Cerullo A, Marini C, Carcangiu R, Baruzzi A, Tinuper P
Neurological Institute, University of Bologna, Italy.
Epileptic Disord. 1999 Mar;1(1):27-33.
The International Classification of Epileptic Syndromes considers epileptic spasms to be typical seizures of West syndrome. Literature reports show that spasms are present in epileptic syndromes other than West syndrome but there are few data on their characteristics in adults. We describe ictal, clinical and video-polygraphic findings in three patients (aged 21, 32 and 57 years) with epileptic spasms and with diffuse (case 2), focal right fronto-parietal (case 1) and bi-opercular (case 3) pachygyria. Spasms had been present since the ages of 1 month, 11 and 27 years respectively. Only one patient is mentally retarded. Two of our patients (cases 2 and 3) have partial seizures. Ictal polygraphic studies showed a positive, diffuse, high amplitude slow wave activity during spasms, with superimposed fast activity, followed by a diffuse flattening in all cases with a typical muscle pattern. Epileptic spasms, as typically described in West syndrome, can maintain the same semeiological and electroencephalographic features during adulthood in certain patients with cortical dysplasia.
《国际癫痫综合征分类》将癫痫性痉挛视为韦斯特综合征的典型发作。文献报道显示，癫痫性痉挛也存在于韦斯特综合征以外的其他癫痫综合征中，但关于其在成人中的特征的数据较少。我们描述了3例患有癫痫性痉挛且分别伴有弥漫性（病例2）、右侧额顶叶局灶性（病例1）和双侧岛盖部（病例3）巨脑回的患者（年龄分别为21岁、32岁和57岁）的发作期、临床及视频脑电图表现。痉挛分别自1个月、11岁和27岁起出现。仅1例患者存在智力发育迟缓。我们的2例患者（病例2和病例3）有部分性发作。发作期脑电图研究显示，痉挛发作时出现阳性、弥漫性、高波幅慢波活动，并伴有叠加的快波活动，随后在所有病例中均出现弥漫性脑电静息，并伴有典型的肌肉图形。如韦斯特综合征中典型描述的那样，癫痫性痉挛在成年期某些患有皮质发育异常的患者中可保持相同的症状学和脑电图特征。
