Neurology
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Full Text (PDF)
Right arrow Correspondence:
Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when Correspondence are posted
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Medori, R.
Right arrow Articles by Waterston, R. H.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Medori, R.
Right arrow Articles by Waterston, R. H.
NEUROLOGY 1989;39:1493
© 1989 American Academy of Neurology

Two dissimilar brothers with Becker's dystrophy have an identical genetic defect

Rossella Medori, MD, Michael H. Brooke, MD and Robert H. Waterston, MD, PhD

From the Departments of Neurology/Neurosurgery and Genetics, Washington University Medical School, St. Louis, MO.

Becker's muscular dystrophy is phenotypically heterogeneous, but the clinical expression is usually similar in patients within the same family. We report here 2 brothers affected with Becker's muscular dystrophy in whom the disease followed completely different courses. The disease started in both patients before their teens. However, the oldest sibling died at 37, following many years of severe disability, whereas the other sibling, now 26, has normal muscle strength. In addition, since the age of 13, the younger brother has had epilepsy and has been treated with phenytoin combined with other antiepileptic drugs. Analysis of the DNA from each of the 2 brothers revealed a similar deletion at the 5' end of the dystrophin gene. The different clinical courses despite the similar mutational event suggest that intrinsic muscle factors due to modified genes or environmental phenomena such as prolonged treatment with phenytoin or other antiepileptic agents may have influenced the clinical course.

Address correspondence and reprint requests to Dr. Medori, Department of Neurology, H. Merritt Clinical Center, Columbia University Medical Center, 630 West 168th Street, New York, NY 10032.

Supported by a Clinical Fellowship Grant to Rossella Medori from the Muscular Dystrophy Association and a Clinical Research Grant from the Muscular Dystrophy Association.

Presented in part at the 40th annual meeting of the American Academy of Neurology, Cincinnati, OH, April 1988.

Received December 2, 1988. Accepted for publication in final form May 11, 1989.






HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Copyright © 1989 by AAN Enterprises, Inc.