[Non-invasive ventilation of the lungs in neuromuscular diseases]. / Neinvazivnaya ventilyatsiya legkikh pri nervno-myshechnykh boleznyakh.

OBJECTIVE: To describe a clinical case and to analyze our own practice of using NIVL in a myasthenia (MG) gravis patient. MATERIAL AND METHODS: Since 2018 in the Republican Research and Clinical Center of Neurology and Neurosurgery NIVL has been performed in 29 patients (21 amyotrophic lateral sclerosis patients and 8 MG patients). The research was carried out using the portable polysomnograph Polymate YH-1000C (BMC, China) and in the Sleep Laboratory of the Republican Clinical Medical Center of the Presidential Administration of the Republic of Belarus using SOMNOlab V 2.19 (Weinmann, Germany). Respiratory support was provided by the Ventimotion 2 device (Weinmann, Germany). RESULTS: The article presents our own experience of using NIVL in MG patient and profound description of the diagnostic and therapeutic complex. The development of chronic respiratory failure in NMD is based on a violation of the ventilation-perfusion ratio in the alveoli as a result of the development of hypoventilation due to restrictive disorders (namely, due to weakness of the respiratory muscles). Compensatory mechanisms eventually lead to an increase in the work on the affected respiratory muscles that leads to the formation of a vicious circle. The use of NIVL provides adequate ventilation of the lungs providing rest for the respiratory muscles. Like any other medical intervention NIVL has indications and contraindications, advantages and disadvantages that are described in this article. CONCLUSION: The use of NIVL helps to reduce the risk and frequency of respiratory complications, the number and duration of hospitalizations that significantly affects the prognosis and course of NMD as well as improves the quality of life and the level of adaptation of patients.

[Psychogenically induced narcolepsy]. / Psikhogenno-indutsirovannaia narkolepsiia.

Based on own clinical experience in diagnostics and treatment of 4 patients, the authors describe main clinical presentations of narcolepsy. A case report of a 20-year female patient with psychogenic narcolepsy induced by a conflict in the family is described in details. According to polysomnography and Multiple Sleep Latency Test, a reduction in latency to sleep is 22 sec - 3 min 30 sec and the time of occurrence of REM sleep is 7 min 30 sec. The diagnosis is made on the basis of diagnostic criteria of narcolepsy. On average, it takes about 7-10 years to diagnose narcolepsy that reduces the quality of life of patients and leads to social and household maladaptation. Physicians should be wary of early identification of this disease for dynamic monitoring, the appointment of symptomatic therapy, prevention of anxiety and depression and the generation of behavior algorithm in the patient and his relatives in order to maximize social and household adaptation.

[Neurospecific proteins in cerebrospinal fluid and in the bloodserum of patients with amyotrophic lateral sclerosis]. / Neirospetsificheskie belki v tserebrospinal'noi zhidkosti i syvorotke krovi u patsientov s bokovym amiotroficheskim sklerozom.

AIM: To determine concentrations of neurospecific enolase (NSE) and S100b protein in bloodserum and cerebrospinal fluid (CSF) in patients with amyotrophic lateral sclerosis (ALS) with different forms (onset) and duration of ALS. MATERIAL AND METHODS: Concentrations of NSE and S100b in serum and CSF were studied in 86 patients with different forms and duration of ALS. RESULTS AND CONCLUSION: Concentrations of NSE and S100b protein in CSF were significantly higher in the group of patients with bulbar form and in the group with duration of disease less than 1 year compared to the control group. There was a negative correlation of S100b protein in CSF with ALSFRSR score in the group of patients with bulbar ALS. An increase in NSE concentration in CSF and serum in patients with cervico-thoracic form of ALS and in patients with disease duration from 1 to 4 years was found. Concentrations of protein S100b remained the same. A significant decrease in the concentration gradient of S100b protein (CSF/serum) in patients with disease duration more than 1 year suggests the disturbances of blood-brain barrier permeability with increasing ALS duration and confirms the role of these changes in the pathogenesis of the disease.

[Polysomnography in patients with amyotrophic lateral sclerosis].

OBJECTIVE: To study the characteristics of nocturnal sleep and reveal breathing disorders during sleep in ALS patients using polysomnography. MATERIAL AND METHODS: The study included 29 patients with the established diagnosis of ALS, 17 women and 12 men, median age 65 [59; 68] years; mean illness duration 12 [22.9; 27.1] months. The control group consisted of 46 volunteers without complaints of sleep disorder and sleep apnea. The sleep diagnostic system Somnolab 2 Weinmann, Germany was used. RESULTS AND CONCLUSION: An increased level of awaketime and a significant decrease in amount of deep sleep and REM sleep were revealed in ALS patients. Sleep breathing disorders are found significantly more often in ALS patients, mainly as alveolar hypoventilation syndrome and less frequently as obstructive sleep apnea. The lower level of mean and minimum blood oxygen saturation and increased respiratory rate were detected. These changes are possibly due to the presence of restrictive respiratory disorders. These disturbances reduce the total duration of sleep, destroy it's structure, exerting a direct influence on the life quality in ALS patients, disrupting their domestic and social activity, contributing to the development of neuropsychological and behavioral disorders.

The Use of Autologous Mesenchymal Stem Cells for Cell Therapy of Patients with Amyotrophic Lateral Sclerosis in Belarus.

We studied a new method of treatment of amyotrophic lateral sclerosis with autologous mesenchymal stem cells. Autologous mesenchymal stem cells were injected intravenously (intact cells) or via lumbar puncture (cells committed to neuronal differentiation). Evaluation of the results of cell therapy after 12-month follow-up revealed slowing down of the disease progression in 10 patients in comparison with the control group consisting of 15 patients. The cell therapy was safe for the patients.