Nissen M D, Appleton D B
Department of Neurology, Royal Children's Hospital, Herston, Queensland, Australia.
J Paediatr Child Health. 1994 Dec;30(6):497-501. doi: 10.1111/j.1440-1754.1994.tb00720.x.
Myoclonic encephalopathy of infancy (MEI) is a unique cause of acute ataxia in infants and is a rare presentation of neuroblastoma. Five cases presenting to a tertiary referral children's hospital during a 10 year period are reviewed. Two cases were associated with a neuroblastoma. All children were treated with intramuscular injections of adrenocorticotropic hormone, with symptomatic improvement. One child died from an opportunistic infection following chemotherapy for neuroblastoma. The four survivors have mild to moderate clinical and intellectual deficits. Investigation and continuing observation for occult neural crest tumours is emphasized for all cases of MEI, though no underlying cause was found in 60% of children in this study.
婴儿肌阵挛性脑病（MEI）是婴儿急性共济失调的一种独特病因，也是神经母细胞瘤的罕见表现形式。本文回顾了一家三级转诊儿童医院在10年期间收治的5例病例。其中2例与神经母细胞瘤有关。所有患儿均接受了促肾上腺皮质激素肌肉注射治疗，症状有所改善。1例患儿在接受神经母细胞瘤化疗后死于机会性感染。4名幸存者有轻度至中度的临床和智力缺陷。对于所有MEI病例，均强调对隐匿性神经嵴肿瘤进行检查和持续观察，尽管在本研究中60%的患儿未发现潜在病因。
