Saratov JOURNAL of Medical and Scientific Research

The analysis of the clinical and tool parameters characterizing a cardiomyopathyat various forms of the progressing muscular dystrophies

Year: 2017, volume 13 Issue: №1 Pages: 160-164
Heading: Neurology Article type: Original article
Authors: Poverennova I.E., Zakharov A.V., Chernikova V.V.
Organization: Samara State Medical University, Samara Regional Clinical Hospital n.a. V. D. Seredavin

Purpose: studying of clinical and tool characteristics of cardiomyopathies at various forms of the progressing muscular dystrophies. Material and methods. There had been 103 patients with hereditary forms of the progressing muscular dystrophies examined, among which 35 persons were with Duchenne muscular dystrophy, 28 with an atrophic myo-tonia, and 40 with a limb girdle dystrophy is conducted. Assessment of clinical and tool features of cardiomyopathies at these types of the hereditary progressing muscular dystrophies had been carried out. Results. In the group of patients with diffusion damage of a myocardium in the form of dystrophic violations had been revealed. Existence of a negative tooth of T in some assignments and lengthenings of an interval of QT is noted. With a dystrophic myotonia violation of a warm rhythm occurred at patients by 87 times more often than in the group of comparison. Violation of a rhythm of heart in group of patients with a limb girdle dystrophy came to light 91 times more often in reference to the group of comparison. Conclusion. Violations in a cardiovascular system at Duchenne muscular dystrophy are preferentially diffusion changes in a myocardium. At a dystrophic myotonia and a limb girdle dystrophy cardial violations concern mainly excitability and the conductivity of heart which are the main reason of developing of this disease at these patients.

1. Illarionishkin SN. Molecular basis of progressive muscular dystrophies. Journal of Neurology and Psychiatry 1998, (98): 55-62
2. Sharkova IV. Modern classification, genetic heterogeneity and differential diagnosis of progressive muscular dystrophies. Medical Genetics 2015; 14 (4): 41-42
3. Polyakov AV. Genetic heterogeneity of Mendelian diseases and DNA diagnostics. Medical Genetics 2016; 15 (2): 65-66
4. Gorbunova VN. Molecular neurology. Part 1: Diseases of the neuromuscular system. SPb., 2000; 320 p.
5. Politano L. Treatment of dystrophinopathiccardiomyopathy: review of the literature and personal results. Acta Myologica 2012; (31): 24-30
6. Tamura T. Management of myocardial damage in muscular dystrophy. Brain Nerve 2011; 63 (11): 1217-1228
7. Nigro V. Genetic basis of limb-girdle muscular dystrophies: the 2014 update. Acta Myologica 2014; 33 (1): 1-12
8. Finsterer J. Arrhythmia-related workup in hereditary myopathies. J Electrocardiol 2012; 45 (4): 376-384
9. Borodulkina YuV. Clinico-molecular-genetic characteristics of frequent autosomal recessive forms of limb-limb progressive muscular dystrophies in patients from the Russian Federation. Medical Genetics 2015; 14 (2): 28
10. Baranich Tl. Limb-zone progressive muscular dystrophy type 2A (calpainopathy). The Russian Bulletin of Perinatology and Pediatrics 2015; 60 (4): 69-74
11. Osetrov AS, Malkova АА, Osipova EV, et al. Neuromuscular diseases: a clinic, diagnostics, treatment. Izhevsk, 2014; 76 p.
12. Groznova OS, Rudenskaya GE, Adyan ТА. Defeat of the heart in hereditary neuromuscular diseases in children. The Russian herald of perinatology and pediatrics 2014; 59 (2): 35-42
13. Cotta A. Common recessive limb girdle muscular dystrophies differential diagnosis: why and how? Arq. Neuropsiquiatr2014;72(9):721-734. 14. Ergul Y, EkiciB, Nisli K. Evaluation of the North Star Ambulatory Assessment scale and cardiac abnormalities in ambulant boys with Duchenne muscular dystrophy. J Paediatr Child Health 2012; (10):1440-1754.

2017_01-1_160-164.pdf239.35 KB

No votes yet