[Sleep disorders in synucleinopathy]. / Narushenie sna pri sinukleinopatiyakh.

The article highlights the current state of the problem of sleep disorders in various neurodegenerative diseases. The clinical picture and diagnosis of these disorders are described in detail. Separately, the emphasis is made on the mechanisms underlying development of these disorders and their features in various forms of synucleinopathies. The mediator and physiological changes that underlie sleep disorders in various nosological units of synucleinopathies are discussed in detail. The current attitude to certain sleep disorders as predictors of neurodegenerative diseases is evaluated. The role of the glymphatic system in the development of these disorders is considered. Also, modern therapeutic strategies for sleep disorders in neurodegenerative diseases are discussed.

[Disorders of arousal: the mechanism of occurrence, neurophysiological features]. / Parasomnii, sviazannye s narusheniem probuzhdeniia iz medlennogo sna: mekhanizm vozniknoveniia, neirofiziologicheskie osobennosti.

The article covers the current state of the problem of disorders of arousal (DOAs). The clinical features and diagnostics of the disease are described in detail. Separately, emphasis is placed on the mechanisms of the occurrence of DOAs and the characteristic changes in the central nervous system. The question of neurophysiological changes preceding the clinical manifestation of DOAs and the concept of local sleep as a physiological basis for the occurrence of DOAs are considered. Current therapeutic approaches in the treatment of these forms of parasomnias are reviewed.

[Low doses of ionizing radiation-induced apoptotic nature of erythrocyte hemolysis].

It is established that exposure of the red blood cells of the rats to low doses of ionizing radiation (0.04; 0.08; 0.16; 0.25 and 0.33 mGr) leads to non-linear changes in the processes of lipid peroxidation in the membrane of erythrocytes, their electrophoretic mobility, osmotic resistance. In a dose range from 0.08 to 0.16 mGr with ionizing radiation apoptosis that determines a temporary slowing of the process of hemolysis and stabilization of erythrocytes that is confirmed by morphological changes in erythrocytes is most likely triggered.

[Efficacy and tolerability of pantogam activ in patients with partial epilepsy].

Randomized double-blind, placebo-controlled study to determine efficacy and tolerability of pantogam activ as an add-on drug in patients with partial epilepsy was conducted. 20 patients received pantogam activ, 20 patients - placebo. Good tolerability and no advers event were detected in the dose of 1800 mg/d. The reduction in the frequency of epileptic seizures was achieved in 72,1% in the group receiving pantogam activ and in 16,7% in the group placebo. Significant improvement in long-term memory and manifestations of anxiety were found in patients using pantogam activ. Quality of life in scale QOLIE-31 improved after taking pantogam and did not change after placebo.

[Lyrica (pregabalin) in the treatment of focal refractory epilepsy in adults].

Efficacy and tolerability of a new antiepileptic drug pregabalin (lyrica) as an add-on drug in patients with focal refractory epilepsy was analyzed. The most commonly used dosage was 450 mg/d. For the 6-month treatment of 19 patients, aged 18-30 years, a positive effect of the combined therapy was observed in 68,4%, the remission was achieved in 31,6% and the significant improvement in 36,8%. Drug was discontinued due to the lack of efficacy and intolerance in 31,6%. Drowsiness was the most frequent adverse event and hemorrhagic vasculitis was registered in one patient. Two cases of effective use of the drug during pregnancy with the birth of healthy children are reported. A case of the high efficacy of pregabalin in migraine-epilepsy is described. High efficacy and good tolerability as well as favorable pharmacokinetic characteristics and distinct anxyolytic effect give indications for use of pregabalin in clinical practice.

[Epilepsy and pregnancy].

