Myasthenia gravis with transformation into amyotrophic lateral sclerosis: a clinical case

The article provides a brief overview of current knowledge about the key mechanisms of pathogenesis, clinical manifestations, and approaches to the treatment of two neuromuscular pathologies: myasthenia gravis and amyotrophic lateral sclerosis. Myasthenia gravis is a chronic autoimmune disease in which the structures of the neuromuscular junction are affected. The clinical picture has a wide heterogeneity, a variety of its phenotypic variants, and symptoms range from local to generalized forms. However, the key is pathological muscle weakness and fatigue. Amyotrophic lateral sclerosis is a neurodegenerative, steadily progressive disease characterized by the death of motor motor neurons in the brain and spinal cord. This pathology is manifested by the development of muscle weakness, atrophy, severe bulbar disorders with increasing respiratory and nutritional insufficiency. The clinical case of a 60‑year-old patient with myasthenia gravis, which transformed into amyotrophic lateral sclerosis, is presented. The diagnosis is confirmed by the presence of diagnostic criteria corresponding to myasthenia gravis at the onset of the disease, their subsequent regression upon the appearance of the first symptoms of amyotrophic lateral sclerosis. Subsequently, the patient experienced a steady progression of symptoms with an increase in bulbar disorders to anarthria, severe dysphagia while taking anticholinesterase drugs and glucocorticosteroids, which required the installation of a gastrostomy. The article also provides a literature review of the frequency of occurrence, the period of development of the transformation of one disease into another both in Russia and in foreign countries. The modern understanding of the pathogenesis of this phenomenon is considered, which is associated with common immunological mechanisms and changes in the structures of the neuromuscular junction.