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ETP01 Efficiency of valproats use in treatment of children with epileptic aphasia
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Abstracts: Poster Presentations, the Seventh European Paediatric Neurology Society (EPNS) Congress
Conclusion: To our knowledge, this association has never been reported before and may be considered a novel additional clinical component of the Axenfeld-Rieger syndrome. DMP023 Developmental status in hydrocephalic children ¨ sık2 , N. Akalan2 , B. Anlar4 . 1 Hacettepe B. Bayoglu1 *, P. Ozı¸ University Children Hospital, Child Development Unit, 2 Hacettepe University, Neurosurgery Department, 3 Hacettepe University, Neurosurgery Department, 4 Hacettepe University, Child Neurology Department, Ankara, Turkey We evaluated the developmental status of children with hydrocephaly due to various causes (prematurity, meningomyelocele, and congenital) in 65 children aged 0−6 years. Twelve % of children were under follow-up without shunt surgery, while 38% were operated within 2 months of life. The Denver II developmental test adapted and standardized for Turkey (Anlar, Yalaz, Bayoglu, 1996) was abnormal in 47% and questionable in 25%. All children who had additional problems (seizure, post-operative infection...) to hydrocephaly had abnormal test results. On the other hand, only 20% of children with isolated hydrocephaly who had shunt surgery within 2 months of life had “normal” test results. We conclude that hydrocephaly constitutes a groups where early intervention and follow-up is indicated even when diagnosis and treatment are done appropriately.
Epilepsy
treatment − I
ETP01 Efficiency of valproats use in treatment of children with epileptic aphasia G.S. Rakhimbaeva, N.M. Tuichibaeva, N. Nurmatova. Tashkent Medical Academy, Uzbekistan One of variants of current epilepsy at children is epileptic aphasia (Landau-Kleffner syndrome). The aim of research: to investigate the efficiency of Depakine preparation chrono in treatment of convulsive seizures at children with Landau-Kleffner syndrome during 1 year. 10 patients at the age from 6 till 12 with complaints of convulsive seizures arising in the morning are examined, especially at awakening and accompanied episodes of auditore agnosia, acquired aphasia. Parents of these patients marked, that last years at children elements of aphasia, agnosia have been determined. On EEG multifocal higher voltage spike waves in the temporal or parieto occipital regions are determined at 90% of children. Conducting of monotherapy of Depakine-chrono at the rate of 30 mg/kg of weight has resulted at 80% of patients to full disappearance of convulsive seizures and stabilization of status. At 20% of patients frequency of seizures have decreased up to 1−2 seizures in a year. Hence the preparation Depakine-chrono in therapeutic dosage it is possible to consider as effective means in treatment of epileptic aphasia. ETP02 Rescue therapy with high dose oral phenobarbitone loading for refractory status epilepticus S. Ackerman, J. Wilmshurst *. Red Cross Children’s Hospital, South Africa Introduction: Parenteral phenobarbitone was withdrawn in South Africa between 2005 and 2006. Oral phenobarbitone may lessen seizure recurrence in the immediate course of the illness. Aim: To establish whether there is a role for oral phenobarbitone in childhood status epilepticus. Method: All patients admitted in status epilepticus between 12/2005−06/2006 were recruited unless impaired gastric absorption or severe systemic compromise was suspected.
