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Electroencephalographic (EEG) RECORDING during sleep induced by melatonin
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e u r o p e a n j o u r n a l o f p a e d i a t r i c n e u r o l o g y 2 1 ( 2 0 1 7 ) e 9 6 ee 1 0 8
P2-7 Rasmussen encephalitis: Hard diagnosis, harder therapy Kova ri, B. Rosdy, K. Kolla r, J. Mo ser, M. Mella r, E. cs, J. Szamosu´jva G. Kiss, G. Rudas, Gy. Varallyay. Heim Pal Children's Hospital, Dept. of Neurology, Budapest, Hungary Objective: Rasmussen encephalitis (RE) is a rare autoimmune chronic inflammatory neurodegenerative disease affecting single cerebral hemisphere, causing progressive neurological deterioration and intractable seizures. Antiepileptic and immunodulatory treatment can be tried, but surgery (hemispherotomy) if applicable is the treatment of choice. Case report: A 9-year-old boy suffered from fever provoked repetitive grand mal seizures, which progressed to epilepsia partialis continua (EPC) with right sided hemiparesis and aphasia. Antiepileptic treatment was not effective. The longitudinally followed brain MRI-s showed hyperintensity on T2/FLAIR sequences in cortical and subcortical regions of the left temporo-parieto-central region, leading to atrophy, suggesting diagnosis of RE. PET-CT presented focal hypometabolic activity in these regions as well. Because eloquent cortex was affected, surgery in that age was not anymore a solution. Beside antiepileptics we started immunomodulatory treatment with pulse steroids and plasmapheresis. Because of septic complications we had to switch to intravenous immunoglobulin (IVIG) combined with long-term oral steroids. IVIG was administered, every 4-6 weeks. Seizure freedom was achieved and hemiparesis disappeared. Oral steroids could be tapered. Because of the cognitive side effects of antiepileptic tritherapy we tried to taper one drug, but EPC reappeared. After 1 year of IVIG treatment, it has failed. Switching to Rituximab was considered, but the percentage of B lymphocytes subpopulation in his cerebrospinal fluid was too low (0, 3 %). Surgery was reconsidered, but language fMRI lateralised only to left, so it was not offered and the family refused it as well. Conclusion: Immunomodulatory treatment combined with antiepileptic drugs should be considered in therapy of RE, but it is just transiently effective. Presurgical evaluation must be performed. If surgery is applicable, the timing of it is a critical point in the treatment decision. Postsurgical neurological deficits are frequently equivalent to those inevitably resulting from RE.
http://dx.doi.org/10.1016/j.ejpn.2017.04.735 P2-8 Electroencephalographic (EEG) RECORDING during sleep induced by melatonin Prpic Igor, Radic Nisevic Jelena, Begic Jelena, Kapovic Radojka, Kolic Ivana. KBC Rijeka, Department of Pediatrics, Division of Pediatric Neurology and Pediatric Psychiatry, Reference Center of the Ministry of Health of the Republic of Croatian for Epilepsy and Convulsive Diseases of Developmental Age, Rijeka, Croatia Objective: Preparation and implementation of electroencephalographic (EEG) recording during sleep is very demanding for the child, parents/caregivers and medical staff. Melatonin, a natural hormone that regulates the circadian rhythm, can be used to facilitate falling asleep and sleeping. It does not affect the EEG recording. The aim of this study was to evaluate the effectiveness of melatonin in order to facilitate rapid falling asleep during the EEG recording. Methods: At the Cabinet of Neurophysiology of the Institute for Child Neurology and Pediatric Psychiatry Department of Pediatrics, University Hospital Rijeka we applied melatonin therapy for the purpose of ease of falling asleep during the EEG
recording. The indications for the use of melatonin were unsuccessful previous attempt of sleep EEG registration or by physician recommendation. We recorded the time required for falling asleep. Results: Of a total of 315 recording sleep EEG, melatonin was applied in 57 children in the study period. The dose of melatonin ranged from 1 mg to 3 mg, depending on the age and weight of the child, and was applied 30 minutes before the scheduled EEG recording. The EEG recording has been successfully implemented in one go in all children. Of the total number of patients in whom melatonin was applied, only two 3 children failed to fall asleep. Side effects of treatment with melatonin have not been reported. Conclusion: The application of melatonin for the purpose of falling asleep during EEG monitoring has significantly reduced time required for falling asleep. The use of melatonin made the diagnostic search less demanding and stressful for both the child and the parent/guardian. The use of melatonin facilitated the planning of daily EEG registrations, as well as reduced waiting lists and financial savings.
