Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disorder caused by antibody to aquaporin-4 (AQP4). NMOSD can infrequently present as a complication of
posterior reversible encephalopathy syndrome (PRES). Moreover, few cases of NMOSD patients with
brain hemorrhage have been reported. We report a rare case of PRES together with NMOSD recurrence, subsequent to pontine
hemorrhage after intravenous
methylprednisolone (IVMP)
therapy. A 51-year-old Japanese woman, with a history of
hypertension and
dyslipidemia, and recurrent episodes of left visual acuity disorder related to AQP4-positive NMOSD, developed
blindness in the left eye. Brain MRI showed a hyperintense lesion in pons. She was initially diagnosed with recurrence of NMOSD and 1000 mg of IVMP was administered for 3 days. After the 3rd course of IVMP, she developed left-sided sensory disturbance, and blood pressure was increased to 202/127 mmHg. Brain computed tomography (CT) showed pontine
hemorrhage, and she was referred to our hospital again. We diagnosed PRES associated with NMOSD recurrence, along with development of pontine
hemorrhage induced by the increase in blood pressure resulting from IVMP. The patient was treated with
nicardipine to strictly control blood pressure, and
tranexamic acid and
glycerol for pontine
hemorrhage and PRES. We also extended IVMP for 5 consecutive days in total, followed by
plasmapheresis. After
therapy,
blindness in the left eye improved to light perception. Collectively, anti-AQP4 antibody could induce PRES together with recurrent NMOSD, and pontine
hemorrhage could thus be induced by blood pressure increases resulting from IVMP.