Aims & Objectives: Introduction: Encephalitis with antibodies against N-methyl-D-aspartate receptor (NMDAR) is recognized as a group of antibody mediated neuropsychiatric syndrome. Autoimmune encephalitis associated with extensive myelitis is an atypical association. An atypical presentation of the disease in children can result in delay of diagnosis and treatment. Methods Case presentation: We report an obese 9 year old boy, who presented with the significant progression of anti-NMDAR encephalitis. With significant clinical findings of autoimmune encephalitis we initiated immunotherapy. He required cyclophosphamide in view of deterioration with unresponsiveness and dysautonimia. All his tumor screens and neuroimaging were normal. Follow up outcome was promising, but he presented with two episodes of relapse. The last episode involved visual loss, with the diagnosis of isolated optic neuritis. He required immunotherapy and recovered over a week’s duration with no aggression and dystonia. With a negative serum anti-NMDAR antibody during the last admission, we had high suspicion of neuromyelitis optica. Neuromyelitis optica is a common presentation of aquaporin-4 receptor antibody encephalitis and could be associated with anti-NMDAR encephalitis. Over the next 5 months, he showed good progression with interaction and activities of daily living. Conclusions Conclusions: Anti-NMDAR encephalitis is a complex reversible syndrome with characteristic symptomatology. Concurrent or separate episodes of demyelinating disorders in patient with anti-NMDAR encephalitis, should not be overlooked. Attention should be paid when patients with demyelinating disorders have co-occurring autoimmune encephalitis. They would have a higher neurological morbidity, and intense immunotherapies is required.
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