Patients received 20 mg/kg phenobarbitone via nasogastric tube after benzodiazepine boluses and a phenytoin infusion. Phenobarbital levels were taken post loading with routine blood sampling. Results: Sixteen patients (7 female: 9 male) were assessed, median age 5 months (range 9 days 168 months). Four were known epileptics, all had seizures related to underlying infections. No adverse effects were apparent from the oral phenobarbitone administration. Therapeutic levels were confirmed in 13 patients, in five patients by 4 hours. Levels taken later and extrapolated back suggested therapeutic levels in the region of four hours. Seizure control was documented within 1 hour, n = 5, and 4 hours, n = 6, following oral phenobarbitone loading. Conclusion: Patients tolerated oral loading with high dose phenobarbitone. Therapeutic levels were attained by 4 hours in a small cohort. This practice was a safe option for centres with limited resources and lacking parenteral phenobarbitone. ETP03 EEG-amplitude response by pyridoxine-IV in children with pyridoxine dependent epilepsy and ALDH7A1-gene mutation. D. Sival1 *, E. Struys2 , C. Jakobs2 , L. Teune1 , J. van der Hoeven1 , N. Maurits1 , L. Bok3 , O. Brouwer1 , D.-J. Reijngoud1 . 1 University Medical Center Groningen, University of Groningen, the Netherlands, 2VU University Medical Center Amsterdam, the Netherlands, 3 Maxima Medical Center, Veldhoven, the Netherlands Background: Pyridoxine dependent epilepsy (PDE) is defined by therapy resistant seizures (TRS) that respond to pyridoxine. In a PDE-subgroup [presence of ALDH7A1-gene mutation and increased concentrations of urine-alpha-AASA (“typicalPDE”)], pyridoxine-IV affects EEG-amplitudes. Recently, however, we also described declined EEG-amplitudes in TRSinfants without PDE (“non-PDE”; Seizure 2007; in press). In the present study, we aimed to investigate whether pyridoxine-IV causes a specific EEG response in “typical-PDE”. Aim: To compare the EEG-response to pyridoxine-IV in “typical-PDE” and “non-PDE”. Methods: Online EEG registrations during 100 mg pyridoxineIV were compared in a typical-PDE (ALDH7A1-gene mutation; 2 assessments) and in 9 non-PDE infants. Urine-alpha-AASA concentrations were <1 (non-PDE) and 71 mmol/mol (typicalPDE). Before and after pyridoxine-IV, EEG segments were digitally analyzed for average background amplitude, for magnitude of background-activity in a frequency-band (total power), and for contribution of a frequency-band to the spectrum (relative power). Results: During pyridoxine-IV, both “typical-” and “non-PDE” revealed: 1. persistent epileptic-EEG activity; 2. decreased EEG-central amplitude (“typical-PDE”: −49% and −27% vs “non-PDE”: −44% to −15% (median-12%); and 3. decreased central-total-power (“typical-PDE”: 47% and −14%; vs “nonPDE”: −43% to 15% (median-17%); and 4. unaffected total power. Conclusion: In “typical-PDE” infants, pyridoxine-IV causes non-specific EEG responses that do not identify ALDH7A1gene mutations. ETP04 Oxcarbazepine in pediatric patients with sleep activated discharges on EEG ¨ G. Turanli, A. Olmez *, D. Acikgoz. ¨ Hacettepe University Department of Pediatric Neurology, Ankara, Turkey Purpose: Oxcarbazepine (OXC) is indicated for the treatment of partial seizures as both monotherapy and adjunctive therapy in children with epilepsy. This study was designed
Abstracts: Poster Presentations, the Seventh European Paediatric Neurology Society (EPNS) Congress
Conclusion: To our knowledge, this association has never been reported before and may be considered a novel additional clinical component of the Axenfeld-Rieger syndrome. DMP023 Developmental status in hydrocephalic children ¨ sık2 , N. Akalan2 , B. Anlar4 . 1 Hacettepe B. Bayoglu1 *, P. Ozı¸ University Children Hospital, Child Development Unit, 2 Hacettepe University, Neurosurgery Department, 3 Hacettepe University, Neurosurgery Department, 4 Hacettepe University, Child Neurology Department, Ankara, Turkey We evaluated the developmental status of children with hydrocephaly due to various causes (prematurity, meningomyelocele, and congenital) in 65 children aged 0−6 years. Twelve % of children were under follow-up without shunt surgery, while 38% were operated within 2 months of life. The Denver II developmental test adapted and standardized for Turkey (Anlar, Yalaz, Bayoglu, 1996) was abnormal in 47% and questionable in 25%. All children who had additional problems (seizure, post-operative infection...) to hydrocephaly had abnormal test results. On the other hand, only 20% of children with isolated hydrocephaly who had shunt surgery within 2 months of life had “normal” test results. We conclude that hydrocephaly constitutes a groups where early intervention and follow-up is indicated even when diagnosis and treatment are done appropriately.
