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]. Clinically, the disease may have a mono- or multiphasic course [3]. The nosology is not clear, and the reports from the literature are confusing. Historically, the disease was defined as a monophasic disorder consisting of fulminant bilateral optic neuritis and myelitis, occurring in close temporal association. Cases of DNMO that followed in the literature described more extensive findings, with a relapsing course, which raised the question of whether DNMO represents a separate syndrome or a variant of MS. One of the largest series published by a group from the Mayo Clinic included 71 patients who had DNMO [4]. Based on their findings, the initial definition was revised. Clinical characteristics, course, and prognosis have been evaluated further on 46 patients from 15 Italian MS centers [5]. Compared with patients who had MS, patients who had DNMO had a poor prognosis, higher age at onset, and a more sever clinical course. DNMO was most like to affect female patients. Corticosteroids are not helpful in DNMO, and the prognosis is poor. Cerebral spinal fluid (CSF) abnormalities include pleocytosis, high protein, and high albumin ratio levels [5].
Read More: Myelopathy due to Devic’s neuromyelitis optica: A transverse myelitis process