May 5, 2010 — Devic’s neuromyelitis optica (DNMO) is a demyelinating disease characterized by bilateral visual disturbance and transverse myelopathy. It was first described in 1894 by Eugene Devic [1] in a woman who suffered from a bilateral optic neuritis and acute transverse myelitis. Pathologically, lesions are restricted to the optic nerves and spinal cord, with areas of necrosis of gray and white matter, cavitations, lack of inflammatory infiltrate, vascular hyalinization, and fibrosis [2]
Background Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that predominantly affects the optic nerves and the spinal cord, and is possibly mediated by an immune mechanism distinct from that of multiple sclerosis (MS).
Neuromyelitis optica (NMO; Devic’s disease) and the NMO spectrum disorders are idiopathic inflammatory demyelinating disorders that affect the central nervous system and have a predilection for optic nerves and spinal cord. The identification of NMO-IgG as a disease-specific marker and aquaporin 4 as the target antigen has renewed interest in NMO.
Neuromyelitis optica (NMO, Devic’s syndrorne.l is characterized by concurrence of optic neuritis and transverse myelitis, typically associated with a lesion in the spinal cord extending over three or more vertebral segments. It is an inflammatory, demyelinating central nervous system disorder, and although it is most commonly relapsing, it is distinct from multiple sclerosis in that it is more severe, tends to spare the brain, and is associated with a longitudinally extensive lesion on spinal cord MRI. Furthermore, NMO is associated with a highly specific serum autoantibody m,1rker, NMO-lgG, which targets the water channel aquaporin-4.
INTRODUCTION: Loss of aquaporin 4 and glial fibrillary acidic protein (GFAP) with necrosis and demyelination is a prominent pathologic feature of neuromyelitis optica (NMO). However, the clinicopathologic significance of astrocytic damage and its relation with demyelination are unknown
Background: Although neuromyelitis optica has been traditionally regarded as a disease without brain involvement, brain abnormalities are not uncommon in patients with neuromyelitis optica-related disorders.Methods: We aimed to characterize the brain magnetic resonance imaging (MRI) abnormalities in neuromyelitis optica spectrum disorder patients who are seropositive for anti-aquaporin-4 autoantibody (AQP4 Ab). Of 236 consecutive patients with inflammatory demyelinating central nervous system diseases, we retrospectively analyzed MRI characteristics of 78 patients who were seropositive for AQP4 Ab.Results: For an average observational period of 6.3 years, 62 patients (79%) had brain lesions on MRI