Jonas R, Nguyen S, Hu B, Asarnow R F, LoPresti C, Curtiss S, de Bode S, Yudovin S, Shields W D, Vinters H V, Mathern G W
Division of Pediatric Neurology, David Geffen School of Medicine, University of California, Los Angeles, USA.
Neurology. 2004 May 25;62(10):1712-21. doi: 10.1212/01.wnl.0000127109.14569.c3.
To compare hemispherectomy patients with different pathologic substrates for hospital course, seizure, developmental, language, and motor outcomes.
The authors compared hemispherectomy patients (n = 115) with hemimegalencephaly (HME; n = 16), hemispheric cortical dysplasia (hemi CD; n = 39), Rasmussen encephalitis (RE; n = 21), infarct/ischemia (n = 27), and other/miscellaneous (n = 12) for differences in operative management, postsurgery seizure control, and antiepilepsy drug (AED) usage. In addition, Vineland Adaptive Behavior Scale (VABS) developmental quotients (DQ), language, and motor assessments were performed pre- or postsurgery, or both.
Surgically, HME patients had the greatest perioperative blood loss, and the longest surgery time. Fewer HME patients were seizure free or not taking AEDs 1 to 5 years postsurgery, but the differences between pathologic groups were not significant. Postsurgery, 66% of HME patients had little or no language and worse motor scores in the paretic limbs. By contrast, 40 to 50% of hemi CD children showed near normal language and motor assessments, similar to RE and infarct/ischemia cases. VABS DQ scores showed +5 points or more improvement postsurgery in 57% of patients, and hemi CD (+12.7) and HME (+9.1) children showed the most progress compared with RE (+4.6) and infarct/ischemia (-0.6) cases. Postsurgery VABS DQ scores correlated with seizure duration, seizure control, and presurgery DQ scores.
The pathologic substrate predicted pre- and postsurgery differences in outcomes, with hemimegalencephaly (but not hemispheric cortical dysplasia) patients doing worse in several domains. Furthermore, shorter seizure durations, seizure control, and greater presurgery developmental quotients predicted better postsurgery developmental quotients in all patients, irrespective of pathology.
比较不同病理基础的大脑半球切除术患者的住院过程、癫痫发作情况、发育、语言和运动功能转归。
作者比较了大脑半球切除术患者(n = 115),其中包括巨脑回畸形(HME;n = 16)、半球皮质发育异常(半侧CD;n = 39)、拉斯姆森脑炎(RE;n = 21)、梗死/缺血(n = 27)以及其他/杂类(n = 12),比较其手术管理、术后癫痫控制及抗癫痫药物(AED)使用情况的差异。此外,在术前或术后,或两者均进行了文兰适应行为量表(VABS)发育商数(DQ)、语言和运动评估。
手术方面,HME患者围手术期失血量最大,手术时间最长。术后1至5年,癫痫发作缓解或未服用AED的HME患者较少,但病理组之间的差异不显著。术后,66%的HME患者语言能力差或几乎没有语言能力,患侧肢体运动评分更差。相比之下,40%至50%的半侧CD儿童语言和运动评估接近正常,与RE和梗死/缺血病例相似。VABS DQ评分显示,57%的患者术后提高了5分或更多,与RE(+4.6)和梗死/缺血(-0.6)病例相比,半侧CD(+12.7)和HME(+9.1)儿童进步最大。术后VABS DQ评分与癫痫发作持续时间、癫痫控制及术前DQ评分相关。
病理基础可预测术前和术后转归的差异,巨脑回畸形(而非半球皮质发育异常)患者在多个领域表现较差。此外,无论病理情况如何,癫痫发作持续时间较短、癫痫得到控制以及术前发育商数较高预示着所有患者术后发育商数较好。