Document Detail

Assessment of cardiovascular autonomic function in myotonic dystrophy type 2 (DM2/PROMM).
MedLine Citation:
PMID:  12868497     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Proximal myotonic myopathy is an autosomal dominant multisystem disorder with a recently defined CCTG expansion on chromosome 3 in the major subgroup (myotonic dystrophy type 2). Cardiac rhythm disturbances have been described in patients with this disease, but it is not known whether myotonic dystrophy type 2/proximal myotonic myopathy patients suffer from dysautonomia and whether cardiac arrhythmias relate to autonomic dysfunction. OBJECTIVES: To investigate cardiovascular autonomic function in myotonic dystrophy type 2/proximal myotonic myopathy patients with and without cardiac arrhythmias. PATIENTS AND METHODS: Standard autonomic function tests (heart rate responses to Valsalva manoeuvre, deep breathing and active change of posture, and blood pressure responses to active change of posture and sustained handgrip), resting heart rate variability in the time- and frequency-domain, and the corrected QT interval length were determined in 16 patients with genetically defined myotonic dystrophy type 2/proximal myotonic myopathy and compared to the results obtained in 16 age- and sex-matched healthy control subjects. RESULTS: Standard autonomic tests yielded similar results in both groups. Measures of heart rate variability tended to be lower in myotonic dystrophy type 2/proximal myotonic myopathy patients compared to healthy controls, but reached statistical significance only for the number of R-R intervals exceeding 50 ms (p50) and the power spectrum density in the low-frequency range (low-frequency power). Four patients (25%) suffered from mild cardiac rhythm disturbances encompassing paroxysmal tachycardia, sinoatrial block, right bundle branch block, ventricular premature beats and bradycardia. The autonomic responses of these patients were essentially similar compared to those without cardiac arrhythmias, apart from a decreased heart rate response to deep breathing in the patients with cardiac arrhythmias. CONCLUSIONS: We found no major abnormalities of cardiovascular autonomic function in patients with myotonic dystrophy type 2/proximal myotonic myopathy, neither in the whole study group nor in the subgroup of patients with cardiac rhythm abnormalities.
Peter Flachenecker; Christiane Schneider; Simone Cursiefen; Kenneth Ricker; Klaus V Toyka; Karlheinz Reiners
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Neuromuscular disorders : NMD     Volume:  13     ISSN:  0960-8966     ISO Abbreviation:  Neuromuscul. Disord.     Publication Date:  2003 May 
Date Detail:
Created Date:  2003-07-18     Completed Date:  2003-08-19     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  9111470     Medline TA:  Neuromuscul Disord     Country:  England    
Other Details:
Languages:  eng     Pagination:  289-93     Citation Subset:  IM    
Department of Neurology, Julius-Maximilians-Universität Würzburg, Josef-Schneider-Strasse 11, D-97080 Würzburg, Germany.
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MeSH Terms
Arrhythmias, Cardiac / physiopathology
Autonomic Nervous System / physiopathology*
Blood Pressure
Cardiovascular Physiological Phenomena
Cardiovascular System / physiopathology*
Case-Control Studies
Heart Conduction System / physiopathology*
Heart Rate
Middle Aged
Myotonic Disorders / physiopathology
Myotonic Dystrophy / physiopathology*
Valsalva Maneuver

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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