婴儿痉挛临床诊治与预后127例分析  

The clinical features and prognosis of infantile spasms:analysis of 127 cases

作　　者：王艺[1] 陈雯[1] 邱鹏玲[1] 孙道开[1] 

机构地区：[1]复旦大学附属儿科医院癫癎中心,上海200032

出　　处：《中国实用儿科杂志》2006年第3期191-193,共3页Chinese Journal of Practical Pediatrics

摘　　要：目的通过对127例婴儿痉挛患儿的临床诊治和随访结果资料分析,了解婴儿痉挛的诊断治疗与预后之间的关系,为婴儿痉挛的临床诊治和预后判断提供依据。方法采用回顾性资料分析和前瞻性队列研究,对1998年1月至2002年12月在复旦大学儿科医院收治的婴儿痉挛患儿进行分析和随访,对影响其预后的相关因素进行分析。结果共有127例婴儿痉挛患儿进入研究。起病年龄为(6·1±3·1)个月,患儿随访时间(38±8)个月;72·4%为症状性,宫内发育迟缓、先天脑发育畸形、出生时窒息为主要病因,脑发育不全、智能发育迟缓多见;27·6%为隐原性或原发性。脑电图表现为高峰节律紊乱、弥漫或局灶性棘慢波发放。促肾上腺皮质激素(ACTH)、丙戊酸钠、氯硝基安定、硝基安定、苯巴比妥、托吡酯、拉莫三嗪为常用的治疗药物。在症状性婴儿痉挛中,76·1%预后不良,原发性或隐原性婴儿痉挛中,有60·8%的病例发作得到控制;有明确病因、起病年龄<3个月、起病前发育落后、EEG背景活动变慢或有局灶性棘慢波发放,可能提示婴儿痉挛预后不良。结论婴儿痉挛多为症状性,有明确病因、起病年龄<3个月、起病前发育异常、EEG背景活动变慢或有局灶性棘慢波发放,与婴儿痉挛预后不良有关。Objective To investigate the relationship between the clinical features and the prognosis of infantile spasms. Methods This is a cohort study. From Jun. 1998 to Dec. 2002,the medical records of patients with IS in children＇s Hospital of Fudan University were analyzed. The demographic data,seizure details,developmental status, neurologic findings at presentation and follow-up, and electroencephalographic （ EEG ）, neuroimaging, metabolic, hematologic, and chemistry test were reviewed. And all the patients were followed up for more than three years. The predictors of prognosis of infantile spasms were analyzed. Results A total of 127 patients who were diagnosed as infantile spasms at the Children s Hospital of Fudan University between January 1999 and December 2002 were involved in the study. All the patients were treated with anti-epileptic drugs （ AEDs） and had been followed up for more than 3 years. The mean age of seizures onset was 6. 12 ±3. 1 months （ range =0. 8 - 12）. The mean duration of follow-up from onset of seizures was 38 ±8 months （ range = 36 - 54 ）. The underlying causes were determined in 72. 4% of patients. There were 27.6% of patients with idiopathic or cryptogenic spasms. The EEG appearances were hypsarrhythmia, general or focal slow spike and waves or burst-suppression. ACTH,valproate, clonazepam, nitrazepam, phenobarbital, topiramate and lamortrigine were used in the therapy of this group. 76. 1% of patients with symptomatic spasms were refractory to medical therapy and with severe subsequent retardation. 60. 8% of children with idiopathic spasms without seizures with AEDs therapy. The prognosis depended on the etiology,age of onset 〈3 month,development delay before onset and focal or muhifocal discharges on interictal EEG. Conclusion In this study,the risk factors that correlate with the prognosis of infantile spasms may include the etiology,age of onset less than 3 month,development delay before onset and focal or muhifocal discharges on interictal EEG.

关 键 词：婴儿痉挛 病因 诊断 抗癫痫药 预后 危险因素 

分 类 号：R72[医药卫生—儿科]