital for children with neurodevelopmental and motoricdisorders, Zagreb, Croatia

Background: Cystic periventricular leukomalacia (cPVL) is atype of hypoxic-ischemic brain injury that occurs mainlyin premature infants. The major long-term sequelae ofcPVL are cerebral palsy, delayed cognitive development,visual impairment, and seizures. Aim of the study is topresent occurrence of epilepsy, convulsions and epileptiformabnormalities on EEG in children with cystic periventricularleukomalacia.Methods: In retrospective study we investigated the fre-quency and characteristics of epilepsy, convulsions andepileptiform abnormalities on EEG (recorded during sleepafter sleep deprivation) in 43 children with cerebral palsycaused by cystic periventricular leukomalacia. The follow-upperiod was 7−13 years (mean 9.8).Results: All children have spastic cerebral palsy, most ofthem have spastic quadriplegia 35/43 (81%). Epilepsy waspresent in 18/43 (42%) children, provoked convulsions in 4/43(9%) and epileptiform abnormalities on EEG (without epilepsydiagnosis) in 20/21 (95%). Ohtahara syndrome was presentin 1/43 child, West syndrome in 11/43 (25%), symptomaticlocalization-related epilepsy in 6/43 (14%) and epilepsy afterWest syndrome in 7/11 (63%). Febrile convulsions wereseen in 3/43 and one child had convulsions during generalanesthesia. The most common location of the epileptiformdischarges on EEG (without epilepsy diagnosis) was in theparieto-occipital area 16/21 (76%), and in the fronto-centralarea.Conclusion: More than a third of the children with cPVLhave epilepsy. They also often have provoked attacks (febrileseizures and convulsions with anesthesia). Most childrenhave epileptiform EEG but without clinical seizures.

P02.9 The prevalence of symptomatic epilepsies amongchildren in two large pediatric neurology centres inPoland − one year prospective study

M. Mazurkiewicz-Beldzinska1 *, B. Steinborn2, M. Szmuda1,A. Winczewska-Wiktor2. 1Dept. of Developmental Neurology,Medical University of Gdansk, Poland, 2Dept. of DevelopmentalNeurology, Poznan Medical University, Poland

Rationale: To evaluate the prevalence of symptomaticepilepsies among children and adolescents with epilepsy.Methods: All epilepsy patients who entered the Develop-mental Neurology Departments (In and Outpatients Clinics)in the period between 01.10.2008 01.10.2009 were includedin the study and followed prospectively. 1053 children andadolescents with dagnosed and treatet epilepsy enteredthe study. The symptomatic epilepsy group cosisted of 523patients − they presented clear documented etiology. Thecomparision of etiology, type of seizures and treatmenteffects was performed.Results: The most common type of seizures amongsymptomatic patients were complex partial seizures andgeneralized seizures (mainly tonic, myoclonic and atypicalabsance). There were 64% of patients from symptomaticgroup whowere drug resistant according to recently proposeddefinition (Kwan et al. 2010). The comparisions betweensymtomatic and non-symptomatic group was done. There

were significant differences in types of treatment andnumber of drugs in therapy.Conclusions: This large population based study showsthe differences between the treatment strategies amongsymptomatic and non-symptomatic groups, in our opinionnot always justified and presents the surprisingly highincidence of drug-reistant epilepsies among children andadolescents with symptomatic epilepsies.

P02.10 A swedish overview of Dravet syndrome

C. Rosander1, T. Hallbook1 *. 1The Queen Silvia children’shospital, Gothenburg, Sweden

Background: Dravet syndrome (DS) is a severe epilepticencephalopathy with seizure on-set in the first year of life.Prolonged hemiclonic or generalized seizures associated withfever are typical and later on atypical absences, myoclonia,mental retardation and autism develop. 75% of patients havea mutation in the SCN1A-gene, which codes for a neuronalsodium channel.This severe myoclonic epilepsy was first described in 1978,and, since then, more than 500 cases have been described.Aim of study: To collect and summarize data regarding allSwedish children diagnosed with DS.Method:A letter was sent to all neuropediatricians in Sweden,asking if they have patients with the electroclincial profile ofDS. If they had such patients, they were asked to completea questionnaire about family history of febrile seizures andepilepsy, genotype, seizure types, seizure frequency, age atseizure onset, age at diagnosis, level of mental capacity, otherfeatures, medications, habilitation and financial support.Results: Out of 28 patients (age 2−20) identified 23 hadthe SCN1A-mutation, 25 had mental retardation, 15 hadautism, 20 had clutched gait and/or ataxia, 22 had had statusepilepticus, 22 had myoclonia, 21 had focal seizures, 7 wereseizure free. Median age at seizure onset was 4m, and atdiagnosis 4y.Conclusions: Other studies have shown an incidence of DSof around 1/30000, which would approximate the number ofcases in Sweden to 53. However, in our study we found only28 cases of diagnosed DS, indicating that the condition isunderdiagnosed in Sweden.

P02.11 Myoclonic-Astatic Epilepsy: an age-dependentepileptic syndrome with favourable seizuresoutcome but variable cognitive evolution

M. Trivisano1 *, N. Specchio2, S. Cappelletti2, V. Di Ciommo2,D. Claps2, M. Di Capua2, M. Valeriani2, L.M. Specchio1,F. Vigevano2, L. Fusco2. 1Clinic of Nervous System Disease,University of Foggia, Foggia, Italy, 2Bambino Gesu Children’sHospital IRCCS, Rome, Italy

Background: Prognosis of Myoclonic-Astatic Epilepsy (MAE)has been reported to be highly variable.Aim of the study: To explore clinical, electroencephalography(EEG), neuropsychological features and above all prognosis ofMAE.Methods: 18 patients had MAE according to clinical and EEGcriteria. We analysed clinical, electroencephalographic andneuropsychological features of these patients.Results and Conclusions: Male predominated (88.9%). Ageat onset ranged from 2.3 to 4.9 years. Median follow-up period was 6.3 years. Familiarity was reported in44.4%. In addition to myoclonic-astatic seizures patientshad myoclonic seizures (66.7%), drop attacks (72.2%), atonicseizures (77.8%) absences (88.9%), tonic-clonic generalizedseizure (77.8%), tonic seizures (38.9%), non-convulsive statusepilepticus (NCSE) (16.7%). Seven patients (38.9%) hadepileptic encephalopathy. At onset interictal epileptiformand slow abnormalities were recorded, respectively, in 100%