[Study of the practice of prescribing phenosanic acid in epilepsy accompanied by asthenic disorders]. / Izuchenie effektivnosti primeneniya fenozanovoi kisloty pri epilepsii, soprovozhdayushcheisya astenicheskimi narusheniyami.

OBJECTIVE: To evaluate the experience of prescribing phenosanic acid in the practice of a neurologist/epileptologist when prescribing the second, third anticonvulsant drug (AED) as part of combination therapy for patients with manifestations of fatigue due to epilepsy. MATERIAL AND METHODS: 501 patients with focal epilepsy accompanied by asthenic disorders were included in the observational program. The observation program protocol included 5 visits, including visit 1, at which screening and inclusion in the OP took place. The observation period was 10 months. At baseline and at the end of the 10-month follow-up, the patients' condition was assessed according to the following indicators: frequency and transformation of attacks with focal onset, severity of fatigue (self-assessment scale MFI-20); quality of life (questionnaire QoLiE-10-P); frequency of attacks with focal onset. The safety of phenosanic acid (Dibufelon) was also assessed. RESULTS: In 10 months after the inclusion of Dibufelon as the 2nd, 3rd AED in the treatment regimen, a statistically significant (p<0.01) decrease in the frequency of seizures was observed: in general - in 88% of patients; by 50% or more - in 76% of patients; transition from the group with a large number of seizures to the group with a smaller number of seizures - 74% of patients. Also when taking phenosanic acid, a positive dynamics of seizure type was noted: a reliable decrease in the proportion of patients with seizures with secondary generalization from 70% to 56%; a decrease in the number of focal seizures with impaired consciousness from 65% to 53%. In addition, there was a 38% decrease in the severity of fatigue on the MFI-20 scale (the greatest decrease on the «Mental fatigue¼ scale), improvement in the quality of life - a 2.7-fold increase in the mean values of the QOLIE-10 questionnaire. CONCLUSION: The addition of phenosanic acid to antiepileptic therapy as a second or third AED allows for better control of seizures, leading to a decrease the frequency and severity of attacks and the severity of fatigue both, and an increase of the quality of life of patients with epilepsy.

[Epilepsy syndromes associated with hearing loss]. / Epilepticheskie sindromy, assotsiirovannye so snizheniem slukha.

Patients with epilepsy who have also hearing loss represent a distinct group of patients, often with aggravated medical history, comorbidities and high potential for disability. The etiopathogenetic factors of epilepsy and hearing loss may be common to these conditions (neuroinfections, craniocerebral injuries, cerebral circulatory disorders, perinatal pathology, etc.). In addition, these two syndromes may occur as part of hereditary diseases, so their timely recognition and genetic diagnosis are important for determining further medical and genetic prognosis. This article provides an overview of orphan genetic diseases associated with epilepsy and hearing loss - MERRF syndrome, MELAS syndrome, EAST syndrome, Ayme-Grippsyndrome, epilepsy, hearing loss and mental retardation syndromes, associated with mutations in SPATA5 gene, DOOR syndrome, Gustavson syndrome.

[COVID-19-associated damage of the peripheral nervous system]. / COVID-19-assotsiirovannye porazheniya perifericheskoi nervnoi sistemy.

Based on the available literature data, the article discusses the prevalence of various forms of damage of the peripheral nervous system in COVID-19 and in the post-COVID period. Information about the clinical features and the course of individual cranial neuropathies, chronic dysimmune neuropathies, Guillain-Barré syndrome, drug-induced neuropathies, fine fiber neuropathy, myasthenia gravis and polyneuropathy of critical conditions was systemized in the context of coronavirus infection. SARS-CoV-2 can trigger various stages of pathogenesis, including neuroimmune ones, which cause long-term consequences of COVID-19, including those associated with the damage of the peripheral nervous system. Awareness of COVID-19-associated pathological conditions will allow assessment of the possible risks of damage of the peripheral nervous system, recognize them at early stages and develop more effective approaches for treatment.

