Document Detail

Relation of CTG expansion and clinical variables to electrocardiogram conduction abnormalities and sudden death in patients with myotonic dystrophy.
MedLine Citation:
PMID:  14678805     Owner:  NLM     Status:  MEDLINE    
We prospectively followed 63 patients with myotonic dystrophy (DM) after establishing diagnosis of DM for an average 8 years in an attempt to detect conduction disturbances (by electrocardiography and/or Holter monitoring) and sudden cardiac events (sudden death, cardiac syncope) and correlate them to potential predicting factors (CTG repeat expansion in the myotonin protein kinase gene and several clinical variables: clinical type and duration of DM, age and sex). Twenty-six patients developed conduction disturbances, five patients died suddenly, and two patients experienced cardiac syncope necessitating urgent implantation of pacemaker. Analysis showed no significant correlation between conduction disturbances and/or cardiac events and CTG expansion. Furthermore, no correlation was found with type of DM, whereas conduction disturbances and sudden cardiac events correlated with patients' age, duration of disease and male sex. Results on our cohort of DM patients show that CTG expansion has no role in predicting neither conduction abnormalities nor sudden death. It seems that risk of sudden death increases with duration of disease and age, and that risk is higher in male patients.
M Sabovic; I Medica; N Logar; E Mandić; J Zidar; B Peterlin
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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 Dec 
Date Detail:
Created Date:  2003-12-17     Completed Date:  2004-02-26     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  9111470     Medline TA:  Neuromuscul Disord     Country:  England    
Other Details:
Languages:  eng     Pagination:  822-6     Citation Subset:  IM    
Division of Medical Genetics, Department of Obstetrics and Gynecology, University Medical Centre, Ljubljana, Slovenia.
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MeSH Terms
Age Factors
Age of Onset
Child, Preschool
Cohort Studies
Death, Sudden, Cardiac / etiology*
Genetic Predisposition to Disease / genetics*
Heart Block / genetics*,  physiopathology
Heart Conduction System / physiopathology
Middle Aged
Myotonic Dystrophy / enzymology,  genetics*,  physiopathology*
Prospective Studies
Protein-Serine-Threonine Kinases / deficiency,  genetics
Sex Factors
Trinucleotide Repeat Expansion / genetics*
Reg. No./Substance:
EC 2.7.1.-/myotonic dystrophy protein kinase; EC Kinases

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

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