Yamatogi Yasuko, Ohtahara Shunsuke
Faculty of Health and Welfare Science, Okayama Prefectural University, 111 Kuboki, Soja-shi, Okayama Prefecture, Japan 719-1197.
J Clin Neurophysiol. 2003 Nov-Dec;20(6):442-8. doi: 10.1097/00004691-200311000-00006.
Severe epilepsy with multiple independent spike foci is an electroclinical entity with the following characteristics: (1) EEG showing multiple independent spike foci (three or more foci in both hemisphere, i.e., at least one in each hemisphere) and diffuse slowing of the background activity, (2) very frequent multiple types of seizures but mainly generalized minor seizures, (3) frequent association with mental retardation and neurologic abnormalities, (4) underlying causes of various nonspecific prenatal, perinatal, and postnatal cerebral conditions, and (5) poor prognoses for seizures and psychomotor development. It represents a diffuse encephalopathy with mutual transition between other age-dependent epileptic encephalopathies. This electroclinical condition may be appropriately named Markand-Blume-Ohtahara syndrome, after Markand and Blume, who initially described in detail the EEG pattern of multifocal spike discharges with its major clinical correlates, and Ohtahara, who extensively studied the group of patients with both clinical and EEG characteristics and proposed the concept of severe epilepsy with multiple independent spike foci, classifying it as one type of generalized epilepsy.
（1）脑电图显示多个独立棘波灶（双侧半球三个或更多灶，即每个半球至少一个）以及背景活动弥漫性减慢；（2）非常频繁地出现多种类型发作，但主要是全身性小发作；（3）常伴有智力发育迟缓及神经学异常；（4）存在各种非特异性产前、围产期及产后脑部疾病的潜在病因；（5）癫痫发作及精神运动发育预后不良。它代表一种弥漫性脑病，与其他年龄依赖性癫痫性脑病相互转化。这种电临床状况可恰当地命名为马尔坎德 - 布卢姆 - 大田原综合征，以纪念马尔坎德和布卢姆，他们最初详细描述了多灶性棘波放电的脑电图模式及其主要临床相关表现，以及大田原，他广泛研究了具有临床和脑电图特征的这组患者，并提出了伴有多个独立棘波灶的严重癫痫的概念，将其归类为一种全身性癫痫。
