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- W2341461004 abstract "OBJECTIVE: Use a patient case to highlight the potential for neuropsychiatric sequelae of neuromyelitis optica (NMO). BACKGROUND: NMO is an autoimmune disease resulting in demyelination of the optic nerves and spinal cord, typically causing vision loss and paralysis. Psychiatric symptoms are uncommon. DESIGN/METHODS: We present a case study of severe behavioral dysregulation as a result of neuromyelitis optica. RESULTS: A 6-year-old girl developed left-sided paralysis progressing to encephalopathy with neurogenic bowel and bladder, shortly after a viral illness. MRI of brain and spinal cord revealed T2 hyperintensities in the cerebellum, temporal white matter, and cervical spinal cord. Initial tests for NMO antibodies were negative. She was diagnosed with Acute Disseminated Encephalomyelitis (ADEM) and improved with intravenous immunoglobulin (IVIG) and steroids. Following prolonged steroid taper, she developed contralateral hemiparesis and optic neuritis. NMO testing was again negative.At age 8, she presented with encephalopathy and seizures. Brain MRI revealed new T2 hyperintense lesions in the medial thalami. Repeat NMO antibody testing was positive. Steroid taper resulted in severe behavioral dysregulation, perseveration of thoughts and phrases, and sudden, violent mood swings which improved modestly with steroid increase. Steroids were ultimately tapered due to side effects; she required a prolonged psychiatric hospitalization. Despite stable neurologic exam, imaging, and effective immunosuppression with rituximab, multiple psychotropic medication trials failed to improve progressively worsening symptoms, including severe unprovoked aggression toward self. CONCLUSIONS: This case illustrates the importance of repeat antibody testing in cases of intractable symptoms, and the formidable psychiatric challenges that may complicate the course of illness in NMO. We hypothesize that medial thalamic lesions contribute to the patient’s significant behavioral changes, representing a novel etiology of neuropsychiatric symptoms in NMO. Study Supported by: NA Disclosure: Dr. Schreiner has received personal compensation for activities with Teva Neuroscience as a speaker. Dr. Karlsson has nothing to disclose. Dr. Levine has nothing to disclose. Dr. Barnes has nothing to disclose. Dr. Binder has nothing to disclose. Dr. Gabriels has nothing to disclose. Dr. Beresford has nothing to disclose." @default.
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- W2341461004 date "2014-04-08" @default.
- W2341461004 modified "2023-09-26" @default.
- W2341461004 title "Neuromyelitis Optica with Severe Behavioral Dysregulation: A Case Report (P2.269)" @default.
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