小儿结节性硬化症合并癫癎的随访研究

摘要
图/表
参考文献(15)
相关文章 (15)
点击分布统计
下载分布统计

全文: PDF (1011 KB)
输出: BibTeX | EndNote (RIS) 背景资料

摘要目的：调查结节性硬化症（TSC）合并癫癎的治疗转归及癫癎反复发作的高危因素。方法：回顾性分析我院66例TSC患儿的资料。结果：66例TSC患儿中，随访47例，随访时间为7个月至9.3年，平均4.5±2.6年。患儿现在年龄7.7±4.1岁，癫癎发作类型：40%有婴儿痉挛症，51%有强直性发作，32%有部分性发作，强直-阵挛性发作占6%，多灶性发作、失张力发作、不典型失神发作、抑制性运动发作各占2%。目前使用抗癫癎药1.9±0.86种，中位数1种。26%仍然癫癎发作，70%无发作，4%死亡。手术治疗3例，均在继续用药，随访1.5年以上，无发作。应用非条件logistic回归方法分析，发现起病年龄（RR=1.8, 95% CI 1.0～3.2, P=0.050）、抗癫癎药的种类(RR=4.8, 95% CI 1.2～18.6, P=0.024)、强直发作(RR=0.003, 95% CI 0.0～0.2, P=0.04)、性别(RR=0.016, 95% CI 0.0～0.5, P=0.017）是癫癎反复发作的高危因素。30例7岁以上儿童57%例可以上普通学校， 10%上特殊学校； 33%因为智力、言语发育落后不能上学。结论：对TSC合并癫癎进行抗癫癎治疗可以达到大部分无发作。癫癎发作起病年龄早、强直发作、需要多种抗癫癎药是癫癎反复发作的高危因素。［中国当代儿科杂志，2009，11（12）：996-998］

	服务

	把本文推荐给朋友
	加入我的书架
	加入引用管理器
	E-mail Alert
	RSS
	作者相关文章
	文家伦
	廖建湘
	陈黎
	胡雁
	李冰
	黄铁栓
	路新国
	王鑫娟

关键词 ：结节性硬化症, 癫癎, 随访, 儿童

Abstract：OBJECTIVE: To investigate the treatment outcome and risk factors for intractable seizures in children with tuberous sclerosis complex（TSC）complicated by epilepsy. METHODS: The medical data of 66 cases of TSC were retrospectively studied. RESULTS: Of the 66 children with TSC, 47 cases were available for follow-up. The follow-up period ranged from 7 months to 9.3 years (average 4.5±2.6 years). The patients′ present average age was (7.7±4.1) years (median 8 years). Among the 47 cases, 19 (40%) had infantile spasms, 24 (51%) had tonic seizures, 15 (32%) had partial seizures, and 3 (6%) had tonic-clonic seizures, and additionally, multifocal seizures, atonic seizures, atypical absence seizures and hypomotor seizures each appeared in 1 case (2%) respectively. The average number of antiepileptic drugs used was 1.9±0.86 (median 1). Among the 47 patients, 12 (26%) still had epileptic seizures and 33 (70%）were seizure-free, and 4% were dead. Three cases underwent surgery and continued to receive medication after surgery. The three patients were seizurefree in a 1.5 years follow-up. Among the 30 children over 7 years old, 17 cases (57%) were enrolled in ordinary schools, 3 cases (10%) in special schools and the other 10 cases were off-school for disabilities of intelligence and speech. The non conditional logistic regression showed that the age of onset (RR=1.8, 95% CI 1.0- 3.2, P=0.050), administration of multiple antiepileptic drugs (RR=4.8, 95% CI 1.2-18.6, P=0.024), tonic seizures (RR=0.003, 95% CI 0.0- 0.2, P=0.04) and sex (RR=0.016, 95% CI 0.0-0.5, P=0.017) were risk factors for intractable seizures. CONCLUSIONS: The majority (70%) of children with TSC complicated by epilepsy can be seizure-free with suitable treatment. The risk factors of poor outcome in seizure control may involve in the early onset age, tonic seizures and the administration for multiple anti-epileptic drugs.［Chin J Contemp Pediatr, 2009, 11 (12):996-998］

Key words： Tuberous sclerosis complex Epilepsy Follow-up Child

PACS:

R748

引用本文:

文家伦,廖建湘,陈黎等. 小儿结节性硬化症合并癫癎的随访研究[J]. 中国当代儿科杂志, 2009, 11(12): 996-998.