http://dx.doi.org/10.1016/j.ejpn.2017.04.736 P2-9 Christianson syndrome: An underestimated cause of electrical status epilepticus in sleep? M.-L. Mathieu, J. de Bellescize, M. Till, A. Labalme, N. Chatron, D. Sanlaville, K. Ostrowsky-Coste, V. Des Portes, G. Lesca. Department of Paediatric Neurology, Lyon University Hospitals, Claude Bernard Lyon I University, Lyon, France Objective: Christianson syndrome (CS) is a syndromic form of Xlinked severe ID associated to progressive microcephaly, ataxia, autistic behaviour, near absent speech and epilepsy in 90% of cases. The natural history of seizures and EEG abnormalities is insufficiently known. We report two additional families including patients with electrical status epilepticus in sleep (ESES) previously described in only two patients. Methods: We describe two unrelated families with CS caused by SLC9A6 mutations (one frameshift and one partial deletion), each including two boys. One family has been followed for over 20 years, providing insights into the natural course of epilepsy in this syndrome. Results: Onset of epilepsy occurred within the first two years of life in all patients. Seizures were of various types. First EEG recordings showed abnormal fast-background activity with poor antero-posterior organization as reported in the literature. In the two boys with a 20 year follow-up, epilepsy was drug-resistant during childhood, then became less active in early adolescence. EEG background rhythm slowed over time to 4e5Hz after the age of 8 years. One patient from the first family fulfilled the electrical criteria of ESES, between 6 and 8 years of age, while sleep-EEG had not been performed in his brother during this range of age. ESES was diagnosed in the older brother from the second family since the age of 4 years and 10 months. The four boys presented severe ID, autistic features with agitation limiting frequent sleep EEG recordings. Conclusion: Our observations and previous reports, suggest that ESES might be an underestimated feature in CS and may participate to the psychomotor degradation of the patients. Interestingly, SLC9A6 mutations underlie dysfunction in the sodiumhydrogen exchanger NHE6 implicated in plasticity of glutamatergic synapses. Sleep EEGs should be performed in these infants between 4 and 8 years of age.
http://dx.doi.org/10.1016/j.ejpn.2017.04.737
e u r o p e a n j o u r n a l o f p a e d i a t r i c n e u r o l o g y 2 1 ( 2 0 1 7 ) e 9 6 ee 1 0 8
P2-7 Rasmussen encephalitis: Hard diagnosis, harder therapy Kova ri, B. Rosdy, K. Kolla r, J. Mo ser, M. Mella r, E. cs, J. Szamosu´jva G. Kiss, G. Rudas, Gy. Varallyay. Heim Pal Children's Hospital, Dept. of Neurology, Budapest, Hungary Objective: Rasmussen encephalitis (RE) is a rare autoimmune chronic inflammatory neurodegenerative disease affecting single cerebral hemisphere, causing progressive neurological deterioration and intractable seizures. Antiepileptic and immunodulatory treatment can be tried, but surgery (hemispherotomy) if applicable is the treatment of choice. Case report: A 9-year-old boy suffered from fever provoked repetitive grand mal seizures, which progressed to epilepsia partialis continua (EPC) with right sided hemiparesis and aphasia. Antiepileptic treatment was not effective. The longitudinally followed brain MRI-s showed hyperintensity on T2/FLAIR sequences in cortical and subcortical regions of the left temporo-parieto-central region, leading to atrophy, suggesting diagnosis of RE. PET-CT presented focal hypometabolic activity in these regions as well. Because eloquent cortex was affected, surgery in that age was not anymore a solution. Beside antiepileptics we started immunomodulatory treatment with pulse steroids and plasmapheresis. Because of septic complications we had to switch to intravenous immunoglobulin (IVIG) combined with long-term oral steroids. IVIG was administered, every 4-6 weeks. Seizure freedom was achieved and hemiparesis disappeared. Oral steroids could be tapered. Because of the cognitive side effects of antiepileptic tritherapy we tried to taper one drug, but EPC reappeared. After 1 year of IVIG treatment, it has failed. Switching to Rituximab was considered, but the percentage of B lymphocytes subpopulation in his cerebrospinal fluid was too low (0, 3 %). Surgery was reconsidered, but language fMRI lateralised only to left, so it was not offered and the family refused it as well. Conclusion: Immunomodulatory treatment combined with antiepileptic drugs should be considered in therapy of RE, but it is just transiently effective. Presurgical evaluation must be performed. If surgery is applicable, the timing of it is a critical point in the treatment decision. Postsurgical neurological deficits are frequently equivalent to those inevitably resulting from RE.
