Heart 1998;79:619-621 ( June )
Case report
Cardiac involvement in proximal myotonic myopathy
a Department of Cardiology,
Georg-August-University, Göttingen, Germany, b Department of Nephrology and
Rheumatology, Georg-August-University, c Department of Pathology, Municipal Hospital, Darmstadt,
Germany, d Department of
Neuropathology, Georg-August-University, e Department of Clinical Neurophysiology,
Georg-August-University
Correspondence to: Dr C D Reimers, Abteilung für klinische Neurophysiologie, Georg-August-Universität, Robert-Koch-Strasse 40, D-37075 Göttingen, Germany. email: cd.reimers{at}med.uni-goettingen.de
Accepted for publication 19 February 1998
Proximal myotonic myopathy (PROMM) is a recently described
autosomal dominantly inherited disorder resulting in proximal muscle weakness, myotonia, and cataracts. A few patients with cardiac involvement (sinus bradycardia, supraventricular bigeminy,
conduction abnormalities) have been reported. The cases of three
relatives with PROMM (weakness of neck flexors and proximal extremity
muscles, calf hypertrophy, myotonia, cataracts) are reported: a 54 year old man, his 73 year old mother, and 66 year old aunt. All three presented with conduction abnormalities and one had repeated, life
threatening, sustained monomorphic ventricular tachycardia. This
illustrates that severe cardiac involvement may occur in PROMM.
© 1998 by Heart
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
-
Nof, E., Luria, D., Brass, D., Marek, D., Lahat, H., Reznik-Wolf, H., Pras, E., Dascal, N., Eldar, M., Glikson, M.
(2007). Point Mutation in the HCN4 Cardiac Ion Channel Pore Affecting Synthesis, Trafficking, and Functional Expression Is Associated With Familial Asymptomatic Sinus Bradycardia. Circulation
116: 463-470
[Abstract] [Full Text] -
Schoser, B. G.H., Ricker, K., Schneider-Gold, C., Hengstenberg, C., Durre, J., Bultmann, B., Kress, W., Day, J. W., Ranum, L. P.W.
(2004). Sudden cardiac death in myotonic dystrophy type 2. Neurology
63: 2402-2404
[Abstract] [Full Text]
- Full Text
- Full Text (PDF)
- Submit a response
- Alert me when this article is cited
- Alert me when eLetters are posted
- Alert me if a correction is posted
Services
- Email this link to a friend
- Similar articles in this journal
- Similar articles in PubMed
- Add article to my folders
- Download to citation manager
- Request Permissions
Citing Articles
Google Scholar
PubMed
Topic Collections
Bookmark with
Register for free content
The full back archive is now available for all BMJ Journals. Institutional subscribers may access the entire archive as part of their subscription. Personal subscribers will also have access to all content when logged in. Non-subscribers who register have free access to all articles published before 2006 right back to volume 1 issue 1. Register here to access the free archive of all BMJ Journals.
Don't forget to sign up for content alerts so you keep up to date with all the articles as they are published.
