结节性硬化所致婴儿痉挛症脑电图特征及术前定位探讨

目的探讨结节性硬化所致婴儿痉挛症患者的脑电图特征及术前定位。方法回顾性分析已接受手术治疗的31例结节硬化所致婴儿痉挛症脑电图背景构型,与年龄的相关性及痫灶定位情况。结果 1岁以内为典型高幅失律,1～3岁呈现多元化为典型失律、变异失律及正常背景活动,3岁以后以变异失律和正常背景为主导,合计占该年龄段的86%,少数演变成慢棘-慢约占10%,极少数残留为典型失律约占4%。定位局灶性放电（单灶）6例,一侧双灶7例,一侧多灶7例,双侧改变以一侧优势11例。本组随访3个月至6年,平均3.5年。31例患者中20例无发作,7例发作减少90%,3例减少75%,1例无改变。平均智商（IQ）从术前52.6分提高到61.8分。结论结节性硬化所致婴儿痉挛症脑电图背景构型可随年龄的增长由典型失律转归成变异失律和正常节律。EEG放电广泛,以双灶或多灶性改变为主,但可综合背景、发作间期及发作期定位找出痫灶放电优势侧,并结合临床表现、影像学特点等,找出致痫结节并予以手术切除,可获得良好的治疗效果。

Characteristics of electroencephalogram and preoperative location of epileptogenic foci of infantile spasms in tuberous sclerosis

Objective To investigate the characteristics of electroencephalogram （EEG） and location of epileptogenic foci of infantile spasms in tuberous sclerosis （TS）. Methods Characteristics of EEG and its relation to age,and location of epileptogenic foci in 31 TS patients who underwent the surgical treatment were reviewed retrospectively. Results Typical hypsarrhythmia was shown in EEG of patients within one-year-old. Typical hypsarrhythmia,variant hypsarrhythmia and normal background were shown in patients of 1 year to 3 years old. In patients over 3 years,86% EEG presented as variant hypsarrhythmia and normal background,10% transformed into slow-spike wave and only 4% presented typical hypsarrhythmia. One epileptogenic focus was indicated in 6 patients,two epileptogenic foci （unilateral） in 7 patients,multifoci （unilateral） in 7 patients,and bilateral changes with unilateral predominance in 11 patients. All patients were followed-up from 3 months to 6 years （average 3.5 years） after surgery. Among 31 patients,20 were seizure free,7 patients had rare seizure,3 patients had a decrease in seizure frequency and 1 patient had no remarkable change. Intelligence quotient （IQ） score of all patients was improved from 52.6 to 61.8. Conclusion EEG of TS patients with infantile spasms can transform from typical hypsarrhythmia to variant hypsarrhythmia or normal background with age advancing. General discharge is shown in EEG with two foci or multifoci changes. However,the unilateral predominance of epileptiform waves can be located by analysis of background,interictal and ictal EEG. which contributes to the favorable surgical outcome after operation,in combination with clinical manifestations and imaging data.