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P115 Absence epilepsy and electroencephalographic focal abnormalities
S56 epilepsy (any kind), or a “benign” epilepsy (primary generalised or benign age-related focal epilepsies with no additional educational needs or neurodisability). Crosschecked with the hospital PAS system, all children who died between 01/01/98 and 31/12/06 were identified and their casenotes reviewed. Each child was assigned to a group (A: identified cause of death, no seizure disorder in life; B: identified cause of death with seizure disorder in life but not thought related to death; C: seizure related death; D: known epilepsy, unexplained death). Casenotes of Groups C and D were reviewed to identify neurodevelopmental disability. The audit was then extended to include children who may not have been seen by a Paediatric Neurologist. Death certificates of all children who died in the region between 1989 and 2005 were reviewed to identify those with epilepsy. Their casenotes have been requested and are to be reviewed. Results: From the initial audit, 67 children died in the period studied. 12 children were thought to have had seizurerelated deaths. SUDEP occurred in 4 cases, three of whom had severe learning disability. The overall epilepsy-related mortality (Groups C&D) was 0.01% (16/1549). The 95% upper confidence interval (CI) on mortality is 0.015%. In the 278 children with “benign” epilepsy there was 1 death due to a road traffic accident (95% upper CI on mortality 0.007%). Further data analysis is on-going. Conclusion: SUDEP is a rare event and not the major cause of death in children with epilepsy. Distingiushing the cause of death in children with epilepsy and neurodisability is difficult. Reference(s) [1] National Sentinel Clinical Audit of Epilepsy-Related Death 2002. [2] Camfield CS, Camfield PR, Veugelers PJ. Death in children with epilepsy: a population based study. Lancet 2002; 359: 1891−95. [3] The epilepsies: diagnosis and management of the epilepsies in children and young people in primary and secondary care. Clinical Guideline 20. NICE October 2004. [4] www.sudep.org
P114 Clinical picture and therapeutic results in epilepsy caused by cytomegalovirus infection D. Dunin-Wasowicz1 *, J. Mulawka2 , P. Zielinski2 . 1 Neurology and Epileptology Department, The Children’s Memorial Health Institute, Warsaw, Poland; 2 Faculty of Electronics and Information Technology, Warsaw University of Technology, Warsaw, Poland Objective: Symptomatic epilepsy, which starts during the first year of life, may be caused by intrauterine and perinatal cytomegalovirus (HCMV) infection, so estimation of seizure semiology and therapy was performed. Methods: Follow-up of 138 infants treated with antiepileptic drugs − AEDs (including 93 with ganciclovir-GCV therapy) due to epilepsy and cytomegalovirus infection. HCMV DNA detection was carried out by PCR methods in CSF, blood leukocytes and urine. Statistical analysis was performed using chi 2 test and ANOVA. Results: Infantile spasms (IS), generalized tonic-clonic (GTCS), partial onset (POS) and polymorphic (PLM) seizures occurred in 109 infants before the age of 6 months. In 32 infants treated with ganciclovir, HCMV DNA was found in CSF, and in 106 only in blood leukocytes and urine. 61 infants were also treated with GCV and 45 infants received AEDs without GCV. Median of observation period was 5 years. 74 infants have cerebral palsy. Patients with GTCS were frequently treated with AEDs monotherapy (p = 0.033). AEDs monotherapy was also possible in infants with epileptic seizures and cytomegalovirus hepatitis (p = 0.006). In patients with IS, tetraparesis was significantly frequent (p = 0.003), but infants with POS usually develop normally (p = 0.002). Patients with tetraparesis received at least two AEDs (p = 0.006) and frequently have SNHL (p = 0.003). In 24 patients (21 treated
