Transverse myelitis syndrom as a result of neuromyelitis optica spectrum disorders, systemic lupus erythematosus and myasthenia gravis combination

Abstract

Neuromyelitis optica spectrum disorders (NMOSD) - autoimmune condition characterized by an inflammatory lesions mainly of the spinal cord with the development of longitudinally extensive transverse myelitis (LETM) and/or involvement of the optic nerve with the development of usually bilateral optical neuritis (ON). In recent years, there has been increased awareness that NMOSD can be combined with other autoimmune diseases, including myasthenia gravis (MG), systemic lupus erythematosus (SLE) et al. The simultaneous presence of several autoimmune diseases in one patient can adversely affect the course of each of the diseases, causing the so-called mutual burden or «overlap syndrome». In this article, we describe our own clinical observation of a 51-year-old woman of European origin who developed acute relapsing TM seropositive for AQP4-IgG, by 23 years after the diagnosis of generalized MG seropositive for antibodies to acetylcholine receptors (AChR-Ab) and the occurrence of SLE, criterially confirmed, several months after the initial TM attack. During the fourth TM attack, partial positive dynamics was achieved only against the background of the combined use of intravenous methylprednisolone (pulse therapy), high-volume plasma exchange, rituximab and cyclophosphamide. The NMOSD is a rare disease leading to severe disability. In patients with MG, when symptoms of damage to the central nervous system appear, an analysis should be performed for AQP4-IgG and possibly for antibodies to myelin glycoprotein of oligodendrocytes (MOG-Ab), as well as markers characteristic of systemic connective tissue diseases (SCTD). In patients with STDD, when symptoms of involvement nervous systemappear, testing for AQP4-IgG (and, if necessary, for MOG-Ab) should be performed to exclude NMOSD, as well as AChR-Ab (and, if necessary, antibodies against muscle specific kinase (MuSK-Ab)) to exclude MG.