LECTURES AND REVIEWS
Many inherited neuromuscular disorders include cardiac involvement as a typical clinical feature. Among the most common of them is the group of muscular dystrophies. Dilated cardiomyopathy, ventricular arrhythmias, atrial fibrillations, atrioventricular and intraventricular conduction abnormalities, and sudden cardiac death are well known pathological findings in Duchenne muscular dystrophies, myotonic dystrophy type I and 2, Emery-Dreifuss muscular dystrophies and different types of limb-girdle muscular dystrophies and other disorders. Detection of cardiac pathology in patients with different muscular dystrophies is possible with ECG, echocardiography and cardiovascular magnetic resonance imaging, which are recommended for screening and early cardioprotective treatment.
Current publication summarizes main indications and benefits of intravenous high-dose immunotherapy (IHI) in the treatment of various autoimmune diseases of the peripheral nervous system. Available products of intravenous immunoglobulin (IVIG) on the Russian market are reviewed. Tactics for choosing optimal medication for IHI based on its effectiveness and safety are analyzed. Dosage calculation and way of administration of IVIG are described, beeing of a high practical value in neurologist’s daily work.
Chronic cerebral ischemia is a common problem especially among the elderly. It gradually leads to social disability and invalidisation of those patients. One of the possible pathogenic mechanisms of this condition is the endothelial dysfunction. Thus, the attempt to repair the latter is one of the treatment goals. It is assumed that actovegin improves tissue microcirculation by expanding precapillary zone – the main marker of the metabolic activity in the microvascular endothelium. Actovegin activates anaerobic metabolism and oxygenation in endotheliocytes. Patients, treated by actovegin, showed improvement of cognitive functions and psycho-emotional state. Due to the absence of proteins, the drug has good tolerance and low toxicity.
Relying on a comprehensive investigation, the author proposes the typology of depressive disorders in epilepsy by identifying ictal, preictal, interictal, and postictal conditions. Risk factors for depressive symptoms are considered. Main approaches to therapy for depressions are offered. Particular emphasis is placed on the normothymic activity of a number of anticonvulsants. Valproates, and depakine in particular, as a normothymic agent are shown to be beneficial in treating affective disorders.
CLINICAL DISCUSSION
Fabry disease is an X-linked, lysosomal storage disease (OMIM: 301500), caused by α-galactosidase A deficiency, resulting in accumulation of its substrates, glycosphingolipids, primarily – globotriaosylceramide, in the lysosomes of multiple cell types with multi-system clinical manifestations, even within the same family, including abnormalities of the central and peripheral nervous system, kidneys, heart, gastrointestinal tract, lungs, organ of vision. Clinical heterogeneity is often the reason of the delayed diagnosis. Nowadays enzyme replacement therapy has proved its efficiency in the treatment of Fabry disease. Including Fabry disease in the differential diagnosis of a large range of disorders is important because of its wide clinical heterogeneity and the possibility of an earlier intervention with a beneficial treatment.
CONFERENCES, SYMPOSIUMS, MEETINGS
ISSN 2413-0443 (Online)