ISSN:
1432-0533
Keywords:
Werdnig-Hoffmann's Disease
;
Electron Microscopy
;
Muscle Biopsy
;
Myotube-like Cells
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary An ultrastructural study was carried out on muscle biopsies of 7 children with Werdnig-Hoffmann's disease. The muscle fibres were classified into 3 categories: 1. Muscle cells of normal diameter without any distinct or only slight changes. 2. Small muscle cells with a large central nucleus with the appearance of myoblast at a later stage of development. 3. Cells resembling myotubes containing in a common basement membrane two to four cells at different stages of maturation. Myoblast and myotube-like cells seemed to resemble foetal muscle fibres. Such fibres appear to represent an arrest in maturation rather than muscle atrophy.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00687634
