Document Detail


Cardiac involvement in the Kugelbert-Welander syndrome.
MedLine Citation:
PMID:  135509     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Two cases of the Kugelberg-Welander syndrome (juvenile form of progressive spinal muscular atrophy) associated with cardiomyopathy and cardiomegaly are presented. The first patient, a 24 year old man, had atrial flutter with complete atrioventricular (A-V) block due to A-H block. Echocardiography revealed an increase in the left atrial and right ventricular dimensions. The second patient was a 26 year old man whose electrocardiogram revealed an A-V junctional rhythm, deep Q wave in leads I, aVL and V5 to V6 and an RS pattern in lead V1. Histologic examination of the myocardium in Case 2 showed slight interstitial fibrosis. Review of previously reported cases shows that (1) the atrium, the ventricular myocardium and A-V conducting tissue may be involved, and (2) atrial arrhythmias, A-V conduction disturbances and congestive heart failure may occur in the Kugelberg-Welander syndrome.
Authors:
H Tanaka; N Uemura; Y Toyama; A Kudo; Y Ohkatsu
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The American journal of cardiology     Volume:  38     ISSN:  0002-9149     ISO Abbreviation:  Am. J. Cardiol.     Publication Date:  1976 Oct 
Date Detail:
Created Date:  1976-12-01     Completed Date:  1976-12-01     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0207277     Medline TA:  Am J Cardiol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  528-32     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Adult
Atrial Flutter / diagnosis
Cardiomegaly / diagnosis
Echocardiography
Genes, Recessive
Heart Block / diagnosis
Heart Diseases / diagnosis*
Humans
Male
Muscular Atrophy / complications,  genetics*
Neuromuscular Diseases / complications

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


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