Muscle histopathology in myotonic dystrophy in relation to age and muscular weakness
Version of Record online: 13 OCT 2004
Copyright © 1994 John Wiley & Sons, Inc.
Muscle & Nerve
Volume 17, Issue 9, pages 1037–1043, September 1994
How to Cite
Tohgi, H., Kawamorita, A., Utsugisawa, K., Yamagata, M. and Sano, M. (1994), Muscle histopathology in myotonic dystrophy in relation to age and muscular weakness. Muscle Nerve, 17: 1037–1043. doi: 10.1002/mus.880170911
- Issue online: 13 OCT 2004
- Version of Record online: 13 OCT 2004
- Manuscript Accepted: 1 APR 1994
- myotonic dystrophy;
- muscular weakness
We studied histopathological changes in the biceps brachii muscle in relation to age and the degree of muscle weakness in 64 patients (aged 11–59 years) with myotonic dystrophy. The proportion of type 1 fibers was unaltered in the adolescent patients compared with control values, but increased with age. The average diameters of all the fiber types were smaller than control values in the adolescents, suggesting immature development; however, there was an increase in diameter with age that was associated with an increase of hypertrophic type 2 fibers. At all ages, type 1 fibers were smaller than type 2 ones. Small angular fibers and small group atrophy consisted mainly of type 1 fibers, their incidences decreasing with age. The severity of muscular weakness was related to the predominance of type 1 fibers, the reduction in the number of hypertrophic type 2 fibers, and the accumulation of adipose cells, but not to the presence of small angular fibers or to small group atrophy. © 1994 John Wiley & Sons, Inc.