Abstract
A family of benign X-linked muscular dystrophy is described. Two of the 3 affected members appear quite representative of Becker's dystrophy. A third shows no pseudohypertrophy, only gross atrophy, affecting proximal and distal muscles and also shows early onset contractures and electrocardiographic abnormalities and is in these ways much more representative of the variety described by Emery and Dreifuss (1966). Two of the cases have distinctly abnormal electrocardiograms with extensive and deep Q waves and abnormal R/S ratios and VI. Both these have shown progression of electrocardiographic abnormalities during a 2-year follow-up. The family is reported to document this very unusual occurrence.
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- Emery A. E. Abnormalities of the electrocardiogram in hereditary myopathies. J Med Genet. 1972 Mar;9(1):8–12. doi: 10.1136/jmg.9.1.8. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Emery A. E., Dreifuss F. E. Unusual type of benign x-linked muscular dystrophy. J Neurol Neurosurg Psychiatry. 1966 Aug;29(4):338–342. doi: 10.1136/jnnp.29.4.338. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Mabry C. C., Roeckel I. E., Munich R. L., Robertson D. X-linked pseudohypertrophic muscular dystrophy with a late onset and slow progression. N Engl J Med. 1965 Nov 11;273(20):1062–1070. doi: 10.1056/NEJM196511112732002. [DOI] [PubMed] [Google Scholar]
- Rotthauwe H. W., Mortier W., Beyer H. Neuer Typ einer recessiv X-chromosomal vererbten Muskeldystrophie: Scapulo-humero-distale Muskeldystrophie mit frühzeitigen Kontrakturen und Herzrhythmusstörungen. Humangenetik. 1972;16(3):181–200. doi: 10.1007/BF00273464. [DOI] [PubMed] [Google Scholar]
- Skinner R., Smith C., Emery A. E. Linkage between the loci for benign (Becker-type) X-borne muscular dystrophy and deutan colour blindness. J Med Genet. 1974 Dec;11(4):317–320. doi: 10.1136/jmg.11.4.317. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Slucka C. The electrocardiogram in Duchenne progressive muscular dystrophy. Circulation. 1968 Nov;38(5):933–940. doi: 10.1161/01.cir.38.5.933. [DOI] [PubMed] [Google Scholar]
- Thomas P. K., Calne D. B., Elliott C. F. X-linked scapuloperoneal syndrome. J Neurol Neurosurg Psychiatry. 1972 Apr;35(2):208–215. doi: 10.1136/jnnp.35.2.208. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Ueda K., Okada R., Matsuo H., Harumi K., Yasuda H. Myocardial involvement in benign Duckenne type of progressive muscular dystrophy. Jpn Heart J. 1970 Jan;11(1):26–35. doi: 10.1536/ihj.11.26. [DOI] [PubMed] [Google Scholar]
- Zellweger H., Hanson J. W. Slowly progressive X-linked recessive muscular dystrophy (type 3b). Report of cases and review of the literature. Arch Intern Med. 1967 Nov;120(5):525–535. [PubMed] [Google Scholar]


