| Cardiac and skeletal muscle involvement in myotonic dystrophy type 2 (DM2): a quantitative 31P-MRS and MRI study. | |
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MedLine Citation:
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PMID: 15452841 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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In myotonic dystrophy type 2 (DM2/PROMM), cardiac muscle involvement is usually more benign than in DM1, but clinically severe cardiomyopathy has been reported in some patients. Using a novel method of magnetic resonance spectroscopy (MRS), we examined the left ventricular myocardium and the left gastrocnemius muscle in 11 unselected DM2/PROMM patients without overt cardiac disease. Data on cardiac morphology and function were obtained by gradient echo two-dimensional cine magnetic resonance imaging (MRI); no significant differences were found between DM2 patients and healthy controls, but using a median split approach older patients showed mildly increased left ventricular (LV) volumes, i.e., 59% increase of end-systolic volume index (ESVI) and 35% increase of end-diastolic volume index (EDVI), and an increase of LV mass (26%). On cardiac MRS, DM2/PROMM patients showed a reduction of phosphocreatine (PCr) and adenosine triphosphate (ATP) by 25 and 20% compared to matched healthy controls. No significant differences were found between younger and older patients. In skeletal muscle of the DM2 patients, no significant decrease of PCr and ATP concentrations was found. However, in older patients, who commonly show overt hip flexor muscle weakness, we observed reduced values for PCr and ATP. Our MRS and MRI findings reveal evidence for subclinical cardiomyopathy in DM2/PROMM patients without overt heart disease. Future prospective studies are needed to clarify the risk of developing overt cardiac disease in DM2 and to define prognostic factors. |
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Authors:
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Christiane Schneider-Gold; Meinrad Beer; Herbert Köstler; Stefan Buchner; Jörn Sandstede; Dietbert Hahn; Klaus V Toyka |
Publication Detail:
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Type: Comparative Study; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Muscle & nerve Volume: 30 ISSN: 0148-639X ISO Abbreviation: Muscle Nerve Publication Date: 2004 Nov |
Date Detail:
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Created Date: 2004-10-25 Completed Date: 2004-12-07 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 7803146 Medline TA: Muscle Nerve Country: United States |
Other Details:
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Languages: eng Pagination: 636-44 Citation Subset: IM |
Affiliation:
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Department of Neurology, University of Würzburg, Josef-Schneider Strasse 11, 97080 Würzburg, Germany. christiane.schneider-gold@mail.uni-wuerzburg.de <christiane.schneider-gold@mail.uni-wuerzburg.de> |
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Analysis of Variance Female Humans Magnetic Resonance Imaging / methods, statistics & numerical data Magnetic Resonance Spectroscopy / methods, standards Male Middle Aged Muscle, Skeletal* / metabolism, pathology Myocardium* / metabolism, pathology Myotonic Dystrophy / classification*, metabolism, physiopathology* Phosphorus Isotopes / metabolism Protons Statistics, Nonparametric |
| Chemical | |
Reg. No./Substance:
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0/Phosphorus Isotopes; 0/Protons |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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