Background and Purpose: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is increasingly recognized in children. Some children have isolated disease while others relapse. The study evaluates clinical, demographic and imaging features children with positive anti-MOG antibodies comparing to previously reported findings and correlate patterns on MR imaging with a relapsing course in MOGAD.Material and methods: All pediatrics patients with serum anti-MOG antibodies were reviewed. Demographic, clinical, and imaging data were evaluated. Patients with a relapsing course were compared to those with a single event. We assessed initial MR images of the brain, orbits and spine obtained at the onset of clinical symptoms, whether performed at our institution or elsewhere.Results: Thirty patients were included, fourteen with a single event and sixteen with more than one clinical event. The mean age was 8.1 years, with a mean follow-up of 58 months (range of 0.67 to 238 months). The relapsing patients had a mean of 3.5 relapses (range 2-12). 55% of patients had long segment optic nerve lesions, 53% of patients had cortical or peripheral white matter lesions, and 46% of patients had thalamic lesions. 43% of patients had spinal cord lesions, with 39% involving the central cord and 26% with long segment involvement. The imaging features between the groups were not statistically significant.Conclusion: There were no distinguishing features in relapsing versus non-relapsing patients. In the absence of any predictive characteristics for future relapse, patients should have regular clinical and imaging follow up.& COPY; 2022 Elsevier Masson SAS. All rights reserved.

Background: Acute flaccid myelitis (AFM) is a childhood illness characterized by sudden-onset weakness impairing function. The primary goal was to compare the motor recovery patterns of patients with AFM who were discharged home or to inpatient rehabilitation. Secondary analyses focused on recovery of respiratory status, nutritional status, and neurogenic bowel and bladder in both cohorts.Methods: Eleven tertiary care centers in the United States performed a retrospective chart review of children with AFM between January 1, 2014, and October 1, 2019. Data included demographics, treat-ments, and outcomes on admission, discharge, and follow-up visits.Results: Medical records of 109 children met inclusion criteria; 67 children required inpatient rehabili-tation, whereas 42 children were discharged directly home. The median age was 5 years (range 4 months to 17 years), and the median time observed was 417 days (interquartile range = 645 days). Distal upper extremities recovered better than the proximal upper extremities. At acute presentation, children who needed inpatient rehabilitation had significantly higher rates of respiratory support (P < 0.001), nutri-tional support (P < 0.001), and neurogenic bowel (P = 0.004) and bladder (P = 0.002). At follow-up, those who attended inpatient rehabilitation continued to have higher rates of respiratory support (28% vs 12%, P = 0.043); however, the nutritional status and bowel/bladder function were no longer statistically different.Conclusions: All children made improvements in strength. Proximal muscles remained weaker than distal muscles in the upper extremities. Children who qualified for inpatient rehabilitation had ongoing respiratory needs at follow-up; however, recovery of nutritional status and bowel/bladder were similar.& COPY; 2023 Elsevier Inc. All rights reserved.

Objective N/A. Background N/A. Design/Methods Introduction: Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis signifies an autoimmune antibody-mediated neuropsychiatric disease that often presents with a set of well-described clinical characteristics and other times manifests with more rare features. The heterogeneity of patient presentation can propose a diagnostic challenge to even the best clinical neurologist. Anti-NMDA receptor encephalitis should be considered for patients who possess an alternative existing diagnosis that shows atypical progression because early recognition and treatment of the disease can help reduce long-term complications. Results Case Report: We illustrate a 14 month-old previously healthy boy with anti-NMDA receptor encephalitis who first presented with focal seizures. Initial neurologic imaging revealed intracranial hemorrhage with underlying cavernous malformations. He responded well to a single anti-seizure agent, but re-presented one week later with transient weakness that was ultimately attributed to worsening intracranial hemorrhage with surrounding edema. Upon his third presentation, he developed dyskinesias, sleep dysfunction, autonomic instability, cognitive changes, and motor regression, prompting further work-up with lumbar puncture. Cerebrospinal fluid analysis showed a positive NMDA antibody titer of 1:40. Treatment with intravenous steroids, plasma exchange (PLEX), and intravenous immune globulin (IVIg), followed by infusions of Rituximab and Cyclophosphamide resulted in gradual, marked clinical improvement. Conclusions This case study and literature review explores the relationship between cavernous malformations, intracranial hemorrhage, and anti-NMDA receptor encephalitis, and how these diagnoses respond to escalating immunomodulation therapies. Consideration of this entity should be made when the neurologic examination does not follow an expected course of a previously established diagnosis. With timely recognition and aggressive treatment approaches, patients can achieve substantial clinical improvement.

