Kira Ryutaro
Department of Pediatric Neurology, Fukuoka Children's Hospital.
Brain Nerve. 2018 Feb;70(2):99-112. doi: 10.11477/mf.1416200962.
Acute flaccid myelitis (AFM) is a newly defined, rare, but clinically distinct syndrome of acute flaccid paralysis with spinal motor neuron involvement of unknown etiology. Clusters of AFM coincided with a nationwide outbreak of enterovirus D68 (EV-D68) in the United States during autumn to winter of 2014 and in Japan during the autumn of 2015. Although EV-D68 was detected in only 20% of the AFM patients, mainly from respiratory specimens without apparent viral detection from blood and cerebrospinal fluid (CSF) samples, strong temporal associations were noted. Core symptoms of AFM are focal limb weakness and cranial nerve dysfunction with acute onset, although limb paralysis varied in type and severity. Most patients showed extensive longitudinal spinal involvement on magnetic resonance imaging, pleocytosis in CSF, and abnormal motor conduction and/or F-waves in neurophysiological investigations in the acute phase which was within two weeks after onset. Immunomodulation therapy consisting of high-dose intravenous immunoglobulin or pulse methylprednisolone did not improve prognosis. Only about 10% of the patients showed complete recovery of limb weakness, but others had variable residual weakness at follow-up. With no effective treatment identified in the acute stage, studies are required to elucidate the pathophysiology and pathomechanisms of AFM and to develop both preventive measures and novel therapeutic interventions.
急性弛缓性脊髓炎(AFM)是一种新定义的、罕见但临床特征明显的急性弛缓性麻痹综合征,累及脊髓运动神经元,病因不明。2014年秋冬,美国全国范围内爆发肠道病毒D68(EV-D68)疫情,2015年秋季日本也出现疫情,期间AFM病例呈聚集性出现。虽然仅在20%的AFM患者中检测到EV-D68,主要是在呼吸道标本中检测到,血液和脑脊液(CSF)样本中未明显检测到病毒,但两者在时间上有很强的关联性。AFM的核心症状是急性起病的局灶性肢体无力和颅神经功能障碍,尽管肢体麻痹的类型和严重程度各不相同。大多数患者在发病两周内的急性期进行磁共振成像检查时显示脊髓广泛纵向受累,脑脊液中细胞增多,神经生理学检查显示运动传导异常和/或F波异常。由大剂量静脉注射免疫球蛋白或脉冲甲基强的松龙组成的免疫调节治疗并不能改善预后。只有约10%的患者肢体无力完全恢复,但其他患者在随访时有不同程度的残留无力。由于在急性期未确定有效的治疗方法,因此需要开展研究以阐明AFM的病理生理学和发病机制,并制定预防措施和新的治疗干预措施。