Dalla Bernardina B, Dulac O, Bureau M, Dravet C, Del Zotti F, Roger J
Rev Electroencephalogr Neurophysiol Clin. 1982 Apr;12(1):8-14. doi: 10.1016/s0370-4475(82)80002-6.
The authors describe the electroclinical and evolutive aspects of 4 cases (including 2 brothers) of myoclonic epileptic encephalopathy beginning between 2 days and 10 weeks of life. From the onset of myoclonic jerks, polymorphous fits (partial seizures, tonic seizures) and multifocal electrical abnormalities are associated. Repeated spasms and 'suppression-burst' patterns appear later. The neurological status deteriorates progressively, leading within a few months to decerebration posture with opisthotonus. In spite of thorough neuroradiological, biochemical, cytological to metabolic investigations, etiology remains unknown. However, the electroclinical and evolutive patterns are similar to that of metabolic diseases, especially non-ketotic hyperglycemia. The authors discuss the relations between their observations and those in the literature and the nosological problems of this particular epileptic encephalopathy of infancy.
作者描述了4例（包括2名兄弟）生后2天至10周起病的肌阵挛性癫痫性脑病的临床电生理及演变情况。自肌阵挛性抽搐起病，多形性发作（部分性发作、强直性发作）及多灶性电异常与之相关。之后出现反复痉挛及“抑制-爆发”模式。神经状态逐渐恶化，数月内导致去大脑强直姿势。尽管进行了全面的神经放射学、生物化学、细胞学及代谢检查，病因仍不明。然而，临床电生理及演变模式与代谢性疾病相似，尤其是非酮症高血糖症。作者讨论了他们的观察结果与文献报道之间的关系以及这种特殊婴儿癫痫性脑病的分类学问题。
