Acute Flaccid Myelitis: Review of Clinical Features, Diagnosis, and Management with Nerve Transfers

BACKGROUND: Acute flaccid myelitis (AFM) is a devastating neurologic condition in children, manifesting as acute limb weakness and/or paralysis. Despite increased awareness of AFM following initiation of U.S. surveillance in 2014, no treatment consensus exists. The purpose of this systematic review was to summarize the most current knowledge regarding AFM epidemiology, cause, clinical features, diagnosis, and supportive and operative management, including nerve transfer. METHODS: The authors systematically reviewed the literature based on Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines using multiple databases to search the keywords ("acute flaccid myelitis"), ('acute flaccid myelitis'/exp OR 'acute flaccid myelitis'), and (Acute AND flaccid AND myelitis). Included articles reported on (1) AFM diagnosis and (2) patient-specific data regarding epidemiology, cause, clinical features, diagnostic features, or management of AFM. RESULTS: Ninety-nine articles were included in this review. The precise cause and pathophysiologic mechanism of AFM remain undetermined, but AFM is strongly associated with nonpolio enterovirus infections. Clinical presentation typically comprises preceding viral prodrome, pleocytosis, spinal cord lesions on T2-weighted magnetic resonance imaging, and acute onset of flaccid weakness/paralysis with hyporeflexia in at least one extremity. Supportive care includes medical therapy and rehabilitation. Early studies of nerve transfer for AFM have shown favorable outcomes for patients with persistent weakness. CONCLUSIONS: Supportive care and physical therapy are the foundation of a multidisciplinary approach to managing AFM. For patients with persistent limb weakness, nerve transfer has shown promise for improving function in distal muscle groups. Surgeons must consider potential spontaneous recovery, patient selection, donor nerve availability, recipient nerve appropriateness, and procedure timing.