http://dx.doi.org/10.1016/j.ejpn.2017.04.735 P2-8 Electroencephalographic (EEG) RECORDING during sleep induced by melatonin Prpic Igor, Radic Nisevic Jelena, Begic Jelena, Kapovic Radojka, Kolic Ivana. KBC Rijeka, Department of Pediatrics, Division of Pediatric Neurology and Pediatric Psychiatry, Reference Center of the Ministry of Health of the Republic of Croatian for Epilepsy and Convulsive Diseases of Developmental Age, Rijeka, Croatia Objective: Preparation and implementation of electroencephalographic (EEG) recording during sleep is very demanding for the child, parents/caregivers and medical staff. Melatonin, a natural hormone that regulates the circadian rhythm, can be used to facilitate falling asleep and sleeping. It does not affect the EEG recording. The aim of this study was to evaluate the effectiveness of melatonin in order to facilitate rapid falling asleep during the EEG recording. Methods: At the Cabinet of Neurophysiology of the Institute for Child Neurology and Pediatric Psychiatry Department of Pediatrics, University Hospital Rijeka we applied melatonin therapy for the purpose of ease of falling asleep during the EEG
recording. The indications for the use of melatonin were unsuccessful previous attempt of sleep EEG registration or by physician recommendation. We recorded the time required for falling asleep. Results: Of a total of 315 recording sleep EEG, melatonin was applied in 57 children in the study period. The dose of melatonin ranged from 1 mg to 3 mg, depending on the age and weight of the child, and was applied 30 minutes before the scheduled EEG recording. The EEG recording has been successfully implemented in one go in all children. Of the total number of patients in whom melatonin was applied, only two 3 children failed to fall asleep. Side effects of treatment with melatonin have not been reported. Conclusion: The application of melatonin for the purpose of falling asleep during EEG monitoring has significantly reduced time required for falling asleep. The use of melatonin made the diagnostic search less demanding and stressful for both the child and the parent/guardian. The use of melatonin facilitated the planning of daily EEG registrations, as well as reduced waiting lists and financial savings.
http://dx.doi.org/10.1016/j.ejpn.2017.04.736 P2-9 Christianson syndrome: An underestimated cause of electrical status epilepticus in sleep? M.-L. Mathieu, J. de Bellescize, M. Till, A. Labalme, N. Chatron, D. Sanlaville, K. Ostrowsky-Coste, V. Des Portes, G. Lesca. Department of Paediatric Neurology, Lyon University Hospitals, Claude Bernard Lyon I University, Lyon, France Objective: Christianson syndrome (CS) is a syndromic form of Xlinked severe ID associated to progressive microcephaly, ataxia, autistic behaviour, near absent speech and epilepsy in 90% of cases. The natural history of seizures and EEG abnormalities is insufficiently known. We report two additional families including patients with electrical status epilepticus in sleep (ESES) previously described in only two patients. Methods: We describe two unrelated families with CS caused by SLC9A6 mutations (one frameshift and one partial deletion), each including two boys. One family has been followed for over 20 years, providing insights into the natural course of epilepsy in this syndrome. Results: Onset of epilepsy occurred within the first two years of life in all patients. Seizures were of various types. First EEG recordings showed abnormal fast-background activity with poor antero-posterior organization as reported in the literature. In the two boys with a 20 year follow-up, epilepsy was drug-resistant during childhood, then became less active in early adolescence. EEG background rhythm slowed over time to 4e5Hz after the age of 8 years. One patient from the first family fulfilled the electrical criteria of ESES, between 6 and 8 years of age, while sleep-EEG had not been performed in his brother during this range of age. ESES was diagnosed in the older brother from the second family since the age of 4 years and 10 months. The four boys presented severe ID, autistic features with agitation limiting frequent sleep EEG recordings. Conclusion: Our observations and previous reports, suggest that ESES might be an underestimated feature in CS and may participate to the psychomotor degradation of the patients. Interestingly, SLC9A6 mutations underlie dysfunction in the sodiumhydrogen exchanger NHE6 implicated in plasticity of glutamatergic synapses. Sleep EEGs should be performed in these infants between 4 and 8 years of age.
http://dx.doi.org/10.1016/j.ejpn.2017.04.737