Neuromyelitis Optica Spectrum Disorder: A Case Report

BACKGROUND: Neuromyelitis optica spectrum disorder is an autoimmune, astrocytopathic CNS disease, mainly involving the optic nerves, spinal cord, and brain stem regions. The “International Panel for NMOSD Diagnosis (IPND) Diagnostic Criteria” was implemented to define the disorder. CASE PRESENTATION: A 38-year-old patient presented with visual loss of eight months' duration and weakness of the lower extremities of one week's duration. The patient had bilateral optic atrophy on fundoscopic examination, and flaccid paraplegia with sensory loss below T4 level. Serological tests for syphilis, HIV infection, and SLE were negative. Aquaporin-4 antibody test was not done due to limited clinical setup. T2-spine MRI revealed long central thoracic segment (T3 to T6) hyperintense lesion with mild cord expansion. Long segment central canal dilation (syrinx) was noted in the cord proximal to the lesion. Diagnosis of opticospinal variant, NMOSD was made using IPND diagnostic criteria. The patient was started on dexamethasone 50 mg, IV, four times daily (QID) for one week, and changed to prednisolone 1 mg/kg (40 mg) PO daily for one month, to be tapered over three-to-six months. The patient was scheduled to initiate azathioprine 50 mg PO twice daily. CONCLUSION: The case emphasizes the existence of neuromyelitis optica spectrum disorder in clinical settings of the developing world. High index of suspicion of this rare disease is required to avoid delayed diagnosis and treatment.