MODERN VIEW OF DIAGNOSIS AND TREATMENT OF MYASTHENIA GRAVIS. CASE REPORT

Abstract

Introduction. Myasthenia gravis (MG) or Erb-Goldflam syndrome is an autoimmune neuromuscular disease based on autoaggression of humoral immunity against elements of neuromuscular synapses and intracellular structures of muscle fibers (antibodies to acetylcholine receptors (AchR), muscle-specific tyrosine kinase (MuSK), protein 4 related to low-density lipoprotein receptors (LRP4), ryanodine receptors (RyR), titin, and skeletal muscle antigens). The exact causes of autoimmune processes in MG are unknown, but abnormalities of the thymus gland (hyperplasia and neoplasia), especially in patients with antibodies to AchR, as well as genetic predisposition are of indisputable importance [1]. Aim. To analyze the researches of pathogenesis, diagnosis and treatment for patients with severe MG from latest literature sources to optimize the treatment of this group of patients. To analyze the own experience of implementing a patient-oriented approach to the treatment and rehabilitation of MG. Materials and methods. Bibliosemantic, comparative and system analysis methods, and clinical and neurological examination of patients with MG. Results. The course of MG is variable and can be represented by episodic muscle weakness, stationary flow, slow or rapid progression [2]. Currently, the complex therapy of patients with MG has a clear tendency to move from symptomatic (acetylcholinesterase inhibitors, potassium preparations, potassium-sparing diuretics) treatment to pathogenetic, mainly etiopathogenetic concepts of therapy are being formed (immunosuppressive therapy, antigen-specific immunotherapy, monoclonal antibodies), and it is also being clarified effectiveness of early thymectomy [3]. Conclusions. Complex therapy of patients with MG is based on the use of a differential diagnostic algorithm for various pathogenetic variants of MG, which forms a mainly etiopathogenetic concept of therapy. Antigen-specific immunotherapy, aimed at restoring tolerance to the attacked autoantigen by targeting only the damaged part of the immune system, while leaving the rest intact, is considered more promising for the treatment of MG. MG treatment should be carried out in the conditions of a specialized neurological center, where a full examination and targeted pathogenetic therapy can be carried out.

Keywords

• myasthenia gravis
• anticholinesterase inhibitors
• corticosteroids
• rituximab
• plasma exchange