[Brain stem syndromes in infectious diseases of the brain].
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Adolescent↗
Biomedical subjects
G P Sushcheva
Publications and source records attributed to G P Sushcheva.
[Clinical picture, pathogenesis and geneology of hereditary progressive spinal muscular atrophies].
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.
Child↗
[Myotonic dystrophy].
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.
Adolescent↗
[Clinico-genalogic characteristics of hereditary diseases of the nervous system in the Kuibyshev region].
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.
Ataxia↗