DEVICS-NEUROMYELITIS-OPTICA - A CLINICOPATHOLOGICAL STUDY OF 8 PATIENTS

We report the clinical, imaging, and laboratory features of 8 patients with Devic's neuromyelitis optica. All patients had severe myelopathy and optic neuritis. In no patient was the brain, the brainstem, or th e cerebellum affected, even after several years of disease. Various im munosuppressive treatments failed to benefit the patients, 5 of whom d ied. Autopsies of these 5 patients demonstrated a severe necrotizing m yelopathy with thickening of blood vessel walls and no lymphocyte infi ltrates. In the appropriate clinical setting, the lack of white matter abnormalities demonstrated by magnetic resonance imaging of the head facilitates the recognition of Devic's syndrome during life. Inasmuch as Devic's myelopathy is necrotizing, rather than demyelinating, the p rognosis of this syndrome is poor.