促肾上腺皮质激素治疗婴儿痉挛症的近期疗效与远期预后及其影响因素的研究

发布时间：2019-01-19 11:37

【摘要】：目的：分析促肾上腺皮质激素(ACTH)治疗婴儿痉挛症的近期疗效和远期预后及其影响因素，为提高婴儿痉挛症治疗效果提供参考依据。方法：根据诊断标准、纳入标准，排除标准，建立数据库，收集病例，随访，分析课题组1999-2010年间在深圳市儿童医院确诊并住院治疗的婴儿痉挛症患儿，分首选ACTH组和非首选ACTH组，比较近期(8周内)无发作率，药物选择次序对癫痫发作的影响；分析ACTH治疗后近期临床无发作疗效与视频脑电图改变相关性；分析非首选ACTH组近期疗效的影响因素；分析远期预后及其影响因素。结果：最后随访时间2013年12月，病例共113例。1.首选ACTH组33例，非首选ACTH组80例，首选组取得近期无发作疗效21例，占63.6%，非首选组近期无发作疗效33例，占41.3%。二组X2=4.69，P0.05，结果有统计学差异。2.113例婴儿痉挛症患儿均选择ACTH治疗，治疗前脑电图均有高峰节律紊乱，近期发作控制组54例，高峰节律紊乱消失38例，占70.4%；发作未控制组59例，高峰节律紊乱消失23例，占39%，二组X2=11.18，P0.05，结果有统计学意义。3.非首选ACTH组80例，近期痉挛发作控制33例，占41.3%，疗效与起病年龄和初诊年龄有关(起病年龄6月、初诊年龄6月的患儿抗癫痫药失败后再用ACTH效果更好)，与性别、病程、病因无关；4.随访末期复发情况：首选ACTH组无发作21例，7例复发，复发率为33.3%；非首选ACTH组无发作33例，14例复发，复发率为42.4%，2组X2=0.78，P0.05，结果无统计学意义。远期发作情况：ACTH治疗后完全控制无复发33例，ACTH联合其它抗癫痫药均未控制55例(其中7例死亡，，12例发作形式改变，包括肌阵挛发作、强直发作、典型或非典型失神发作、局灶性发作等)，ACTH治疗后未控制但其它抗癫痫药控制15例。5.远期智力运动发育好转或正常有49例，未见好转或倒退有57例。起病年龄6月、初诊年龄6月、ACTH近、远期发作完全控制、病因为隐源性的患儿智力运动发育更好，智力运动发育预后与性别、ACTH近期高峰节律紊乱消失情况无关。结论：1.婴儿痉挛症首选ACTH治疗可以获得较高近期无发作率(63.6%)，对抗癫痫药物失败后的患儿，ACTH治疗获得无发作的机会较低(41.3%)，但仍然值得尝试；2.视频脑电图监测是判断ACTH治疗后近期疗效的重要指标，临床“无发作”情况不能完全反映视频脑电图高峰节律紊乱消失情况；3.非首选ACTH近期疗效：起病年龄6月、初诊年龄6月的患儿抗癫痫药失败后再用ACTH效果更好；4.远期复发率与是否首选ACTH治疗无关；5.远期预后：起病年龄6月、初诊年龄6月、ACTH近期及远期发作完全控制、病因为隐源性的患儿智力运动发育更好。
[Abstract]:Objective: to analyze the short term curative effect, long term prognosis and influencing factors of adrenocorticotropic hormone (ACTH) in the treatment of infantile spasticity, so as to provide reference for improving the therapeutic effect of infantile spasm. Methods: according to diagnostic criteria, inclusion criteria, exclusion criteria, establishment of database, collection of cases, follow-up, analysis of children with infantile spasticity diagnosed and hospitalized in Shenzhen Children's Hospital from 1999 to 2010. The patients were divided into the first choice ACTH group and the non first choice ACTH group. There was no seizure rate in the near future (8 weeks) and the influence of drug selection order on epileptic seizure. To analyze the correlation between the effect of clinical non-seizure after ACTH treatment and the changes of video-EEG; to analyze the influencing factors of short-term curative effect of non-first-choice ACTH group; to analyze the long-term prognosis and its influencing factors. Results: in December 2013, 113 cases were followed up. 1. 1. 33 cases of the first choice ACTH group, 80 cases of the non-preferred ACTH group, 21 cases (63.6%) of the first choice group had no attack in the short term, 33 cases (41.3%) of the non-first choice group had no recent attack effect. 2.113 cases of infantile spasm were treated with ACTH. The electroencephalogram (EEG) had peak rhythm disorder before treatment. In the control group, 54 cases had peak rhythm disorder and 38 cases had disappeared peak rhythm disorder. 70.4%; In the uncontrolled group, the peak rhythm disorder disappeared in 23 cases (39%), and in the second group, X2 + 11.18 P 0.05, the results were statistically significant (3. 3%, P 0. 05, P 0. 05). There were 80 cases of non-first-choice ACTH group, 33 cases (41.3%) had recent spasm control. The curative effect was related to the age of onset and the age of first visit (the age of onset was 6 months, the effect of ACTH was better after the failure of antiepileptic drug in the first six months), and the effect of ACTH was better than that of sex. The course of disease was not related to the etiology. 4. At the end of follow-up, 21 cases had no attack, 7 cases recurred with a recurrence rate of 33.3% in the first choice ACTH group, and 33 cases (14 cases) recurred in the non-preferred ACTH group, and the recurrence rate was 42.4% (P 0.05). Long-term seizure: after ACTH treatment, 33 cases were completely controlled without recurrence, 55 cases were not controlled by ACTH combined with other antiepileptic drugs (7 cases died, 12 cases had changes of seizure form, including myoclonic seizure, tonic attack, typical or atypical aphasia, etc. 15 cases were controlled by other antiepileptic drugs, but 15 cases were not controlled after), ACTH treatment. 5. 5%. There were 49 cases with improved or normal intellectual motor development, 57 cases without improvement or regression. The onset age was 6 months, the first diagnosis age was 6 months, the ACTH was near, the long term attack was completely controlled, the cause of the disease was the better intellectual motor development of the children with cryptic origin, the prognosis of the intellectual motor development was not related to the sex, and the disordered peak rhythm of ACTH disappeared in the near future. Conclusion: 1. The first choice of ACTH treatment for infantile spasm can obtain a higher rate of short term non-seizure (63.6%), but the chance of ACTH treatment is lower (41.3%) after the failure of antiepileptic drugs, but it is still worth trying; 2. Video EEG monitoring is an important index to judge the short-term curative effect after ACTH treatment. Clinical "no attack" can not completely reflect the disappearance of peak rhythm disorder of video EEG. 3. The short term efficacy of non first choice ACTH: the onset of the disease in 6 months, the first six months of age after the failure of antiepileptic drugs after the use of ACTH is better; 4. The long term recurrence rate was not related to the first choice of ACTH treatment. Long term prognosis: the onset age was 6 months, the first visit age was 6 months, the short and long term attack of ACTH was completely controlled, and the cause of the disease was the better intellectual motor development of the children whose etiology was cryptogenic.
【学位授予单位】：遵义医学院
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R742.1

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