Vol XXIX, No 1-2 (1997)

The outstanding role in development of home and the world neurology by professor V.M. Bekhterev, having founded the basis of the modem integrative science of the nervous system is shown. The purpose of this work is to awake interest of the medical society to the personality of V.M. Bekhterev, a founder of the Kazan neurological school, a scientist and a doctor, to his works of the Kazan period of his activity.

There given the results of brain imaging methods (CT and MRT) in posttraumatic epilepsy. On the background of complex investigation (EEG, CT and MRT) it was shown that imaging methods significantly replenish information on localization and character of focus, especially in heavy craniocerebral injury, the MRT method being more informative. Changes, reflected by CT are revealed in 68% of patients (32% — focal), while changes by MRT — in 64% of patients (52% — focal). To achieve optimal results it is necessary to complexly use EEG and imaging methods, for it is important for making a prognosis, improving conservative and surgical treatment of patients.

There discussed and systematized different diagnostic mistakes in patients, suffering epilepsy and nonepileptic paroxysmal conditions at early stage of disease. There given the analysis of clinical and paraclinical examination data of 525 patients. Decisive factors in diagnosis are revealing basic clinical symptocomplex and its analytical understanding in comparsion with the findings of investigation methods, conducted by special devices.

It was studied the content of endogenic convulsive agent, kynurenine, in the blood and liquor of 57 epileptic patients. It was revealed raising of kynurenine level in the blood of epileptic patients as compared with the level of control group, as well as more high level of kynurenine in the blood of patients with frequent seizures and lowering its level in liquor just after paroxysm. The abovesaid confirms the certain role of kynurenine in the pathogenesis of epilepsy.

Results of immunological investigation of antibody level to galactocerebrosides in children with paroxismal disturbances of consciousness of different severity and in children of control group, as well as in mothers of the ill and healthy children are presented. It has been determined that level of antibodies related to galactocerebrosides correlates with the severity of the course of epileptic paroxisms in children. In all children with nonepileptic paroxysms titers of antibodies related to galactocerebrosides coincide with age-dependent estimated data. Titers of antibodies related to galactocerebrosides of diagnostic value are absent in all the women whose children suffer from nonepileptic as well as benign epileptic paroxysms. The obtained results in conjunction with clinical biochemical findings allow to carry out early diagnosis of severe forms of epileptic paroxisms in children, to prognose the course of disease and promote to timely administration of pathogenetic treatment.

Results of complex clinical electrophysiological examination of 110 patients with temporal epilepsy, who underwent implantation of electrodes into different brain structures for diagnostic and therapeutic purposes, are presented. It has been found that limbico-temporal structures at the moment of sezure development are functioning as united functional system with predominant involvement of amigdala into ep. ileptogenesis. It is illustrated, that low-frequency electrostimulation of neuronal structures of temporal lobe, surrounding epileptic foci, can exert antiepileptic clinical electrophysiological effect.

Excretion of catecholamines indiumal urine of 75 children with febrile convulsionns has been studied. It has been revealed that functional state of sympathetic adrenal system in children with febrile convulsions is characterized by a number of peculiarities. They include an increase of adrenaline and norepinephrine. In the case of transformation of febrile into afebrile seizures additional increase of dopamine excretion is observed. That’s why hypersecretion of the three mentioned amines is estimated as prognostically unfavourable factor of the course of febrile convulsions. According to our findings, progression of seizures in sleeping state is also prognostically unfavourable symptom of the course of febrile seizures. The obtained biochemical ringings together with clinical information can serve as prognostic criteria in solving the problem of variations of the course of febrile convulsions.

For treating active forms of secondarily-progressing multiple sclerosis a preliminary clinical trial was performed by two courses: antibody-to-cytokine course (γINT, αINT and TNFα) and cyclosporin А-course (sandimmun). Data of preliminary test of antibodies to cytokines evidence to possibility of performing further clinical trials "antibodies to γINT and to TNFα (to a lesser degree)" during active forms of progressive MS. Were obtained the results, evidencing to a possible use of sandimmun in constantly progressive forms of secondarily progressing disease, when favourable clinical effect coincided with supression of cytokine IL-2 production and lowering of activeness of immunocytes.

85 children with diphtheria complicated by diphtheric polyneuropathy (DPN) underwent clinical-instrumental examination. The dependence of incidence of DPN progression on the degree of primary intoxication has been determined. In toxic forms characterized by prolonged immunosuppression, multiwave course and in local forms onewave course of DPN takes place.

There studied changes of electroneuiomyographical indices for evaluation of incidence and dynamics of lesion of peripheral nervous system during diphtheria. For this purpose 37 patients with diphtheria polyneuropathy at the age of 2—14 years at different terms of disease were examined. It was shown that during diphtheria most often the nerves of lower extremities are damaged: fibular and tibial. Information of ENMG indices of the latter is more significant. For revealing the initial symptoms of the nervous system lesion it is useful to investigate the state of myelinization of proximal parts of peripheral nerves according to F-wave latency.

The paper is concerned with a case history of a girl with a rare combination of 2 rare anomalies — monosomia by X chromosome and Duchenne's muscular dystrophy. Mosaicism 45,X/46,XX in a girl with a mild picture of Duchenne's muscular dystrophy was confirmed by a study of the kariotype in lymphocytes of the peripheral blood.

In the Republic of Bashkortostan computer data bank on myotonia dystrophica (MD) was created where information on all diagnosed patients and members of their families was entered. For the first time in the republic qualitative molecular genetic RNA-diagnosis of MD started, with the help of which one phenotypically healthy mutation carrier is revealed. Clinical genetic characteristic of MD in Bashkortostan and main subpopulation of the republic is presented. Besides neuromuscular symptoms cerebral and extraneural symptoms are analyzed.

Time parameters of locomotor functions are studied by computer analysis of walking phase with the aim of correcting pace-rhythmic structure of locomotor act, disturbed in conditions of organic brain pathology. As a result of comparison with average statistical norm, time frequency was selected and 83 offered to patient by functioning of portable photophonostimulator as the main walking speed. As a result of using this method (referential bioadaptation) in 7 patients parameters of locomotor functions significantly approximated to the norm. This approach is considered promising for locomotor rehabilitation in neurology.

On the basis of study of results of exteroceptive suppression parameters in 140 patients with different variants of facial hyperkineses the state of reflex inhibitory neuronal chains was defined more precisely. Received data may be used in process selection and monitoring of therapy and promote to understanding of facial musculature dyskinesia forming mechanisms.

Currently, epilepsy is one of the most pressing problems in pediatric neurology. The incidence of epilepsy in the pediatric population ranges from 0.5 to 0.75%. The problem of epilepsy is a problem of accurate syndromological diagnosis and treatment. The modern classification of epilepsy, epileptic syndromes and similar disorders distinguishes about 40 different forms of epilepsy, differing in clinical symptoms, principles of therapy, prognosis.