本文描述9例肌阵挛性癫痫性脑病的临床、脑电图状况及其进展。婴儿出生时健康,早期发病,临床进展每况愈下,可能是1岁以内癫痫性脑病的一种特殊类型,作者将其命名为早期肌阵挛性癫痫性脑病。9例中,男4女5,4例家族有类似患者,患儿在妊娠期、分娩期及出生时检查(包括头围、肌张力和反应性)均正常,出生后2～70天发病,开始为肌阵挛性局部的痉挛和周期性的阵发性的脑电图异常,反复的全身痉挛与部份的痉挛和爆发性抑制(sup This article describes the 9 cases of myoclonic epileptic encephalopathy clinical, EEG status and progress. The baby is healthy at birth, has early morbidity, and its clinical progress is deteriorating. It may be a special type of epileptic encephalopathy within 1 year of age, which the author named early myoclonic epilepsy encephalopathy. Among the 9 cases, 5 males and 4 females had similar patients in 4 and 4 families. The children were normal during pregnancy, childbirth and birth (including head circumference, muscle tension and reactivity), and were born 2 to 70 days after birth. Beginning with myoclonic local spasms and periodic paroxysmal EEG abnormalities, recurrent systemic spasms and partial spasticity and overt supination