Pseudohypertrophic muscular dystrophy
- 1 September 1963
- journal article
- research article
- Published by Wolters Kluwer Health in Neurology
- Vol. 13 (9) , 728
- https://doi.org/10.1212/wnl.13.9.728
Abstract
Observations are presented from an anatomical study provided by the body of a 16-year-old white male with pseudohypertrophic muscular dystrophy. These indicate that in this form of dystrophy all of the skeletal muscles are diseased. Degeneration proceeds in all, but at different rates. The function of a muscle, or portion of a muscle, is a fundamental factor in determining the rate at which it will deteriorate. Those muscles or portions of a muscle,performing the greatest degree of sustained activity wear out first and become replaced by fat. Those assuming the least degree of sustained activity suffer the least. Deformity in one part of the body as a result of muscle wasting facilitates the wasting of muscles in other portions of the body by causing them to become additionally activated in the performance of substitute or auxiliary roles. It appears that in this particular form of dystrophy the underlying molecular defect is intimately related to, or affected by, physical activity which hastens the muscle''s demise. This might also be true for the Leyden-Moebius form. The clinical distribution of muscle weakness alone makes it seem exceedingly unlikely that the same is true for myotonic dystrophy, Gower''s distal form, Erb''s juvenile form, the Landouzy-Dejerine form, or progressive external ophthalmoplegia.Keywords
This publication has 7 references indexed in Scilit:
- The function of the erectores spinae muscles in certain movements and postures in man*The Journal of Physiology, 1955
- ON THE CLASSIFICATION, NATURAL HISTORY AND TREATMENT OF THE MYOPATHIESBrain, 1954
- MUSCULAR CONTROL OF THE ARCHES OF THE FOOT IN STANDING - AN ELECTROMYOGRAPHIC ASSESSMENT1954
- AN ELECTROMYOGRAPHIC STUDY OF CERTAIN MUSCLES OF THE LEG AND FOOT IN THE STANDING POSITION1954
- THE MECHANICS OF THE FOOT .2. THE PLANTAR APONEUROSIS AND THE ARCH1954
- STUDIES IN DISORDERS OF MUSCLE. IV. THE CLINICAL MANIFESTATIONS AND INHERITANCE OF CHILDHOOD PROGRESSIVE MUSCULAR DYSTROPHYAnnals of Internal Medicine, 1951
- ELECTROMYOGRAPHIC STUDY OF PATTERNS OF ACTIVITY OF THE ANTERIOR ABDOMINAL WALL MUSCLES IN MAN1950