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Review
. 2012 Oct;31(2):110-6.

The heart and cardiac pacing in Steinert disease

Gerardo Nigro  1 , Andrea Antonio Papa, Luisa Politano
Affiliations
Review

The heart and cardiac pacing in Steinert disease

Gerardo Nigro et al. Acta Myol. 2012 Oct.

Abstract

Myotonic dystrophy (Dystrophia Myotonica, DM) is the most frequently inherited neuromuscular disease of adult life. It is a multisystemic disease with major cardiac involvement. Core features of myotonic dystrophy are myotonia, muscle weakness, cataract, respiratory failure and cardiac conduction abnormalities. Classical DM, first described by Steinert and called Steinert's disease or DM1 (Dystrophia Myotonica type 1) has been identified as an autosomal dominant disorder associated with the presence of an abnormal expansion of a CTG trinucleotide repeat in the 3' untranslated region of DMPK gene on chromosome 19. This review will mainly focus on the various aspects of cardiac involvement in DM1 patients and the current role of cardiac pacing in their treatment.

Keywords: arrhythmias; cardiac pacing; myotonic dystrophy type 1.

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Figures

Figure 1.
Figure 1.
The grip test is a quick and easy way to determine the presence of active myotonia. After contraction of the fist, the patient is unable to relax the muscles of the hand.
Figure 1.
Figure 1.
The grip test is a quick and easy way to determine the presence of active myotonia. After contraction of the fist, the patient is unable to relax the muscles of the hand.
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References

    1. Emery AE. The muscular dystrophies. Lancet. 2002;359:687–695. - PubMed
    1. Emery AE. Heritability of common neuromuscular diseases. Neuromuscul Disord. 2010;20:476–476. - PubMed
    1. Harper PS. Myotonic Dystrophy: some genetic problems. Birth Defects Orig Artic Ser. 1974;10:120–125. - PubMed
    1. Harley HG, Brook JD, Rundle SA, et al. Expansion of an unstable DNA region and phenotypic variation in myotonic dystrophy. Nature. 1992;355:545–546. - PubMed
    1. Walton JN. Clinical examination of the neuromuscular system. In: John Walton., editor. Disorders of Voluntary Muscle. London: Churchill Livingstone; 1981.

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