Andersen E W, Kornberg A J, Freeman J L, Leventer R J, Ryan M M
Department of Neurology, The Royal Children's Hospital, Melbourne, Victoria, Australia.
Department of Paediatrics and Child Health, University of Otago Wellington, Wellington, New Zealand.
Eur J Neurol. 2017 Aug;24(8):1077-1083. doi: 10.1111/ene.13345. Epub 2017 Jun 22.
Clusters of acute limb weakness in paediatric patients have been linked to outbreaks of non-polio enteroviruses, termed acute flaccid myelitis (AFM). Outside these clusters, in countries where polio is not endemic, this poliomyelitic-like illness is rare in childhood and its natural history is not well defined. We describe presenting features, investigation findings and long-term outcome of a series of children with AFM.
This was a retrospective cohort study.
Eight children (six females) aged 3 months to 8 years (median age 5 years) met case criteria. Initial symptoms were pain (n = 7) followed by limb weakness with hypotonia (n = 8). Flaccid paralysis occurred in only three patients. Two had cranial nerve dysfunction. Magnetic resonance imaging of the spinal cord demonstrated grey matter involvement particularly affecting the anterior cord, with longitudinally extensive changes in three children. Cerebrospinal fluid examination showed pleocytosis in six children with raised cerebrospinal fluid protein in five. Nerve conduction and electromyography findings were consistent with a motor neuronopathy. Residual deficits were common, with moderate to severe weakness seen in five patients. Median follow-up was 28 months (range 17-108 months, 30.4 patient years in total).
Acute flaccid myelitis is an uncommon condition in childhood with a high rate of significant long-term morbidity. AFM should be considered in children presenting with acute limb pain and weakness.
小儿患者中急性肢体无力的聚集性发作与非脊髓灰质炎肠道病毒的暴发有关，称为急性弛缓性脊髓炎（AFM）。在这些聚集性发作之外，在非脊髓灰质炎流行的国家，这种脊髓灰质炎样疾病在儿童期很少见，其自然病史也不明确。我们描述了一系列AFM患儿的临床表现、检查结果及长期预后。
这是一项回顾性队列研究。
8名年龄在3个月至8岁（中位年龄5岁）的儿童（6名女性）符合病例标准。初始症状为疼痛（n = 7），随后出现肢体无力伴肌张力减退（n = 8）。仅3例患者出现弛缓性麻痹。2例有脑神经功能障碍。脊髓磁共振成像显示灰质受累，尤其是前索，3例患儿有纵向广泛改变。脑脊液检查显示6例患儿有细胞增多，5例脑脊液蛋白升高。神经传导和肌电图结果与运动神经元病一致。残留缺陷很常见，5例患者有中度至重度无力。中位随访时间为28个月（范围17 - 108个月，共30.4患者年）。
急性弛缓性脊髓炎在儿童期并不常见，长期严重发病的发生率较高。对于出现急性肢体疼痛和无力的儿童应考虑AFM。
