Summary
The incidence of electrocardiographic features “typical” for the Duchenne progressive muscular dystrophy (PMD) was investigated in 191 carriers: 136 mothers and 55 daughters. The typical PMD pattern was seen in 6.6% of the mothers, and a pattern similar to that observed in PMD in 2.9%. Among daughters of carriers the percentages were 16.4 and 1.8 respectively. The daughters obviously belong to a much lower age bracket (average 13.3 years) and therefore must be followed for the eventual disappearance of the “juvenile” ECG features, which, in this group, may reduce the incidence of the typical PMD electrocardiograms.
The need for more thorough clinical appraisal of the state of the myocardium in carriers is emphasized, especially in those with the “typical” PMD electrocardiograms, with the help of hemodynamic investigations.
Zusammenfassung
Bei 191 sicheren oder möglichen Konduktorinnen der Duchenneschen progressiven Muskeldystrophie wurde die Häufigkeit „typischer“ elektrokardiographischer Veränderungen untersucht. Es handelte sich um 136 Mütter und 55 Töchter. Unter den Müttern fand man in 6,6% der Fälle die typischen Veränderungen und in 2,9% der Fälle diesen letzteren ähnliche EKG-Formen. Unter den Töchtern von Konduktorinnen waren die Prozentzahlen 16,4 respektive 1,8. Die Töchter waren naturgemäß im Durchschnitt wesentlich jünger (13,3 Jahre). Es ist deshalb nötig, in einem späteren Zeitpunkt Kontrollen des EKGs durchzuführen. Es ist denkbar, daß in dieser Altersgruppe “juvenile” EKG-Besonderheiten die Häufigkeit der für progressive Muskeldystrophie typischen EKG-Veränderungen vermindert. Die Autoren befürworten eine sorgfältige klinische Analyse des Herzens bei Konduktorinnen, besonders bei jenen mit den typischen EKG-Veränderungen.
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Work supported by N.I.H., N.I.N.D.S., Bethesda, agreement 05-002-1.
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Łukasik, E. Electrocardiographic studies in female carriers of duchenne muscular dystrophy. J. Neurol. 209, 279–285 (1975). https://doi.org/10.1007/BF00314367
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DOI: https://doi.org/10.1007/BF00314367