Otani K, Abe J, Futagi Y, Yabuuchi H, Aotani H, Takeuchi T
Division of Pediatric Neurology, Osaka Medical Center, Japan.
Brain Dev. 1989;11(5):332-7. doi: 10.1016/s0387-7604(89)80064-6.
We report a clinico-electroencephalographical follow-up study on a male patient with early myoclonic encephalopathy. Frequent massive and fragmentary myoclonic seizures, and myoclonic-clonic seizures were the initial symptoms at the age of 3 days. EEG revealed a suppression-burst pattern at the onset in which burst phases often coincided with myoclonic seizures. Subsequently, non-epileptic erratic myoclonus, various partial seizures and flexor spasms were observed. The partial seizures ceased at around 4 months of age, while the non-epileptic myoclonus and flexor spasms have persisted beyond the age of 6 months. The EEG pattern evolved into atypical hypsarhythmia at two months of age. No specific biochemical or neuroradiological findings were disclosed. His neuropsychiatric development was arrested from the onset. These observations suggest that early myoclonic encephalopathy is an independent epileptic syndrome and that it might be different from early-infantile epileptic encephalopathy described by Ohtahara.
我们报告了一例早期肌阵挛性脑病男性患者的临床-脑电图随访研究。频繁的大量和片段性肌阵挛发作以及肌阵挛-阵挛发作是该患者3日龄时的初始症状。脑电图在发病初期显示为抑制-爆发模式,其中爆发期常与肌阵挛发作同时出现。随后,观察到非癫痫性不规则肌阵挛、各种部分性发作和屈肌痉挛。部分性发作在4个月龄左右停止,而非癫痫性肌阵挛和屈肌痉挛持续至6个月龄以后。脑电图模式在2个月龄时演变为非典型高峰节律紊乱。未发现特异性生化或神经放射学异常。其神经精神发育从发病起就停止了。这些观察结果表明,早期肌阵挛性脑病是一种独立的癫痫综合征,可能与大原(Ohtahara)描述的早期婴儿癫痫性脑病不同。