WEN Jia-Lun,LIAO Jian-Xiang,CHEN Li et al. Follow-up of tuberous sclerosis complex complicated by epilepsy in children[J]. CJCP, 2009, 11(12): 996-998.

链接本文:

http://www.zgddek.com/CN/ 或 http://www.zgddek.com/CN/Y2009/V11/I12/996

	［1］廖建湘,陈黎,李冰,黄铁栓,胡雁. 结节性硬化症［J］.中国实用儿科杂志，2002, 17(10):631-634.
	［2］廖建湘,陈黎,李冰,黄铁栓,肖宇寒.儿童结节性硬化症的临床诊断探讨［J］.中国实用儿科杂志, 2002, 17(10):599-601.
	［3］Roach ES, Gomez MR, Northrup H. Tuberous sclerosis complex consensus conference: revised clinical diagnostic criteria［J］. J Child Neurol, 1998, 13(12):624-628.
	［4］Kallen K. Hypomotor seizures in infants and children［J］. Epilepsia, 2002, 43(8):882-888.
	［5］Roach ES, Sparagana SP. Diagnosis of tuberous sclerosis complex［J］.J Child Neurol, 2004, 19(9):643-649.
	［6］Schwartz RA, Fernández G, Kotulska K, Jó'zwiak S. Tuberous sclerosis complex: advances in diagnosis, genetics, and management［J］.J Am Acad Dermatol, 2007, 57(2):189-202.
	［7］Curatolo P, Bombardieri R, Cerminara C.Current management for epilepsy in tuberous sclerosis complex［J］.Curr Opin Neurol, 2006, 19(2):119-123.
	［8］Kwan P, Brodie MJ. Early identification of refractory epilepsy［J］. N Engl J Med, 2000, 342(5):314-319.
	［9］Jansen FE, van Huffelen AC, Algra A, van Nieuwenhuizen O. Epilepsy surgery in tuberous sclerosis: a systematic review［J］.Epilepsia, 2007, 48(8):1477-1484.
	［10］Madhavan D, Schaffer S, Yankovsky A, Arzimanoglou A, Renaldo F, Zaroff CM, et al. Surgical outcome in tuberous sclerosis complex: a multicenter survey［J］.Epilepsia, 2007, 48(8):1625-1628.
	［11］Jansen FE, Van Huffelen AC, Van Rijen PC, Leijten FS, Jennekens-Schinkel A, Gosselaar P, et al. Epilepsy surgery in tuberous sclerosis: the Dutch experience［J］.Seizure, 2007, 16(5):445-453.
	［12］Lachhwani DK, Pestana E, Gupta A, Kotagal P, Bingaman W, Wyllie E. Identification of candidates for epilepsy surgery in patients with tuberous sclerosis［J］.Neurology, 2005, 64(9):1651-1654.
	［13］左启华. 小儿神经系统疾病: 结节性硬化症［M］.第2版.北京:人民卫生出版, 2002, 851-854.
	［14］Chandra PS, Salamon N, Nguyen ST, Chang JW, Huynh MN, Cepeda C, et al. Infantile spasm-associated microencephaly in tuberous sclerosis complex and cortical dysplasia［J］.Neurology, 2007, 68(6):438-445.
	［15］Zaroff CM, Barr WB, Carlson C, LaJoie J, Madhavan D, Miles DK, et al.Mental retardation and relation to seizure and tuber burden in tuberous sclerosis complex［J］. Seizure, 2006, 15(7):558-562.