Epilepsy
treatment − I
ETP01 Efficiency of valproats use in treatment of children with epileptic aphasia G.S. Rakhimbaeva, N.M. Tuichibaeva, N. Nurmatova. Tashkent Medical Academy, Uzbekistan One of variants of current epilepsy at children is epileptic aphasia (Landau-Kleffner syndrome). The aim of research: to investigate the efficiency of Depakine preparation chrono in treatment of convulsive seizures at children with Landau-Kleffner syndrome during 1 year. 10 patients at the age from 6 till 12 with complaints of convulsive seizures arising in the morning are examined, especially at awakening and accompanied episodes of auditore agnosia, acquired aphasia. Parents of these patients marked, that last years at children elements of aphasia, agnosia have been determined. On EEG multifocal higher voltage spike waves in the temporal or parieto occipital regions are determined at 90% of children. Conducting of monotherapy of Depakine-chrono at the rate of 30 mg/kg of weight has resulted at 80% of patients to full disappearance of convulsive seizures and stabilization of status. At 20% of patients frequency of seizures have decreased up to 1−2 seizures in a year. Hence the preparation Depakine-chrono in therapeutic dosage it is possible to consider as effective means in treatment of epileptic aphasia. ETP02 Rescue therapy with high dose oral phenobarbitone loading for refractory status epilepticus S. Ackerman, J. Wilmshurst *. Red Cross Children’s Hospital, South Africa Introduction: Parenteral phenobarbitone was withdrawn in South Africa between 2005 and 2006. Oral phenobarbitone may lessen seizure recurrence in the immediate course of the illness. Aim: To establish whether there is a role for oral phenobarbitone in childhood status epilepticus. Method: All patients admitted in status epilepticus between 12/2005−06/2006 were recruited unless impaired gastric absorption or severe systemic compromise was suspected.
Patients received 20 mg/kg phenobarbitone via nasogastric tube after benzodiazepine boluses and a phenytoin infusion. Phenobarbital levels were taken post loading with routine blood sampling. Results: Sixteen patients (7 female: 9 male) were assessed, median age 5 months (range 9 days 168 months). Four were known epileptics, all had seizures related to underlying infections. No adverse effects were apparent from the oral phenobarbitone administration. Therapeutic levels were confirmed in 13 patients, in five patients by 4 hours. Levels taken later and extrapolated back suggested therapeutic levels in the region of four hours. Seizure control was documented within 1 hour, n = 5, and 4 hours, n = 6, following oral phenobarbitone loading. Conclusion: Patients tolerated oral loading with high dose phenobarbitone. Therapeutic levels were attained by 4 hours in a small cohort. This practice was a safe option for centres with limited resources and lacking parenteral phenobarbitone. ETP03 EEG-amplitude response by pyridoxine-IV in children with pyridoxine dependent epilepsy and ALDH7A1-gene mutation. D. Sival1 *, E. Struys2 , C. Jakobs2 , L. Teune1 , J. van der Hoeven1 , N. Maurits1 , L. Bok3 , O. Brouwer1 , D.-J. Reijngoud1 . 1 University Medical Center Groningen, University of Groningen, the Netherlands, 2VU University Medical Center Amsterdam, the Netherlands, 3 Maxima Medical Center, Veldhoven, the Netherlands Background: Pyridoxine dependent epilepsy (PDE) is defined by therapy resistant seizures (TRS) that respond to pyridoxine. In a PDE-subgroup [presence of ALDH7A1-gene mutation and increased concentrations of urine-alpha-AASA (“typicalPDE”)], pyridoxine-IV affects EEG-amplitudes. Recently, however, we also described declined EEG-amplitudes in TRSinfants without PDE (“non-PDE”; Seizure 2007; in press). In the present study, we aimed to investigate whether pyridoxine-IV causes a specific EEG response in “typical-PDE”. Aim: To compare the EEG-response to pyridoxine-IV in “typical-PDE” and “non-PDE”. Methods: Online EEG registrations during 100 mg pyridoxineIV were compared in a typical-PDE (ALDH7A1-gene mutation; 2 assessments) and in 9 non-PDE infants. Urine-alpha-AASA concentrations were <1 (non-PDE) and 71 mmol/mol (typicalPDE). Before and after pyridoxine-IV, EEG segments were digitally analyzed for average background amplitude, for magnitude of background-activity in a frequency-band (total power), and for contribution of a frequency-band to the spectrum (relative power). Results: During pyridoxine-IV, both “typical-” and “non-PDE” revealed: 1. persistent epileptic-EEG activity; 2. decreased EEG-central amplitude (“typical-PDE”: −49% and −27% vs “non-PDE”: −44% to −15% (median-12%); and 3. decreased central-total-power (“typical-PDE”: 47% and −14%; vs “nonPDE”: −43% to 15% (median-17%); and 4. unaffected total power. Conclusion: In “typical-PDE” infants, pyridoxine-IV causes non-specific EEG responses that do not identify ALDH7A1gene mutations. ETP04 Oxcarbazepine in pediatric patients with sleep activated discharges on EEG ¨ G. Turanli, A. Olmez *, D. Acikgoz. ¨ Hacettepe University Department of Pediatric Neurology, Ankara, Turkey Purpose: Oxcarbazepine (OXC) is indicated for the treatment of partial seizures as both monotherapy and adjunctive therapy in children with epilepsy. This study was designed