Posters with GCV) AEDs withdrawal was possible. AEDs withdrawal was successfully performed without recurrence of epileptic seizures in 11 out of 32 with HCMV neuroinfection, in 10 out of 61 also treated with GCV and only in 3 out of 45 infants without antiviral therapy. AEDs withdrawal was most frequent in patients with epilepsy and HCMV neuroinfection treated with GCV (p = 0.009). Conclusion: GCV therapy is very important for AEDs withdrawal, especially when HCMV neuroinfection is diagnosed early. P115 Absence epilepsy and electroencephalographic focal abnormalities S. Teber1 *, A. Yılmaz1 , G. Deda1 . 1 Pediatric Neurology, Ankara University Faculty Of Medicine, Ankara, Turkey Typical absences are associated with bilateral, synchronous and symmetric spike-and wave discharges at 3 cycles per second. However, the coexistence of focal EEG discharges associated with absence epilepsy has been rarely documented. Video EEG recordings of twelve patients; 7 was childhood absence epilepsy and 5 was juvenile absence epilepsy were evaluated. Eight of them had focal EEG abnormalities but none of them had focal clinical manifestations. A family history of epilepsy was found in seven cases (58%). Neurologic and neuropsychological examinations were normal in all cases. Brain MRI was performed in 7 cases and were normal. The background EEG rhythms were normal in all patients. The interictal EEG recordings revealed left centroparietal spikes in 1 patient, frontocentral spike in one, left temporooccipital spike in one, left frontal spike in one, bilateral frontal spike in four, bilateral frontal multiple spike wave in four, temporooccipital slow wave with left predominance in 2 and bilateral temporooccipital slow wave in two patients. Photoparoxysmal response on the EEG was positive in three children, and closing of the eyes did not activate absences in any of the children. The cause of the existence of focal EEG discharges in cases with CAE has not been explained as yet. Focal and generalized EEG paroxysms may be an expression of common and shared genes in families with generalized epilepsy and families with benign focal epilepsies. From pathophysiological point of view, another issue that could be involved is the theory stating that absence epilepsy might in fact have a focal onset. P116 Adrenocorticotrop hormone therapy in acquired childhood epileptic aphasia 1 L. Szabo´ 1 , J. Nagy1 , R. Kalm *. 1 2nd Department of ´ anchey ´ Paediatrics, Semmelweis University, Budapest
Although Landau Kleffner syndrome, a childhood-acquired epileptic aphasia, is frequently studied both its underlying pathophisiology and optimal therapy have remained unknown. Our study is aimed to investigate the efficacy of ACTH therapy in Landau Kleffner syndrome. We have analysed retrospectively the documentation of 5 children treated by ACTH, who suffered from Landau Kleffner syndrome. We studied the longitudinal changes of the four most characteristic sympthoms and signs of the syndrome: epileptiform EEG, speech and behaviour disorders, seizures together with the ACTH regimes. Besides, we analysed the relation between the starting date of the therapy and its efficacy. Before giving ACTH, epileptiform EEG and speech disorders were observed in all the 5 children, seizures in 4 of them, behaviour disorders in 3 of them. With 2 patients the speech disorder had been persisting for years before. Due to the starting ACTH stosstherapy (20E/day for 1−2 weeks) all the four examined signs disappeared or showed quick softening in all the 5 children in maximum 2 weeks. We adjusted
P114 Clinical picture and therapeutic results in epilepsy caused by cytomegalovirus infection D. Dunin-Wasowicz1 *, J. Mulawka2 , P. Zielinski2 . 1 Neurology and Epileptology Department, The Children’s Memorial Health Institute, Warsaw, Poland; 2 Faculty of Electronics and Information Technology, Warsaw University of Technology, Warsaw, Poland Objective: Symptomatic epilepsy, which starts during the first year of life, may be caused by intrauterine and perinatal cytomegalovirus (HCMV) infection, so estimation of seizure semiology and therapy was performed. Methods: Follow-up of 138 infants treated with antiepileptic drugs − AEDs (including 93 with ganciclovir-GCV therapy) due to epilepsy and cytomegalovirus infection. HCMV DNA detection was carried out by PCR methods in CSF, blood leukocytes and urine. Statistical analysis was performed using chi 2 test and ANOVA. Results: Infantile spasms (IS), generalized tonic-clonic (GTCS), partial onset (POS) and polymorphic (PLM) seizures occurred in 109 infants before the age of 6 months. In 32 infants treated with ganciclovir, HCMV DNA was found in CSF, and in 106 only in blood leukocytes and urine. 