[Results of 3 years work of the Russian hospital register of chronic heart failure (RUssian hoSpital Heart Failure Registry - RUS-HFR): relationship between management and outcomes in patients with chronic heart failure].

AIM: To analyze management and outcomes in patients with CHF managed by specialists in heart failure (HF) or general cardiologists/physicians in real-life clinical practice. MATERIALS AND METHODS: Survival rate, rehospitalization rate, general health condition, and the administered therapy were evaluated for HF patients with reduced LV ejection fraction at three years of discharge from cardiological hospitals. These patients had been included in a prospective, multicenter, observational study, "The Russian Hospital HF Registry" (RUS-HFR). The first group consisted of patients who were managed at a specialized HF department of the Federal Center and followed up at the outpatient stage by a cardiologist specializing in HF (Group 1, St.­Petersburg; n =74). The other two groups (Group 2 and Group 3) included patients who were managed at other cardiological departments of the Federal Center (n=186) or the Regional Center (n=130) and subsequently followed up at the place of residence. RESULTS: After the discharge from the hospital, 58-95 and 12-19% of RUS-HFR patients were followed up by a cardiologist or a physician, respectively, on an outpatient basis while 5-23% of patients did not visit a doctor at all. In three years, the survival rate of Group 1, 2, and 3 patients was 80 vs. 78 (р>0.05) vs. 52% (р0.05) vs. 100% (p1,2.

[Results of Russian Hospital Chronic Heart Failure Registry in Three Subjects of Russian Federation].

Aim of the RUssian hoSpital Heart Failure Registry (RUS-HFR) was to analyze clinical and epidemiological characteristics, application of modern drug and non-drug therapy among hospitalized patients with systolic chronic heart failure (CHF) in 3 subjects of Russian Federation. MATERIAL AND METHODS: We conducted a prospective multicenter observational study in 3 cardiological centers (Petersburg, Orenburg, Samara) in which we recruited patients aged 18-75 years with left ventricular ejection fraction less or equal 40% without ongoing myocardial infarction or pulmonary thromboembolism. RESULTS: Between October 2012 and June 2014 we included 524 patients (mean age 60 years, >79% men) mostly with NYHA class III CHF. Main etiological factors were ischemic heart disease and arterial hypertension, which was not corrected by the time of admission in 20-40.7% of patients. Portions of patients who had undergo the myocardial revascularization, surgery because of valvular heart defects, and implantation of permanent pacemaker including device for cardiac resynchronizing therapy were 11-41, 0-4, and 0-19.5%, respectively. In up to 25% of patients therapy with one of main medications or diuretic was initiated after hospitalization. At discharge 82.3-8.3, 76.3-95.8, 65.9-81.1, 80.7-94.6% of patients received ACEI/ARB, -adrenoblockers, MRA, diuretic therapy, respectively. Implantation of cardioverter ICD/CRT, ICD and heart transplantation were considered indicated in 0-21.2 and 0-6.6% of patients, respectively. Duration of hospitalization was 26+/-14, 15+/-10, and 13+/-5 bed-days in Petersburg, Orenburg, and Samara, respectively. CONCLUSIONS: Compared with data obtained 10 years earlier portrait of a patient with in general remained unchanged. However portion of patients who had undergone cardio-surgical and electrophysiological interventions became tangible, and numbers of patients receiving therapy with ACEI/ARB, -adrenoblockers, and MRA became comparable with those in European registries. Significant deviations from guidelines on drug therapy were revealed at prehospital stage; and high-technology methods of therapy were insufficiently often recommended in participating hospitals. Portion of hospitalizations not related to acute decompensation was 5-37%, while duration of such hospitalizations as well as hospitalizations because of acute decompensation of CHF significantly exceeded this parameter in EU and USA. Absence of obligatory drug therapy prior to admission in a part of patients in RUS-HF raises questions on correction of patients compliance and on creation of a system of specialized ambulatory surveillance of CHF patients after discharge from hospital.