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[Acute Flaccid Myelitis].
Brain and Nerve = Shinkei Kenkyū No Shinpo 2018 Februrary
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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