Abstracts: Poster Presentations, the Seventh European Paediatric Neurology Society (EPNS) Congress 119

NOP10 Phakomatosis pigmentovascularis type IIbassociated with Sturge-Weber syndrome

F.M. Sonmez1, A. Aksoy1 *, A. Sari2. 1KTU Medical Faculty, Deptof Child Neurology, Trabzon, Turkey, 2KTU Medical Faculty, Dept ofRadiology, Trabzon, Turkey

Phakomatosis pigmentovascularis is a rare congenital cuta-neous malformation syndrom characterised by vascular andmelanocytic components. According to findings of dermalor systemic, four types have been recognised. Sturge Webersyndrome is a rare neurocutaneous disorder characterisedby port-wine nevus, leptomeningeal angiomatosis, choroidalvascular lesions and neurologic detoriation. In this article, wereport a case of phakomatosis pigmentovascularis type IIbassociated with Sturge-Weber syndrome from Turkey.Case: A three months old girl was admitted to our clinicwith complaints of cyanosis and congenital vascular andpigmentary cutaneous lesions. At one-month-old-age, shewas operated for bilaterally glaucoma in another clinic.Cranial tomography of the patient was normal at twomonths old. At the admission, there were vascular lesionsover the face and melanocytic lesions over the trunk andextremities. Cranial tomography showed calcification in theright parietal region, cerebral atrophy. Cranial MRI revealedright serebral atrophy, diffuse pial enhancement in rightserebral hemisphere and the left temporo-oksipital region.Conclusion: The association of Phakomatosis pigmentovas-cularis tip IIb with Sturge-Weber syndrome is very rare.Cerebral atrophy may occur very quickly in this case. Forthat reason, this patients must be frequently evaluatedfor occurrence of serebral atrophy and leptomeningealangiomatosis.

NOP11 Sturge-Weber syndrome. Study of 55 patients

I. Pascual-Castroviejo*, S.-I. Pascual-Pascual, R. Velazquez-Fragua, J. Viano. 1University Hospital La Paz, Madrid, Spain,2Nuestra Senora del Rosario clinic, Madrid, Spain

Objective: Epilepsy, hemiparesis, mental retardation andocular problems are the most frequent and severe featuresof patients with Sturge-Weber syndrome (SWS). Follow-upduring more than thirty years was able in several of thepatients.Methods: Fifty-five patients with SWS, 30 males and25 females, were studied.Results: The nevus flammeus was unilateral in 35 (63.5%)patients, bilateral in 17 (31%), but with unilateral lep-tomeningeal angioma in 7 (41%), and leptomeningealangioma without facial nevus flammeus in 3 (5.5%) patients.Seizures in 47 patients (85.5%) Good seizure control wasobtained in 20 patients (42.5%), but 2 of these 20 patients werecontrolled only after lobectomy. All patients with unilateralor bilateral upper eyelid nevus flammeus had unilateralor bilateral choroid-retinal angiomas. Only 36% of patientshad normal or borderline mental levels (IQ up of 70). Norelationship was observed between the size of the facialnevus flammeus or its unilateral or bilateral location.Conclusions: SWS followed a progressive course duringchildhood. Early surgical treatment controlled the seizuresbut the other problems showed a lesser response. None of ourpatients were married despite some of them having normalmental levels, seizure control, and acceptable aestheticappearance.

Epilepsy treatment − II

ETP020 Can oral ketamine administration control pediatricrefractory nonconvulsive status epilepticus?

C. Okuyaz1 *, E. Mert3, G. Bozlu2. 1Mersin University MedicalFaculty, Division of PediatricNeurology, Mersin, Turkey, 2MersinUniversity Medical Faculty, Department of Pediatrics, Mersin,Turkey, 3Mersin University Medical Faculty, Department of FamilyMedicine, Mersin, Turkey

Pediatric nonconvulsive status epilepticus is a rare disorderthat can be difficult to treat. Response to benzodiazepines,oral steroids, intravenous immunoglobulin and GABAergicmedications are variable. Ketamine is a noncompetitiveNMDA glutamate receptor antagonist that can be usedin refractory convulsive status epilepticus. In this paperwe present two cases with Lennox-Gastaut syndrome andrefractory nonconvulsive status epilepticus. In both of thepatients, nonconvulsive status epilepticus did not respondto the treatments with clonazepam, clobazam, oral steroid,intravenous immunoglobulin, valproate, topiramate, andlevetiracetam. Oral ketamine at a dose of 1.5 to 5mg/kg/day(in two divided doses) was administrated to both patients inorder to treat nonconvulsive status epilepticus. Oral ketaminetreatment was continued at a dose of 5mg/kg/day for atleast 5 days before the completion of treatment. The patientswere followed clinically and also with daily electroencephalo-graphic examinations. Oral ketamine treatment did notled to clinical and electroencephalographic improvement inpatients with nonconvulsive status epilepticus. In contrast toa previous study, our observation shows that oral ketamineis not effective in the treatment of refractory nonconvulsivestatus epilepticus in children.

ETP021 Changes of serum lipoproteins duringanticonvulsive treatment in epileptic children

L. Cvitanovic-Sojat1 *, R. Gjergja-Juraski1, Z. Jurcic1, I. Bielen2,T. Sojat1. 1Department of Pediatrics UH Sestre Milosrdnice,2Department of neurology GH Sveti Duh, Croatia

Purpose: The aim of the study was to evaluate serumlipoproteins in epileptic children on mono and polytherapy.Methods: Serum lipoproteins and hepatic enzymes weremeasured in 63 children with different types of epilepsy after�3 months of treatment and in 150 healthy children. Childrenwere separated in group 1: 4 received CBZ, 1 PH; group 2:30 received VPA, 5 TPM, 3 LTG; group 3: 14 received 2 AEDs;group 4: 6 received 3 AEDs.Results: Overage of 63 children with epilepsy was 10.5 yrs (28girls mean BMI 19.1 and 35 boys mean BMI 19.4) and of 150controls 10.4 yrs (71 girls mean BMI 18.6 and 79 boys meanBMI 18.6). In all children receiving enzyme inducing AEDstotal cholesterol, LDL and hepatic enzymes ware significantlyhigher than in controls. After replacing these AEDs with noninducing AEDs, values of total cholesterol, LDL and hepaticenzymes become normal.Conclusion: Serum lipoprotein profile should be carefullymonitored in children receiving enzyme inducing AEDs(CBZ, PH) in mono or politherapy. Because of a risk ofatherosclerotic disorders we suggest the substitution of AED.

ETP022 Antiepileptic drugs effect on vitamin D status ofepileptic children

S. Shiva*, M. Barzegar. Tabriz Children Hospital, Iran

Background: As epilepsy and seizure disorders are commonin children, antiepileptic drugs used more commonly in thisage group rather than adult. This study was carried out inorder to determining the vitamin D and calcium status of