Neuromyelitis Optica Presenting as Non-Communicating Hydrocephalus: A Case Report

Neuromyelitis Optica (NMO) is a demyelinating disease predominantly involving optic nerves, spinal cord and peri-ventricular regions which are rich in Aquaporin-4 receptors. Aquaporin-4 (AQP4) antibodies are implicated in the pathogenesis of NMO. Association of hydrocephalus ( communicating and non communicating) with NMO is very rare. We report a case of 32 years old female patient who presented with 2 months history of progressive headache, visual obscurations and gait imbalance . Clinical examination revealed bilateral papilloedema with preserved visual acuity. She had truncal and gait ataxia. Rest of the examination of nervous system was normal. MRI brain showed non- communicating hydrocephalus and T2 and FLAIR hyperintensities in periventricular and periaqueductal regions. AQP4 antibodies were positive in serum and negative in cerebrospinal fluid(CSF). Ventriculo - peritoneal shunt was placed and she was treated with steroids and azathioprine. Her headache and visual symptoms improved. However, after 8 months she presented with acute optic neuritis of right eye which was treated with intravenous methylprednisolone and plasmapharaesis.