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Autonomic Nervous System Function in Genetic Neuromuscular DisordersHereditary Motor-Sensory Neuropathy and Myotonic Dystrophy
Thomas D. Bird, MD;
Andrew M. Reenan, MS;
Michael Pfeifer, MD
Arch Neurol. 1984;41(1):43-46.
Abstract
• Three quantitative measures of autonomic nervous system (ANS) function were evaluated in 12 patients with two types of autosomal dominant neuromuscular disorder, type 1 hereditary motorsensory neuropathy and myotonic dystrophy. The three measures were latency of the pupillary light reflex, dark-adapted pupil size during parasympathetic blockade, and ECG RR interval variation. The results showed deficits in sympathetic and/or parasympathetic nervous system function in several patients, with a variability of expression expected in autosomal dominant genetic diseases. The ANS function may commonly be deficient in both of these disorders. Further investigation is required to determine the potential clinical importance of these findings.
Author Affiliations
From the Department of Medicine, University of Washington School of Medicine (Drs Bird and Pfeifer) and the Veterans Administration Medical Center (Drs Bird and Pfeifer and Mr Reenan), Seattle. Dr Pfeifer is now with the VA Medical Center, Louisville.
Footnotes
Accepted for publication March 26, 1983.
Reprint requests to Neurology Section, VA Medical Center, 4435 Beacon Ave S, Seattle, WA 98108 (Dr Bird).
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