Lombroso C T
Department of Neurology, Harvard Medical School, Boston, Massachusetts 02115.
J Clin Neurophysiol. 1990 Jul;7(3):380-408. doi: 10.1097/00004691-199007000-00005.
Nosological confusion within the epilepsies with myoclonic manifestations occurring in early life has led several epileptologists to separate more rigorously true myoclonic seizures from pseudomyoclonic ones and to identify clusters of homogeneous parameters that may lead to the formulation of syndromatic groupings. In recent years, four neonatal, infantile, and early myoclonic syndromes have been proposed: early myoclonic encephalopathy (EME), early infantile epileptic encephalopathy (EIEE), benign infantile myoclonic epilepsy (BIME), and severe infantile myoclonic epilepsy (SIME). These are reviewed critically, historically, and in the context of personal observations. The author's conclusions are that there is some justification to support, provisionally, a nosological place for the EME syndrome, that a nosologically separate position for the EIEE syndrome appears less firm, and that it seems safer to consider it at this time as an early variant of the West syndrome. From personal observations it appears that BIME and SIME, while justifiably constituting recognizable entities, may best be combined into a single syndrome of "infantile myoclonic epilepsy following febrile convulsions," with variable clinical outcomes.
在生命早期出现的伴有肌阵挛表现的癫痫中，疾病分类的混淆使得一些癫痫学家更严格地将真正的肌阵挛发作与假性肌阵挛发作区分开来，并确定可能导致综合征分组形成的同质参数群。近年来，已经提出了四种新生儿、婴儿期和早期肌阵挛综合征：早期肌阵挛性脑病（EME）、早期婴儿癫痫性脑病（EIEE）、良性婴儿肌阵挛癫痫（BIME）和严重婴儿肌阵挛癫痫（SIME）。本文对这些综合征进行了批判性、历史性的回顾，并结合个人观察进行了探讨。作者的结论是，有一定理由暂时支持EME综合征在疾病分类中的地位；EIEE综合征在疾病分类上单独存在的地位似乎不太稳固，目前将其视为韦斯特综合征的早期变体似乎更稳妥。从个人观察来看，BIME和SIME虽然有理由被视为可识别的实体，但最好将它们合并为一个单一的“热性惊厥后婴儿肌阵挛癫痫”综合征，其临床结果各不相同。
