ABSTRACT
Data of histological and histochemical examinations of 85 biopsy specimens of muscular tissue taken from patients with hereditary muscular and neuromuscular diseases are presented. The diseases were: the extremity-and-lumbar form of myopathy congenital myopathies, Charcot-Marie's neural amyotrophy, Steinert-Batten's atrophic myotonia, and other rare muscular diseases. Use was made of histochemical methods of determining the activity of the enzymes of the Krebs cycle and glycolysis. The data obtained are compared with the clinical peculiarities of each disease.
Subject(s)
Muscles/enzymology , Muscular Diseases/enzymology , Neuromuscular Diseases/enzymology , Charcot-Marie-Tooth Disease/enzymology , Dihydrolipoamide Dehydrogenase/metabolism , Glycogen Storage Disease Type V/enzymology , Histocytochemistry , Humans , L-Lactate Dehydrogenase/metabolism , Muscular Dystrophies/enzymology , Myotonic Dystrophy/enzymologySubject(s)
Muscles/pathology , Muscular Dystrophies/pathology , Adolescent , Adult , Biopsy , Female , Humans , Male , Muscles/metabolism , Muscular Dystrophies/genetics , Muscular Dystrophies/metabolism , SyndromeABSTRACT
In 77,9 +/- 3,5% of the children with an active phase of rheumatic fever, developing with a prevalent lesion of the heart and joints, symptoms of animal and vegetative nervous system lesions were found. The neurological symptoms correlated with changes of the brain bioelectrical activity. The authors point to some dynamics of symptoms under the influence of anti-rheumatic treatment. Normalization of the brain bioelectrical activity, a regress of symptoms of the vegetative nervous system and muscular hypotonia are somewhat behind the clinical recovery. The existence of pathological activity on the EEG in children after an arrest of clinical symptoms of rheumatic fever may indicate to the possible subsequent development of chorea, the diencephalic syndrome, cerebrasthenic syndrome in these children.