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Muscular changes in Engelmann's disease
Archives of Disease in Childhood
|September 1, 1980
Abstract:
In a case of Engelmann's disease in an 11-year-old Japanese boy the muscular changes were studied in detail. Muscle weakness was maximal about the pelvic girdle. Muscle biopsy showed the selective atrophy of type II fibres, and no degenerative change could be seen histologically, histochemically, or electron-microscopically. Although the distribution of muscular weakness in Engelmann's disease is similar to that of a progressive muscular dystrophy, the disease does not seem to be a myopathy.
Insights
Engelmann
Area of Science:
- Neurology
- Muscle Biology
Background:
- Engelmann's disease is a rare condition.
- Understanding its muscular changes is crucial.
Observation:
- A case study of an 11-year-old Japanese boy with Engelmann's disease.
- Muscle weakness was most pronounced in the pelvic girdle.
Findings:
- Muscle biopsy revealed selective atrophy of type II muscle fibers.
- No degenerative changes were observed via histological, histochemical, or electron-microscopic analysis.
Implications:
- The findings suggest Engelmann's disease may not be a myopathy.
- Further research is needed to differentiate it from progressive muscular dystrophy.