[儿童结节性硬化症合并癫痫的随访]

Wen Jia-Lun, Liao Jian-Xiang, Chen Li, Hu Yan, Li Bing, Huang Tie-Shuan, Lu Xin-Guo, Wang Xin-Juan

Epilepsy Center, Shenzhen Children's Hospital, Shenzhen, Guangdong 518026, China.

Zhongguo Dang Dai Er Ke Za Zhi. 2009 Dec;11(12):996-8.

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 + or - 2.6 years). The patients' present average age was (7.7 + or - 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 + or - 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.

目的

探讨结节性硬化症（TSC）合并癫痫患儿难治性癫痫的治疗效果及危险因素。

方法

回顾性分析66例TSC患儿的临床资料。

结果

66例TSC患儿中，47例获得随访，随访时间7个月至9.3年（平均4.5±2.6年）。患儿目前平均年龄为（7.7±4.1）岁（中位数8岁）。47例中，19例（40%）有婴儿痉挛症，24例（51%）有强直发作，15例（32%）有部分性发作，3例（6%）有强直-阵挛发作，另外，多灶性发作、失张力发作、不典型失神发作和运动减少性发作各有1例（2%）。使用抗癫痫药物的平均数量为1.9±0.86（中位数1）。47例患者中，12例（26%）仍有癫痫发作，33例（70%）无发作，4%死亡。3例接受手术治疗，术后继续用药。这3例患者在1.5年的随访中无发作。30例7岁以上儿童中，17例（57%）进入普通学校，3例（10%）进入特殊学校，另外10例因智力和语言残疾未上学。非条件logistic回归显示，发病年龄（RR=1.8，95%CI 1.0-3.2，P=0.050）、多种抗癫痫药物治疗（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）和性别（RR=0.016，95%CI 0.0-0.5，P=0.017）是难治性癫痫的危险因素。