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Updated: Jun 23, 2026

Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
09:18

Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy

Published on: January 12, 2019

Autonomic function in patients with Duchenne muscular dystrophy.

Miki Inoue1, Kazuhiro Mori, Yasunobu Hayabuchi

  • 1Department of Pediatrics, School of Medicine, University of Tokushima, Tokushima, Japan. mikinoue1129@yahoo.co.jp

Pediatrics International : Official Journal of the Japan Pediatric Society
|April 18, 2009
PubMed
Summary

Autonomic function is frequently abnormal in Duchenne muscular dystrophy (DMD) patients, even with normal cardiac markers. Mean heart rate at night can predict abnormal autonomic function, offering a simple evaluation method.

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Published on: January 31, 2013

Area of Science:

  • Cardiology
  • Neurology
  • Pediatrics

Background:

  • Assessing autonomic function is crucial for chronic heart failure management.
  • Autonomic function changes and their clinical correlations in young Duchenne muscular dystrophy (DMD) patients are not well understood.

Purpose of the Study:

  • To investigate autonomic function in DMD patients.
  • To correlate autonomic function with cardiac parameters like brain natriuretic peptide (BNP) and shortening fraction (SF).

Main Methods:

  • Ambulatory electrocardiogram (ECG) for heart rate variability (HRV) analysis in 57 DMD patients.
  • Comparison of HRV data with serum BNP levels and echocardiographic SF.
  • Analysis of 24-hour ECG parameters.

Main Results:

  • Standard deviation for all normal R-R intervals (SDNN) was the most frequently abnormal HRV parameter (96%).
  • Abnormalities in parasympathetic and sympathetic activity were common (74-78% and 43%, respectively), even with normal SF and BNP.
  • Mean heart rate at night accurately predicted abnormal SDNN (94% sensitivity, 85% specificity at 71 bpm).

Conclusions:

  • Autonomic dysfunction, particularly reduced SDNN, is prevalent in DMD patients, irrespective of cardiac function markers.
  • Mean heart rate during nighttime emerges as a simple, effective tool for evaluating autonomic function in DMD.