Premium
Muscle histology in becker muscular dystrophy
Author(s) -
Kaido Misako,
Arahata Kiichi,
Hoffman Eric P.,
aka Ikuya,
Sugita Hideo
Publication year - 1991
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880141105
Subject(s) - atrophy , medicine , muscular dystrophy , abnormality , dystrophin , histology , pathology , dystrophy , age of onset , anatomy , disease , psychiatry
Twenty patients with Becker muscular dystrophy (BMD), confirmed by dystrophin tests, were studied histologically. There were several morphological differences between younger (≤15‐year‐old) and older (>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.