Posters, Epilepsy diagnosis (EpiD) S35

033EpiDP Tourette syndrome in children diagnosed withattention-deficit/hyperactivity disorder andepileptiform abnormalities

D. Socanski1, D. Aarsland2, A. Herigstad1. 1StavangerUniversity Hospital, Stavanger Hospital Trust, Department of childpsychiatry, Stavanger, Norway, 2Stavanger University Hospital,Stavanger Hospital Trust, Division of psychiatry, Stavanger,Norway, 3Stavanger University Hospital, Stavanger Hospital Trust,Department of clinical neurophysiology, Stavanger, Norway

Tourette syndrome (TS) and attention-deficit/hyperactivitydisorder (ADHD) are common neuropsychiatric disorders andoccur as co-morbidities in many children.The aim of this study was to investigate the relationshipsbetween the occurrence of TS, ADHD symptoms andepileptiform abnormalities (EA) in children with ADHD.Method: Subjects were 517 children (81.6% male) agedbetween 5−14 years, mean 9.5+2.6. They were diagnosedwith ADHD at the Department of Child Psychiatry, betweenJanuary 2000 and December 2005. At least one standard EEGwas performed on all patients. EEG findings were coded aseither EEG with EA or EEG with nonepileptiform activity.Results: 476 (92.1%) of 517 ADHD children demonstrated EEGfinding with nonepileptiform activity. Inattentive subtypeof ADHD was diagnosed in 51 (10.7%). TS as co-morbiditywas diagnosed in 27 (5.7%) of 476 patients. 2 (7.4%) of 27ADHD children with non-epileptiform activity and TS hadinattentive subtype of ADHD. 41 (7.9%) of 517 ADHD childrendemonstrated EEG with EA. 27 children had no epilepsy co-morbidity, 14 patients had epilepsy. 17 (41.5%) of 41 patientswith EA were diagnosed with predominantly inattentive-subtype of ADHD. TS as co-morbidity was diagnosed in 6(14.8%) of 41 ADHD patients with EA. 5 of them had noepilepsy comorbidity, while one patient had epilepsy. Among6 ADHD patients with EA and TS, 2 (33.3%) patients hadinattentive subtype of ADHD.TS occurs more often in ADHD children with EA. Thesignificance of this finding is unclear but caution is warrantedregarding to diagnosis and treatments implications. ADHDchildren with EA, as well as ADHD children with EA and TS,had often inattentive subtype of ADHD.

034EpiDP A case of plasminogen deficiency associatedwith hydrocephalus, epilepsy andmultiorgan involvement

F.M. Sonmez1, E. Baltacioglu2, U. Cobanoglu3, M. Kalyoncu4.1Karadeniz Technical University, Faculty of Medicine, Dept ofChild Neurology, Trabzon, Turkey, 2Karadeniz Technical University,Faculty of Dentistry, Dept of Periodontology, Trabzon, Turkey,3Karadeniz Technical University, Faculty of Medicine, Dept ofPathology, Trabzon, Turkey, 4Karadeniz Technical University,Faculty of Medicine, Dept of Child Nephrology, Trabzon, Turkey

Introduction: Congenital plasminogen deficiency is a rareautosomal disorder characterized clinically by chronicmuscosal pseudomembranous lesions Although ophthalmicinvolvement is the most common manifestation of the dis-ease, other systemic involvement of other membranes suchas gingiva and an association with congenital hydrocephalushave been reported.We describe a case of plasminogen deficiency who hashydrocephalus, epilepsy and other systemic findings of thedisease.Case: A 14-year-old female patient who was born afteran uneventful delivery. At one year of age, she developedbilateral conjunctival pseudomembranes and was diagnosedwith ligneous conjunctivitis. These membranes recurredrepeatedly and surgical removal was made five timesat another hospital. At 3 years of age, she developedrecurrent urinary tract infection. Ureteroneosystostomy

operation was made when she was 3 years old becauseof hydronephrosis and vesicoureteral reflux. In the sameyear, she developed focal seizures and phenobarbital theparywas started. Cranial and Spinal MRI at this time showedbiventricular hyprocephalus, aquaduct stenosis, and tetheredcord. The ventriculoperitoneal shunt was applied andtetharing revision was made. One year later, she hadtwo ureteroneocutaneostomy operation because of urinaryobstruction. At nine years old, she admitted to our clinicbecause of focal motor seizures once a month. Valporoattherapy was started. Plasminogen level was 16% (normal80 100%). She was operated two times because of gingivalhypertrophy, and ligneous periodontitis was diagnosed.At 11 and 12 years of age, she was operated becauseof the shunt disfunction caused by pseudomembrans.Additional systemic manifastations included otitis media,focal glomerular sclerosis, hyperlipidemia, and osteoporosis.Result: All of the patients with ligneous conjunctivitis mustbe evaluated for plasminogen deficiency and if deficiency wasdetermined, they must be followed up for other systemicinvolvement.

035EpiDP The coexistence of idiopathic partial epilepsy andidiopathic generalized epilepsy in one child

E. Szczepanik, I. Terczynska. Department of Neurology ofChildren and Adolescents, Institute of Mother and Child, Warsaw,Poland

Purpose: to present the diagnostic and management dilem-mas in the case of 15-year-old boy with two idiopathicepileptic syndromes: benign rolandic epilepsy and juvenilemyoclonic epilepsy (JME) occurred successively.Methods: the medical record of a patient was analyzed withinterest on the age of onset, seizures types, EEG features,imaging studies and response to treatment.Results: The patient was referred to our clinic as 4-year-old child with focal seizures. At that time he wasdiagnosed as having benign rolandic epilepsy. Because ofnumerous seizures since age of 5 years he was treatedwith carbamazepine. When patient was 10 years old thepattern of seizures had changed. After occurrence of gener-alized tonic clonic seizures carbamazepine was withdrawn.Repeated EEGs revealing multiple generalized polyspike-wavedischarges accompanied by myoclonic seizures and GTCSallowed us to recognize JME. Valproic acid was introducedwith full clinical response.Conclusions: 1. Changing of the clinical and EEG picturein the course of disease should lead to considering theoccurrence of another epileptic phenotype; 2. coexistenceof both focal and generalized idiopathic epilepsies inone patient may be either coincidental or may presentthe possibility of common molecular background of theseepileptic syndromes.

036EpiDP Severe myoclonic epilepsy in infancy(SMEI or Dravet Syndrome) confirmedmolecularly a case report

E. Szczepanik1, D. Hoffman-Zacharska2, I. Terczynska1. 1Dept.of Neurology, Institute of Mother and Child, Warsaw, Poland,2Dept. of Medical Genetics, Institute of Mother and Child, Warsaw,Poland

Purpose: to present genetic background in the case of thechild with SMEI syndrome.Methods: Both clinical study of the medical record focusingon the age of onset, seizures types, course of diseaseand genetic study focusing on SCN1A gene analysis wereperformed.Results: The patient is a boy born in 1997 year withoutany obvious problems. There was no a family history of