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Background Acute Flaccid Myelitis (AFM), diagnosed almost exclusively in children, is characterized by sudden onset flaccid weakness in one or more extremities with distinct gray matter spinal cord lesions on magnetic resonance imaging (MRI), with or without cerebrospinal fluid (CSF) pleocytosis. Outbreaks of AFM have occurred biennially since 2014. Although the definitive causative agent(s) remain unknown, current data support an association with Enteroviruses D68 and A71. Treatment is supportive and long-term prognosis is variable, with many children having persistent motor deficits. Methods In this prospective cohort study, we identified patients with clinical and radiographic presentation consistent with AFM at Children’s National Hospital (CNH) from 2013–2019. Medical records and MRIs of identified patients were then reviewed by members of the multidisciplinary CNH AFM Task Force to identify those meeting diagnostic criteria for AFM. Identified patients had follow-up arranged in the multidisciplinary AFM clinic for exam, functional motor assessment and quality of life questionnaires (Peds QL, PROMIS and NeuroQoL). Results Since 2013, we identified 22 patients meeting criteria for AFM at CNH. The average age of our patients was 7.25 years (range 6 months to 16 years); almost 2/3 of patients had CSF pleocytosis. Half of patients presented with initial neurologic complaint of single limb weakness. Other presenting neurological symptoms included ataxia, bilateral lower extremity weakness and ophthalmoplegia. A potential infectious cause was identified in the CSF, blood, nasopharynx or stool of 9 patients. As of November 2019, 7 of 21 patients have had follow-up evaluation; 1 had no improvement, 4 had partial improvement but with persistent motor deficits, and 2 had complete resolution. Two patients/families have completed quality of life questionnaires to date. CNH AFM Patient Characteristics and Results Conclusion Similar to other centers, the majority of CNH AFM patients with follow-up to date have persistent and significant long term motor deficits. Assessment of quality of life is an important aspect that has not yet been formally assessed in other studies and will provide useful information regarding the experience of these patients and help identify goals for optimizing care in the future. Disclosures All Authors: No reported disclosures

Objectives Although secondary hemophagocytic lymphohistiocytosis (HLH) has been reported in children with critical illness of various etiologies, it has not been reported in patients with febrile infection-related epilepsy syndrome (FIRES). We describe a series of patients with concurrent HLH and FIRES in an effort to establish common pathophysiologic abnormalities. Methods Five patients with FIRES who were assessed for HLH were identified from a neurocritical care database. All were previously healthy and had extensive diagnostic testing. All had clinical deterioration with multiorgan dysfunction prompting HLH screening 20-29 days after hospitalization. Markers for inflammatory dysregulation were assessed in cerebrospinal fluid (CSF) and serum at various time points. Outcomes were assessed 6 months after presentation. Results Three patients met clinical criteria for secondary HLH. Elevation of specific cytokines/chemokines was variable. CSF neopterin, high mobility group box 1 (HMGB1), and C-X-C motif chemokine ligand 8 (CXCL8) were significantly elevated in all. Interleukin-1β (IL-1β) and IL-18 were not elevated in any of the samples. Treatment and outcomes were variable. Significance We describe 3 patients with HLH and FIRES. The co-occurrence of these 2 rare disorders suggests the possibility of a common immune dysregulation phenotype prolonging epileptogenesis. HLH screening in critically ill patients with FIRES may yield a broader understanding of shared inflammatory processes.