| Assessment of cardiovascular autonomic function in myotonic dystrophy type 2 (DM2/PROMM). | |
| | |
MedLine Citation:
|
PMID:Â 12868497 Â Â Â Owner:Â NLM Â Â Â Status:Â MEDLINE Â Â Â |
Abstract/OtherAbstract:
|
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. |
| | |
Authors:
|
Peter Flachenecker; Christiane Schneider; Simone Cursiefen; Kenneth Ricker; Klaus V Toyka; Karlheinz Reiners |
Related Documents
:
|
11408337Â -Â Clinical, biochemical and molecular genetic characteristics of 19 patients with the sjÃ... 746017Â -Â 'epidemic neuromyasthenia' 1955-1978. 8582397Â -Â Frequency of a 22q11 deletion in patients with conotruncal cardiac malformations: a pro... 8960317Â -Â Myopathology in patients with a noonan phenotype. 21470837Â -Â Circulating soluble receptor for advanced glycation end product (srage) and left ventri... 14693167Â -Â Thrombogenesis and endothelial damage/dysfunction in peripheral artery disease. relatio... |
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:Â 2003-07-18 Â Â Â Completed Date:Â 2003-08-19 Â Â Â Revised Date:Â 2008-11-21 Â Â Â |
Medline Journal Info:
|
Nlm Unique ID: 9111470    Medline TA: Neuromuscul Disord    Country: England    |
Other Details:
|
Languages: eng    Pagination: 289-93    Citation Subset: IM    |
Affiliation:
|
Department of Neurology, Julius-Maximilians-Universität Würzburg, Josef-Schneider-Strasse 11, D-97080 Würzburg, Germany. |
Export Citation:
|
APA/MLA Format    Download EndNote    Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adult Arrhythmias, Cardiac / physiopathology Autonomic Nervous System / physiopathology* Blood Pressure Cardiovascular Physiological Phenomena Cardiovascular System / physiopathology* Case-Control Studies Electrocardiography Female Heart Conduction System / physiopathology* Heart Rate Humans Male Middle Aged Myotonic Disorders / physiopathology Myotonic Dystrophy / physiopathology* Posture Respiration Valsalva Maneuver |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Increased resistance to oxidation of betalain-enriched human low density lipoproteins.
Next Document: Cardiomyopathic features associated with muscular dystrophy are independent of dystrophin absence in...