Neuromyelitis Optica

Abstract

Neuromyelitis optica (NMO), formerly known as Devic disease, is an autoimmune astrocytopathic disease characterized by transverse myelitis and optic neuritis. Most patients demonstrate a relapsing course with incomplete recovery between attacks, resulting in progressive disability. The pathogenesis involves production of aquaporin-4 antibodies (AQP4-IgG) by plasmablasts in peripheral circulation, disruption of the blood-brain barrier, complement-mediated astrocyte injury, and secondary demyelination. The diagnosis relies on characteristic clinical manifestations in the presence of serum AQP4-IgG positivity or specific neuroimaging findings, and exclusion of alternative etiologies. Current treatment involves aggressive immunosuppression with pulse-dose steroids during acute attacks and long-term immunosuppression for attack prevention.

Involvement of Aquaporins in the Pathogenesis, Diagnosis and Treatment of Sjögren’s Syndrome

“…Neuromyelitis optica, also known as Devic’s syndrome, is a rare autoimmune disease affecting the central nervous system. NMO spectrum disorders (NMOSD) are sometimes found in association with other autoimmune disorders, including SS [ 67 ]. The prominent fact that antibodies against AQP4 have been found in the majority of patients suffering from NMOSD differentiates the latter from other demyelinating diseases such as multiple sclerosis [ 68 ].…”

Magnetic resonance imaging of intraocular optic nerve disorders: review article

“…The optic nerve lesion in NMO-SD, like MS, is nonspecific on MRI (Figure 3), but exists for longer than that in MS [13]. On brain MRI, high signal on T2-weighted images is seen mainly in the dorsal side of the medulla, the fourth ventricle peripheries, the thalamus, and the hypothalamus, where the expression of AQP4 is high [14].…”

“…The optic nerve can also be affected by various infectious diseases such as Bartonella, cytomegalovirus, Epstein-Barr virus, human immunodeficiency virus, Lyme disease, syphilis, toxoplasmosis, toxocariasis, and varicella zoster virus, but there are no comprehensive reports about MRI findings in optic neuritis due to these infections [14].…”

Cerebrospinal fluid flow. Part 2. Aquaporins and their role in CNS water homeostasis

“…Оптикомиелит (болезнь Девика) -аутоиммунное воспалительное заболевание ЦНС, характеризующееся селективным поражением зрительного нерва и спинного мозга [102]. Рассматривая роль AQP4 в развитии патологических состояний ЦНС нельзя не упомянуть оптикомиелит, при котором AQP4-специфические антитела идентифицированы как высокоспецифичная терапевтическая мишень.…”