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Journal of Child Neurology
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Respiratory Sinus Arrhythmia in Patients With Prader-Willi Syndrome

Francis J. DiMario, Jr, MD

Department of Pediatrics, Division of Pediatric Neurology University of Connecticut, Farmington, CT

Lance Bauer, PhD

Department of Psychiatry University of Connecticut, Farmington, CT

Jana Volpe, BS

Department of Psychiatry University of Connecticut, Farmington, CT

Suzanne B. Cassidy, MD

Department of Genetics and Center for Human Genetics Case Western Reserve University and University Hospitals of Cleveland, Cleveland, OH

In this investigation, we sought to further test the hypothesis that parasympathetic deficiency exists among persons with Prader-Willi syndrome, by examining respiratory sinus arrhythmia. The study sample comprised two groups of patients: 14 subjects with Prader-Willi syndrome and 14 age- and sex-matched controls. Each subject's electrocardiogram was recorded in a quiet room and digitized by a personal computer during five 1-minute periods. RR intervals within each 1-minute period were converted to heart rate in 120 successive 0.5-second intervals. The resultant heart rate time series was converted to its underlying frequency composition by a fast Fourier transform and averaged across minutes. Respiratory sinus arrhythmia was defmed as the variability in the time series over a frequency range (0.096 to 0.48 Hz) corresponding to a range of respiratory rates from six to 30 breaths/minute. Analysis revealed significantly less variability in the heart rates of subjects with Prader-Willi syndrome relative to age- and sex-matched controls (group x frequency bin: F = 2.26, P < .05). An analysis of covariance adjusting for body mass index differences between the groups produced identical results. These findings support the existence of a parasympathetic deficiency among subjects with Prader-Willi syndrome independent of their body mass. This is likely due to dysregulation of the central autonomic network. (J Child Neurol 1996;11:121-125).

Journal of Child Neurology, Vol. 11, No. 2, 121-125 (1996)
DOI: 10.1177/088307389601100212


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