61 infants were also treated with GCV and 45 infants received AEDs without GCV. Median of observation period was 5 years. 74 infants have cerebral palsy. Patients with GTCS were frequently treated with AEDs monotherapy (p = 0.033). AEDs monotherapy was also possible in infants with epileptic seizures and cytomegalovirus hepatitis (p = 0.006). In patients with IS, tetraparesis was significantly frequent (p = 0.003), but infants with POS usually develop normally (p = 0.002). Patients with tetraparesis received at least two AEDs (p = 0.006) and frequently have SNHL (p = 0.003). In 24 patients (21 treated
Posters with GCV) AEDs withdrawal was possible. AEDs withdrawal was successfully performed without recurrence of epileptic seizures in 11 out of 32 with HCMV neuroinfection, in 10 out of 61 also treated with GCV and only in 3 out of 45 infants without antiviral therapy. AEDs withdrawal was most frequent in patients with epilepsy and HCMV neuroinfection treated with GCV (p = 0.009). Conclusion: GCV therapy is very important for AEDs withdrawal, especially when HCMV neuroinfection is diagnosed early. P115 Absence epilepsy and electroencephalographic focal abnormalities S. Teber1 *, A. Yılmaz1 , G. Deda1 . 1 Pediatric Neurology, Ankara University Faculty Of Medicine, Ankara, Turkey Typical absences are associated with bilateral, synchronous and symmetric spike-and wave discharges at 3 cycles per second. However, the coexistence of focal EEG discharges associated with absence epilepsy has been rarely documented. Video EEG recordings of twelve patients; 7 was childhood absence epilepsy and 5 was juvenile absence epilepsy were evaluated. Eight of them had focal EEG abnormalities but none of them had focal clinical manifestations. A family history of epilepsy was found in seven cases (58%). Neurologic and neuropsychological examinations were normal in all cases. Brain MRI was performed in 7 cases and were normal. The background EEG rhythms were normal in all patients. The interictal EEG recordings revealed left centroparietal spikes in 1 patient, frontocentral spike in one, left temporooccipital spike in one, left frontal spike in one, bilateral frontal spike in four, bilateral frontal multiple spike wave in four, temporooccipital slow wave with left predominance in 2 and bilateral temporooccipital slow wave in two patients. Photoparoxysmal response on the EEG was positive in three children, and closing of the eyes did not activate absences in any of the children. The cause of the existence of focal EEG discharges in cases with CAE has not been explained as yet. Focal and generalized EEG paroxysms may be an expression of common and shared genes in families with generalized epilepsy and families with benign focal epilepsies. From pathophysiological point of view, another issue that could be involved is the theory stating that absence epilepsy might in fact have a focal onset. P116 Adrenocorticotrop hormone therapy in acquired childhood epileptic aphasia 1 L. Szabo´ 1 , J. Nagy1 , R. Kalm *. 1 2nd Department of ´ anchey ´ Paediatrics, Semmelweis University, Budapest
Although Landau Kleffner syndrome, a childhood-acquired epileptic aphasia, is frequently studied both its underlying pathophisiology and optimal therapy have remained unknown. Our study is aimed to investigate the efficacy of ACTH therapy in Landau Kleffner syndrome. We have analysed retrospectively the documentation of 5 children treated by ACTH, who suffered from Landau Kleffner syndrome. We studied the longitudinal changes of the four most characteristic sympthoms and signs of the syndrome: epileptiform EEG, speech and behaviour disorders, seizures together with the ACTH regimes. Besides, we analysed the relation between the starting date of the therapy and its efficacy. Before giving ACTH, epileptiform EEG and speech disorders were observed in all the 5 children, seizures in 4 of them, behaviour disorders in 3 of them. With 2 patients the speech disorder had been persisting for years before. Due to the starting ACTH stosstherapy (20E/day for 1−2 weeks) all the four examined signs disappeared or showed quick softening in all the 5 children in maximum 2 weeks. We adjusted