Objective: To compare clinical, diagnostic, management, and outcome factors in children with anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis and a history of herpes simplex encephalitis (HSE) to children with NMDAR encephalitis without a history of HSE. Methods: All patients with anti-NMDAR antibodies in cerebrospinal fluid treated at our institution between 2012 and 2019 were identified and divided into those with a history of HSE (HSE+NMDAR group) and those without a history of HSE (NMDAR-only group). Demographic data, clinical characteristics, immunotherapy, and outcome data were collected on all patients and compared between the 2 groups. Results: Seventeen patients were identified with anti-NMDAR antibodies in cerebrospinal fluid, 6 of whom had a history of HSE. Mean age in the HSE+NMDAR cohort was significantly younger in the HSE+NMDAR cohort, as 5 of the 6 patients were infants. Of HSE+NMDAR patients, 50% had behavioral symptoms, 67% had movement disorders, and 100% had seizures at disease nadir. In the NMDAR-only group, 100% had behavioral symptoms, 73% had movement disorders, and 73% had seizures at nadir. HSE+NMDAR patients received a median of 1 immunotherapy, compared to a median of 4.5 immunotherapies in the NMDAR-only group. Conclusion: Behavioral symptoms were more common in NMDAR-only patients, whereas seizures were more common in HSE+NMDAR patients. Both groups had significant disability at disease nadir, with more improvement in disability over time in the NMDAR-only group. HSE+NMDAR patients received fewer immunotherapies than NMDAR-only patients. Outcomes of infants with HSE appear to primarily reflect sequelae from HSE.

Objective To observe whether cases of acute flaccid myelitis (AFM) before and since August 1, 2014, had important differences and to further characterize patients with AFM regarding clinical, laboratory, imaging, and treatment findings. Methods All pediatric patients with AFM at our institution were reviewed. Demographic, clinical, and diagnostic data were collected through medical record review. Patients with onset before August 1, 2014, and after that date were compared and when applicable compared with Centers for Disease Control and Prevention data. Results Sixteen patients were included, 6 in the pre-2014 and 10 in the post-2014 group. The mean age in the pre-2014 group was 7.4 years and in the post-2014 group was 6.4 years. Initial symptoms were similar in both groups, as were functional and motor abilities at disease nadir and the most recent follow-up. Post-2014 patients had a higher mean CSF white blood cell count (57) and neutrophil count (30%) compared with pre-2014 patients (3.2 and 0.5%, respectively). Eighty percent of post-2014 patients had positive enterovirus/rhinovirus testing, with 57% of specimens positive for enterovirus D68 (EV-D68). On acute imaging, a triad of brainstem, cervical cord gray matter involvement, and ventral nerve root/cauda equina (CE) thickening/enhancement was found in 5 patients. Conclusion The groups had more similarities than differences but with a more inflammatory picture in the post-2014 patients. The constellation of cervical cord gray matter, brainstem, and nerve root/CE thickening should raise suspicion for AFM in the appropriate clinical setting. Most post-2014 patients had associated enterovirus infections, and over half tested for EV-D68 were positive. There was minimal clinical improvement in both groups despite various immunotherapies.

Objective: Compare children with acute flaccid myelitis (AFM) with onset after August 2014 to children with similar presentation before August 2014. Background: The CDC defined AFM as acute flaccid limb weakness and spinal cord grey matter injury in patients ≤21 with onset after August 1st 2014. An increase in AFM cases coincided with an outbreak of enterovirus D68 infections in 2014, leading to a hypothesized association between the two processes. While similar cases of flaccid paralysis had previously been observed, they had not been labeled AFM. Design/Methods: A tertiary children’s hospital demyelinating database with >500 patients prospectively enrolled since 2000 was queried for patients with flaccid weakness. Children with flaccid weakness onset prior to 2014 (n=5) were compared to children with AFM (n=6) with respect to demographics, clinical and MRI features, laboratory studies, treatment and outcomes. Results: The groups were similar with respect to age (mean 7.3 years pre-2014 vs 7.9 years post-2014), sex (40% vs 67% female) and race (40% vs 67% Caucasian). Weakness was isolated to one limb (40% vs 33%) or in multiple limbs (60% vs 67%). 80% of pre-2014 children and 100% of post-2014 children had MRI cervical spinal cord involvement. Both groups showed minimal clinical recovery despite aggressive treatment with immunotherapy. Post-2014 cases featured higher incidence of fever (100% vs 40%), recent infection (100% vs 60%), and hyperacute onset (67% vs 40%). Additionally, the post-2014 group demonstrated more elevated CSF protein (52.8 vs 26.8 mean, p-value 0.0032) and WBC count (50.4 vs 3.17, p-value 0.0147). Enterovirus was not detected in any pre-2014 children, but in half the post-2014 children. Conclusions: Although the etiology of AFM has not been definitively determined, increased inflammatory markers in post-2014 patients suggest a potential distinct underlying cause. Regardless of etiology, both groups experienced poor recovery, pointing to an unmet need for improved treatments. Disclosure: Dr. Marcus has nothing to disclose. Dr. Ness has nothing to disclose.