[Vegetative and trophic disorders and functional status of the sympatho-adrenal system in Rossolimo-Curschmann-Batten-Steinert myotonic dystrophy]. / Vegetativno-troficheskie narusheniia i funktsional'noe sostoianie simpatiko-adrenalovoi sistemy pri miotonicheskoi distrofii Rossolimo--Kurshmana--Battena--Shteinerta.

In a comprehensive examination of 16 patients with the Rossolimo-Curschmann-Steinert-Batten myotonic dystrophy the authors determined catecholamine levels in the adrenergic structures of skeletal muscles, levels of adrenaline and noradrenaline in the blood and their daily excretion with the urine. The findings showed elevated concentrations of catecholamines in the adrenergic structures of skeletal muscles which suggests an intensified diffusion of catecholamines from adrenergic structures into the adjacent muscular tissue. The sympathoadrenal system functioned under stress. Myotonic dystrophy was associated with disturbances of both central and peripheral mechanisms of vegetative regulation of muscular activity.

[Catecholamine concentration in the blood and excretion in the urine in different forms of progressive muscular dystrophy]. / Soderzhanie katekholaminov v krovi i ikh ékskretsiia s mochoi pri razlichnykh formakh progressiruiushchikh myshechnykh distrofii.

A combined study of the blood content of catecholamines (C) and of their urinary excretion in 42 patients with primary forms of myodystrophies and denervated amyotrophies allowed adequate identification of the features of the sympathoadrenal system functioning under conditions of the myodystrophic process. The study revealed changes in C levels. The primary forms of progressive muscular dystrophies (PMD) were associated with increased blood levels of adrenalin (A) while denervated amyotrophies were related to elevated concentrations of noradrenalin (NA). A statistically significant increase in A excretion with urine was found in Duchenne's PMD and in Kugelberg-Welander's spinal amyotrophy, i. e. the disorders with the most malignant course among all the groups studied. NA concentrations were lowered in all forms of PMD with the exception of Charcot-Marie's neural amyotrophy.

[Fluorescence histochemical study of catecholamine concentrations in the skeletal muscles of patients with different forms of progressive neuromuscular disease]. / Fliuorestsentno-gistokhimicheskoe issledovanie soderzhaniia katekholaminov v skeletnoi myshtse u bol'nykh s razlichnymi formami progressiruiushchikh nervno-myshechnykh zabolevanii.

The content of catecholamines was examined in the skeletal muscle of 16 patients with progressing muscular dystrophies (PMD) with the fluorescent histochemical method of Folck-Hillarp modified by V.N. Shvalev and N. I. Zhuchkova. Of the 16 patients 4 were suffering from Becker's PMD; 3, from Erb-Roth's PMD; 1, from Dreifus PMD; 3, from Kugelberg-Welander's spinal amyotrophy; 3, from Charcot-Marie's neural amyotrophy; 2, from Roussy-Levy's syndrome; and 2 from Curschmann-Steinert-Batten's myotonic dystrophy. The skeletal muscles of patients hospitalized at urological and orthopedic divisions served as control. Considerable accumulations of catecholamines were revealed in the skin, subcutaneous fat, and loose amorphous connective tissue surrounding the muscular fibres in the patients with PMD and myotonic dystrophies. In the patients with denervation amyotrophies a sharp fall of the fluorescence intensity with a reduction of the number of fluorescent fibres, their thinning, and diminution of the size of varicose thickenings were revealed.