[Brain stem syndromes in infectious diseases of the brain].
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Biomedical subjects
Publications and source records attributed to L N Nesterov.
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A clinical-genealogical and electromyographic investigation of 142 patients with spinal muscular atrophies demonstrated a heterogeneous nature of spinal amyotrophies. A group of amyotrophies was specified as determined by the degeneration of only motor cells of the anterior corns. This group includes Werdnig-Hoffmann's infantile spinal amyotrophy, late childhood spinal amyotrophy, Kugelberg-Welander's juvenile amyotrophy, and late distal spinal amyotrophy. The group of spinal neural amyotrophies is made up of the clinical variants which are characterized by the parallel involvement of spinal motor cells and their axons. Spinal neural amyotrophies include the autosomal-recessive childhood and the autosomal-dominant juvenile variants.
Clinicogenealogical examination of 63 patients with myotonic dystrophy as well as the electromyographic and muscular biopsy findings showed this disorder to represent a distinctive clinical form rather than a variant of Thomsen's myotonia. Considerable clinical inter- and intrafamilial polymorphism of myotonic dystrophy was revealed. The characteristics of the disease among the population of the Kuibyshev region are provided, including the description of its specific manifestations in Ukrainian and Russian families. Incomplete penetration of the myotonic dystrophy gene was elicited, with 83% in the Ukrainian, and 91% in the Russian families.
Permanent and paroxysmal vegetative disorders were detected in 68,8% of the cases with temporal brain tumors. The intensity of the vegetative disorders was determined by intralobal localizations of the tumor, by the phase of the tumorous process and the histological structure of the neoplasm. Electrophysiological (rheographical) studies of cardiovascular changes detected in most cases marked to a varied extent regional cerebral and systemic disturbances of hemodynamics. Analysis of the cardiac activity demonstrated an increase in the physiological asynchronism of the systolic phases in the right and left ventricles, due to a pronounced disturbance of the right ventricle contractility.
The report contains data of a clinico-genealogical analysis of 450 observations of hereditary diseases of the nervous system, and the prevalence rates of neurohereditary diseases in the Kuibyshev region. The authors stress the significance of the founder effect as a factor lying at the basis of a concentration of autosome-dominant forms in some of the areas of the region. The role of increased inbreeding in the enlargement of the amount of autosome-recessive forms is being confirmed. The results of the study denote that in the population of the studied region the group of nervous-muscular hereditary diseases is most frequent. The main neurohereditary diseases are being clinically defined with an indication of the type of hereditary transmission. The authors underline the significant clinical intra- and inter-familial polymorphism of such diseases as the Charcot-Marie-Tooth neuronal amyotrophy, scapulohumeral-facial myopathy of Landusi-Dejenrinne, primary pelvic-humeral progressive muscular dystrophy, autosoma-dominant myatrophic ataxia, myotonic dystrophy. The authors indicate the necessity of a screening of patients with hereditary diseases of the nervous system.
The study is based on the analysis of 10 cases of sarcomatosis of the brain and spinal pia mater. A progressive development of the disease, cachaxia, the existence of focal symptoms, radicle pain, xantochromia in the CSF, protein-cell dissociation and some other signs are characteristic of primary sarcomatosis of the pia mater. On the basis of clinical symptoms and CSF changes it is possible vitaly to perform a differential diagnosis with chronic infectious diseases and especially with tubercular meningitis and polyradiculomyelitis. Pathomorphological studies revealed tissue infiltration of the surrounding intracerebral vessels by tumors cells without proliferation into the dura mater and radicles of the spinal cord.
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The authors studied 600 cases of supratentorial tumors of the brain. The clinical picture in 306 of them (51%) was marked by epileptic seizures. They analysed the frequency of various types of epileptic seizures according to the localization and histological structure of the tumors. On basis of the results of complex clinico-electrophysiological examination a conception of the formation of the epileptic system and some pathogenetic mechanisms of epileptic seizure development in brain tumors is suggested.
The clinico-pathophysiological analysis of the main forms of extrapyramidal dyskinesias is discussed. The authors distinguish 2 main forms of hyperkinesias and dystonias which are designated conditionally phasic and tonic. Stereotaxic interventions on deeply located brain structures (ventrolateral complex of thalamic nuclei, subthalamic region, putamen, dentate nuclei of cerebellum) are substantiated anatomicofunctionally. On the basis of the results of 569 stereotaxic operations the indications and contraindications for various types of operative interventions were elaborated. The result was good in 26.7% of patients, considerable improvement occurred in 32%, mild improvement in 32.6%, no changes occurred in the condition of 5.2% of patients.
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The results of a combined electro-physiological study of cerebral circulation, peripheral circulation and cardiac activity in 100 patients with supratentorial tumors of the brain were examined. Regional-cerebral and systemic hemodynamic disorders increase with the development of intracranial hypertension and in a definite way depend on the histological structure of the new growth and the effect of the median brain structure.