[Neuromyelitis optica following thymectomy with severe spinal cord atrophy after frequent relapses for 30 years].

A 60-year-old woman had frequent relapses of neuromyelitis optica (NMO) for 30 years despite receiving steroid and azathioprine therapy. She developed MGFA Class IIIb type of myasthenia gravis (MG) at the age of 23, and thymectomy resulted in complete remission of MG. The initial symptoms of NMO, including headache, high fever, retrobulbar optic neuritis, and neurogenic bladder, appeared at the age of 30. Two years later, paraplegia also developed. Although she received oral administration of steroids or azathioprine and intravenous steroid pulse therapy for treatment of NMO for over 30 years, she experienced frequent relapses. The examination at the ages of 58 and 60 years showed that anti-aquaporin-4 antibody was absent. Intravenous immunoglobulin therapy administered in January and June 2009 was effective, and she had 2 years of remission of NMO attack. Spinal MRI after frequent NMO attacks for 30 years revealed an extended spinal cord atrophy involving the lower cervical region and the entire thoracic region. We describe and discuss the prognosis of NMO and the effectiveness of therapies in an NMO patient who underwent thymectomy for MG.