We report a case of neuromyelitis optica (NMO) with a large cerebral lesion. The patient had an episode of fever and consciousness disturbance with a tumefactive frontal white matter lesion at age 43, and then repeated bilateral optic neuritis and transverse myelitis until she died at age 63. Histopathological examinations revealed that marked tissue destruction, cavities and inflammatory changes typical of NMO were seen in the cerebrum as well as the optic nerves and spinal cord. This is the first autopsied case of NMO with a tumefactive cerebral lesion that later became cavitary.
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