Course and outcome of 200 pregnancies, deliveries and post-partum periods of 127 women with the onset of epilepsy before or during pregnancy were compared to clinical form of the disease, frequency of seizures and antiepileptic therapy. In addition, 104 children born to 97 mothers with epilepsy, 85 children--from the moment of birth to 1 year old and others from 1 to 16 years old, were studied. The particular attention was focused on the early neonatal adaptation. It was shown that, along with the last weeks of pregnancy, the first 7 days period after the delivery was critical for epilepsy severity. The development of seizures at this period was related to sleep deprivation and marked hormonal changes. There were no significant changes between the body mass of newborns of mothers with epilepsy and those of healthy mothers. The period of neonatal adaptation in children of affected mothers was favorable in 46% of cases. Some peculiarities of a child's state (withdrawal syndrome, hepatitis A etc.) were mostly related to the treatment of a mother with high dosages of antiepileptic preparations, i.e. valproic acid. These disturbances vanished to the age of 1-3 years. In summary, the active supervision of women by an epileptologist during the pregnancy period allows to optimize the prognosis of the child's health and better course of epilepsy in the mother.

Resonant two-wave mixing in photorefractive materials with the aid of dc and ac fields.

A recently predicted resonant effect for the enhancement of two-wave mixing in photorefractive materials is investigated. The resonance occurs when the frequency of the applied ac field agrees with the eigenfrequency of the excited space-charge wave. Experimentally a clear resonance is found, as predicted by the theory, for high dc electric fields, but the resonance is smeared out for lower fields. A modified theory, taking into account the second temporal harmonic of the space-charge wave, shows good agreement with the experimental results.

[Proximal muscular dystrophy with contractures and malignant course: is it a variant of Emery-Dreifuss disease?]. / Proksimal'naia miodistrofiia s kontrakturami i zlokachestvennym techeniem--variant bolezni Emeri-Dreifussa?

The authors relate a unique observation of the familial form of proximal myodystrophy with early contractures and malignant course. The primary character of muscular injury was confirmed on electromyography. The data of electrocardiography and echocardiography attested to the presence in the patients of the signs of cardiomyopathy. Since the disease was diagnosed in 3 brothers, the X-coupled recessive type of its inheritance is assumed. An opinion is advanced that the described form is a clinical variety of Emery-Dreyfus myodystrophy.

[Organization of therapeutic aid to patients with hereditary neuromuscular diseases]. / Organizatsiia lechebnoi pomoshchi bol'nym s nasledstvennymi nervno-myshechnymi zabolevaniiami.

The paper summarizes experience gained for many years by the All-Union Research Methodological Center for Study of Hereditary Neuromuscular Diseases. The specialists of the Center render counselling and therapeutic assistance to patients afflicted with neuromuscular diseases. The counselling and diagnostic services are characterized by the fact that it is based on the activity of a large hospital intended for the treatment of various diseases. The problems which are being solved by the out- and inpatient services of the Center are considered in detail. The advantages of the setting up of the common in- and outpatient complex on the basis of the hospital intended for the treatment of various diseases are described.

[Active electrogenic transport H+ in plasma membrane vesicles of cow parsnip phloem cells]. / Aktivnyi élektrogennyi transport H+ v vezikulakh plazmaticheckikh membran kletok floémy borshchevika.

Generation of electric (delta psi) and chemical (delta pH) components of electrochemical proton gradient delta muH+, in plasma membrane vesicles of Heracleum sosnovskyi phloem cells was investigated. ATP-dependent generation of delta psi at pH 6.0 in the presence of Mg2+ and K+ was established with the help of fluorescent probes AU+ and ANS-. Protonophore CCCP and proton ATPase inhibitor DCCD suppressed generation, whereas oligomycin, the inhibitor of mitochondrial ATPases did not affect it. Measurings of delta psi value indicated its oscillations within the limits from 10 to 60 mV. ATP-dependent generation of delta pH was established by means of fluorescent probe 9-AA. The effect was eliminated by CCCP and stimulated by K+, that may testify to the transformation of a part of delta psi into delta pH at antiport H+/K+. Existence of H+-ATPase in the plasma membranes of higher plant cells insuring generation of delta muH+ is supposed.