Muscle histology in Becker muscular dystrophy. |
| |
Authors: | M Kaido K Arahata E P Hoffman I Nonaka H Sugita |
| |
Affiliation: | National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan. |
| |
Abstract: | Twenty patients with Becker muscular dystrophy (BMD), confirmed by dystrophin tests, were studied histologically. There were several morphological differences between younger (less than or equal to 15-year-old) and older (greater than 15-year-old) patients. In the younger patients, active muscle fiber necrosis followed by a regenerating process was conspicuous. In the older patients, the active degenerative changes appeared less prominent and, instead, more chronic myopathic changes such as moth-eaten fibers, fiber splitting, and hypertrophic fibers were evident. These age-dependent differences in the pathology of BMD were irrespective of the duration of clinical symptoms, i.e., BMD patients of a similar age showed a similar morphological feature regardless of age at onset. Although the presence of mild fiber type grouping and some small angulated atrophic fibers suggested a certain degree of neurogenic involvement, none of biopsies showed significant grouped atrophy as seen in neuropathic disorders. There was no correlation between the histological changes and the specific dystrophin abnormality. |
| |
Keywords: | Becker muscular dystrophy dystrophin muscle histochemistry myonecrosis fiber type analysis |
|
|