Objective To investigate the clinical features and changes of EEG in children with late onset epilepsy spasm.
Methods The clinical data, treatment, follow-up and outcome of 13 patients with late-onset epilepsy spasms were analyzed retrospectively from June 2010 to August 2015 in Bo ai Hospital of Zhong Shan City.Affiliated Southern Medical University
Results Thirteen cases of children were enrolled in the group, including 9 males and 4 females, the onset of age were 1 year 3 months to 5 years 7 months, duration of treatment were 1 year 5 months to 4 years 8months.Seven cases of children had clear cause in 13 patients: 2 cases of viral encephalitis, 3 cases of HIE, 1 case of neonatal sepsis, ARDS, and 1 case of methylmalonic acid hyperchomocysteinemia.Six cases did not clear the cause.Spasm is still the main type of Seizures.Seven cases had seizures with partial origin.the most onset time were awake period and wake up for the time, and coexisted with other types of seizures.EEG in Epileptic seizures period was a broad range of high amplitude slow wave, slow bursts, complex or non-composite low amplitude fast wave, sometimes with the burst after the voltage attenuation of a few seconds, string or isolation occurs.Synchronous bilateral deltoid EMG monitoring showed bilateral or unilateral synchronous EMG 1 ~ 2s Bilateral or unilateral synchronous EMG outbreak1-2s.Intermittent EEG showed multifocal and extensive epileptic discharge, still sharp (spine) slow wave continuous release based.Treatment: All children underwent ACTH or methylprednisolone immunoregulation treatment, 3 cases underwent ketone diet therapy.At the same time choice valproic acid, topiramate, clonazepam, lamotrigine, levarabesilan and other anti-broad-spectrum antiepileptic drugs, according to the history.all children were taken in combination with the way.Prognosis: 13 patients'seizures reduced or controled after the end of the ACTH or methylprednisolone immunotherapy course.followed-up 3 to 12 months, the clinical attack control were failed 3 cases had relatively good prognosis, treated with Ketogenic diet (Lasted for 1 year 3 mothes~2 years 5 mothes), one case of attack control, mental improvement significantly, Another 2 cases, the numbers of episodes were reduced and the level of intelligence were significantly improved.
Conclusion Perinatal factors and acquired brain injury are the most common cause of pathogenesis.Spasm as a major form of attack, and other forms of coexistence.EEG is not typical of high degree of performance.Simultaneous EMG monitoring shows bilateral or unilateral synchronous EMG outbreaks.The treatment of various antiepileptic drugs were ineffective.The vast majority of patients developed refractory epilepsy.Ketogenic diet treatment may be a relatively good choice.
Citation：
LUO Xufeng, ZHANG Sheng, ZHU Jianping, XIE Huiyuan, LIU Nan, ZHOU Tao, FU Simao. Clinical characteristics and electroencephalogram changes of late-onset epileptic spasm in children. Journal of Epilepsy, 2017, 3(5): 389-394. doi: 10.7507/2096-0247.20170060
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Copyright © the editorial department of Journal of Epilepsy of West China Medical Publisher. All rights reserved
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Ishikawa N, Kobayashi Y, Fujii Y, et al. Late-onset epileptic spasms in a patient with 22q13.3 deletion syndrome.Brain & Dev, 2016, 38(1): 109-112.
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吴舒华, 刘晓燕, 张月华, 等.晚发型癫痫性痉挛临床与脑电图特征分析.中国实用儿科杂, 2010, 25(4): 266-270.
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Lei Chen, Min Zhu, Hao Zhou, et al. Clinical study of West syndrome with PS and late-onset epileptic spasms.Epilepsy Research, 2010, 89(1): 82-88.
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Ohtsuka Y, Kobayashi K, Ogino T, et al. Spasms in clusters in epilepsise other than typical west syndrome.Brain & Development, 2001, 23(7): 473-481.
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Auvin S, Lamblin MD, Pandit F, et al. Infantile epileptic encephalopathy with late-onset spasms: report of 19 patients.Epilepsia, 2010, 51(7): 1290-1296.
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刘晓燕.癫痫性痉挛的临床诊治进展.中华儿科杂志, 2010, 48(4): 312-316.
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周渊峰, 龚晓妍, 周水珍, 等.晚发型癫痫性痉挛患儿临床特征及生酮饮食近期疗效分析.中国循证儿科杂志, 2015, 10(4): 287-291.
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- 1. Ronzano N, Valvo G, Ferrari AR, et al. Late-onset epileptic spasms: clinical evidence and outcome in 34 patients.Journal of Child Neurology, 2015, 30(2): 153-159.
- 2. Nordli DR, Korff CM, Goldstein J, et al. Cryptogenic late-onset epileptic spasms or late infantile epileptogenic encephalopathy.Epilepsia, 2007, 48(1): 206-208.
- 3. Ohtsuka Y, Kobayashi K, Ogino T, et al. Spasms in clusters in epilepsies other than typical West syndrome.Brain Dev, 2001, 23(7): 473-481.
- 4. Isermann MM, Ville D, Sonfflet C, et al. Cryptegenic late-onset ikpdc spasms: an overlooked syndrome of early childhood.Epilepsia, 2006, 47(5): 1035-1042.
- 5. Engel J Jr.Report of the ILAE classification core group.Epilepsia, 2006, 47(9): 1558-1568.
- 6. Caumes R, Boespflug-Tanguy O, Villeneuve N, et al. Late onset epileptic spasms is frequent in MECP2 gene duplication: electroclinical features and long-term follow-up of 8 epilepsy patients.European Journal of Paediatric Neurology, 2014, 18(4): 475-481.
- 7. Ishikawa N, Kobayashi Y, Fujii Y, et al. Late-onset epileptic spasms in a patient with 22q13.3 deletion syndrome.Brain & Dev, 2016, 38(1): 109-112.
- 8. 吴舒华, 刘晓燕, 张月华, 等.晚发型癫痫性痉挛临床与脑电图特征分析.中国实用儿科杂, 2010, 25(4): 266-270.
- 9. Lei Chen, Min Zhu, Hao Zhou, et al. Clinical study of West syndrome with PS and late-onset epileptic spasms.Epilepsy Research, 2010, 89(1): 82-88.
- 10. Ohtsuka Y, Kobayashi K, Ogino T, et al. Spasms in clusters in epilepsise other than typical west syndrome.Brain & Development, 2001, 23(7): 473-481.
- 11. Auvin S, Lamblin MD, Pandit F, et al. Infantile epileptic encephalopathy with late-onset spasms: report of 19 patients.Epilepsia, 2010, 51(7): 1290-1296.
- 12. 刘晓燕.癫痫性痉挛的临床诊治进展.中华儿科杂志, 2010, 48(4): 312-316.
- 13. 周渊峰, 龚晓妍, 周水珍, 等.晚发型癫痫性痉挛患儿临床特征及生酮饮食近期疗效分析.中国循证儿科杂志, 2015, 10(4): 287-291.
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