Neuromyelitis optica

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By Adil Javed MD PhD and John Pula MD

Neuromyelitis optica, also known as Devic disease, affects either the optic nerves or spinal cord, or both.  It may be misdiagnosed as multiple sclerosis and vice versa-ed.

Neuromyelitis optica, also known as Devic disease, is a CNS inflammatory disease. It is a devastating disease and leaves many patients with permanent vision loss and paralysis. Neuromyelitis optica is thought to be caused by abnormal activity of B cells. In fact, most neuromyelitis optica patients have elevated levels of an IgG antibody to a water channel called aquaporin-4 (NMO-IgG). This antibody has been hypothesized to be disease-causing because it can fix complement and, thus, can cause tissue destruction. However, not all patients have the anti-aquporin-4 antibody. Hence, other pathological mechanisms must exist that are responsible for tissue destruction as well. Patients presenting with neuromyelitis optica must be treated urgently with aggressive measures, such as chemotherapeutic agents. Also, these patients must be recognized early in the disease course. The authors here review extensive literature regarding the pathology of neuromyelitis optica, including their own observations. Also, mixed with their own clinical experience, they review treatment options available for neuromyelitis optica.