Myasthenia gravis in children (clinical case)

K.A. Voroshirina, E.V. Grankin

Regional Children’s Clinical Hospital, Orenburg

Myasthenia gravis is an autoimmune disease accompanied by a neuromuscular transmission disorder. The clinical picture in this disease is represented by a transient or increasing weakness of the skeletal (striated) muscles of the face, trunk and limbs. The article describes a clinical case of a 13-year-old male patient with a generalized rapidly progressive form of myasthenia gravis. Presumably, in the setting of an acute respiratory infection, the patient developed generalized weakness, bulbar disorders with the flaccid tetraplegia development, respiratory arrest with a transfer to an artificial lung ventilation (ALV). A diagnostic search was conducted concerning inflammatory, infectious and neoplastic lesions of the spinal cord and brain, and other diseases characterized by the neuromuscular transmission disorder. According to the MRI results of the cervical spine, examination of the cerebrospinal fluid, electron euromyography, determination of acetylcholine receptor antibodies, no pathology was found. Treatment with the use of specific immunotherapy with high doses of human IgG, as well as cholinesterase inhibitors, provided positive dynamics in the form of spontaneous breathing restoration, regression of bulbar disorders, and an increase in the movement volume of the extremities. Neurological symptoms completely regressed during regular use of cholinesterase inhibitors. Since myasthenia gravis is characterized by clinical polymorphism of symptoms and the absence of specific changes according to the study results, the doctor needs to know the characteristics of the disease clinical course. It will timely allow to establish the correct diagnosis and prescribe effective treatment.

Keywords: myasthenia gravis, generalized weakness, bulbar disorders, acetylcholine receptor antibodies, cholinesterase inhibitors, immunosuppressive therapy.

For citation: Voroshirina K.A., Grankin E.V. Myasthenia gravis in children (clinical case). RMJ. 2021;5:55–58.