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NEUROLOGY 1980;30:497
© 1980 American Academy of Neurology

Electrocardiographic abnormalities in carriers of Duchenne muscular dystrophy

Russell J.M. Lane, B.Sc., M. B., B. S, David Gardner-Medwin, M.D and Allen D. Roses, M.D.

Howard Hughes Medical Institute (Drs. Lane and Roses), Departments of Medicine (Neurology) and Biochemistry, Duke University Medical Center, Durham, NC, and the Muscular Dystrophy Research Laboratories (Dr. Gardner-Medwin), Regional Neurological Centre, Newcastle General Hospital, University of Newcastle upon Tyne, England.

Electrocardiographic (ECG) abnormalities characteristic of Duchenne muscular dystrophy (tall R waves in the right precordial leads and deep Q waves in the lateral chest leads) are rarely observed in carriers, but Emery (1969) found that the (R-S) amplitude sum in lead V1 was significantly greater in carriers than in age-matched controls. In a prospective single-blind study, we analyzed coded records from 11 carriers and 12 age-matched normal women for (R-S) amplitude sums and R/S ratios in leads V1 and V2. Values in carriers were significantly greater for all these characteristics, discrimination being most marked for R/S in V2 (p < 0.01). This was confirmed in a further retrospective study, comparing records from 18 carriers with 100 normal ECGs from women of the same age range. Density functions for (R-S) in V1 and the R/S ratio in V2 derived from carrier and normal population can be incorporated into probability calculations to determine risk of carrier status.

Address correspondence and reprint requests to Dr. Lane, Duke University School of Medicine, P.O. Box 2900, Duke University Medical Center, Durham, NC 27710.

Dr. Lane is a Muscular Dystrophy Association Research Fellow and also gratefully acknowledges support from the Wellcome Foundation, the Muscular Dystrophy Association of Great Britain, and the University of Newcastle upon Tyne, England. This work was supported in part by NINCDS Grant No. NS 13455, Grant No. RR 30 from the General Clinical Research Center's Program of the Division of Research Resources, National Institutes of Health, and a Clinical Research Grant from the Muscular Dystrophy Association of America.

Accepted for publication August 14, 1979.




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