BRAIN and NERVE Shinkei Kenkyu no Shinpo Volume 70, Issue 2 (February 2018)

Acute Flaccid Myelitis Ryutaro Kira 1 1Department of Pediatric Neurology, Fukuoka Children's Hospital Keyword: エンテロウイルスD68 , ポリオ様麻痺 , 急性弛緩性麻痺 , 急性弛緩性脊髄炎 , enterovirus D68 , polio-like illness , acute flaccid myelitis , acute flaccid paralysis pp.99-112
Published Date 2018/2/1
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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.

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BRAIN and NERVE-神経研究の進歩
70巻2号 (2018年2月)
電子版ISSN 1344-8129 印刷版ISSN 1881-6096 医学書院