Abstract
Autonomic Nervous System (ANS) function has been assessed in several muscular dystrophies. In the last two decades it has been recognized a significant relationship between ANS and cardiac mortality (Politano et al., 2008). Cardiovascular ANS is frequently studied by using cardiovascular autonomic reflex tests and spectral analysis of heart rate in time and frequency domain (Ewing and Clarke, 1982; van Ravenswaaij-Arts et al., 1993). Heart rate variability (HRV) analysis, which involves measurement and analysis of heart rate variation, provides a quantitative marker of autonomic activity and has proved to be able to detect and measure modification in sympathetic or vagal activity. Moreover, it can be used to estimate the susceptibility for heart arrhythmias (Heart rate variability, 1996a,b).
Highlights
DYSTROPHINOPATHIES Duchenne (DMD) and Becker (BMD) muscular dystrophies are X-linked recessive disorders due to complete or partial loss of dystrophin protein
The authors demonstrated that, Heart rate variability (HRV) tended to decrease with the progress of clinical severity, it cannot be considered a good predictor of death in Duchenne muscular dystrophy (DMD) (Yotsukura et al, 1995, 1998)
The neuropathological evidence of Autonomic Nervous System (ANS) involvement in DMD has been shown in mdx mice, where a selective loss of the sympathetic superior cervical ganglion (SCG) neurons, combined with the dystrophin-associated muscle damage, might lead to ganglion neuron death (De Stefano et al, 2005)
Summary
DYSTROPHINOPATHIES Duchenne (DMD) and Becker (BMD) muscular dystrophies are X-linked recessive disorders due to complete or partial loss of dystrophin protein. In 1997, the arrhythmic profile in a population of 20 BMD patients was investigated to correlate the severity of arrhythmic events, the cardiac autonomic balance (assessed by HRV analysis in the time domain) and the degree of left systolic impairment (Ducceschi et al, 1997).
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