EVALUATION OF HUMAN IMMUNOGLOBULIN EFFECTIVENESS IN PATIENTS WITH SENSORY-MOTOR POLYNEUROPATHY ASSOCIATED WITH MONOCLONAL GAMMAPATHY OF UNDETERMINED SIGNIFICANCE

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Chronic inflammatory demyelinating polyneuropathy (CIDP) is a peripheral neuropathy, predominantly motor neuropathy, with a progressive or relapse-remitting course. Fatigue is a condition characterized by a physical or mental feeling of lack of energy or lack of motivation for action, which is often present in patients with CIDP.Objective: to assess the severity of asthenia in CIDP patients.Patients and methods. Examinations were made in 34 inpatients treated for documented CIDP that met the international European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria. A study group included patients with CIDP, whereas a comparison group consisted of volunteers without psychiatric illness, who were compensated for somatic diseases.Results and discussion. In the patients with CIDP, the level of fatigue was found to be much higher than normal. Approximately half of the CIDP patients had obvious asthenia. However, the level of fatigue did not correlate with the severity of the course of CIDP.Conclusion. The findings suggest that fatigue is important in patients with CIDP that should be taken into account in the treatment of these patients.

Charcot — Marie — Tooth disease belongs to a genetically heterogeneous group of monogenic diseases with a predominant lesion of the peripheral nervous system associated with predominant degeneration of the myelin sheath and/or axon of the motor and sensory nerves and spinal roots, accompanied by motor disorders and specific pain syndrome. The article presents a description of 2 clinical cases of patients (father and daughter), whose disease was manifested by chronically progressive weakness and atrophy of the distal limb muscles, reflexes reduction, foot and hand deformation, gait changes and sensory disorders. Genetic tests were performed to detect duplication/deletion of the PMP22 gene and the expansion of GAA repeats in the FXN gene, which yielded negative results. Based on complaints, neurological examination, family medical history and data of instrumental research (EMNG), we made a clinical diagnosis of the CMT with an autosomal dominant type of inheritance and a high degree of penetrance. At the department, patients received the infusion therapy (neurometabolic therapy, vitamin therapy), physical therapy courses according to an individual plan, physiotherapy and reflexotherapy with a moderate positive effect in the form of a subjective increase in muscle strength in the lower extremities, increasing walking stability and confidence. The article also highlights the peculiarities of the pathogenesis of different genetic variants of CMT and prospect for pathogenetic treatment of this disease.