P4.31 Measurement of the muscle volume in patients with muscular dystrophy using muscle CT

2010 ◽  
Vol 20 (9-10) ◽  
pp. 667
Author(s):  
T.N. Nakayama
2013 ◽  
Vol 2013 (may31 1) ◽  
pp. bcr2013009301-bcr2013009301
Author(s):  
T. Nakayama ◽  
S. Kuru ◽  
M. Kawai

1995 ◽  
Vol 17 (2) ◽  
pp. 95-103 ◽  
Author(s):  
Yumi Arai ◽  
Makiko Osawa ◽  
Yukio Fukuyama

1996 ◽  
Vol 6 (2) ◽  
pp. S20
Author(s):  
M. Cappellini ◽  
I. Mahjneh ◽  
A. Pizzi ◽  
G. Marconi

Author(s):  
S. E. Miller ◽  
G. B. Hartwig ◽  
R. A. Nielsen ◽  
A. P. Frost ◽  
A. D. Roses

Many genetic diseases can be demonstrated in skin cells cultured in vitro from patients with inborn errors of metabolism. Since myotonic muscular dystrophy (MMD) affects many organs other than muscle, it seems likely that this defect also might be expressed in fibroblasts. Detection of an alteration in cultured skin fibroblasts from patients would provide a valuable tool in the study of the disease as it would present a readily accessible and controllable system for examination. Furthermore, fibroblast expression would allow diagnosis of fetal and presumptomatic cases. An unusual staining pattern of MMD cultured skin fibroblasts as seen by light microscopy, namely, an increase in alcianophilia and metachromasia, has been reported; both these techniques suggest an altered glycosaminoglycan metabolism An altered growth pattern has also been described. One reference on cultured skin fibroblasts from a different dystrophy (Duchenne Muscular Dystrophy) reports increased cytoplasmic inclusions seen by electron microscopy. Also, ultrastructural alterations have been reported in muscle and thalamus biopsies from MMD patients, but no electron microscopical data is available on MMD cultured skin fibroblasts.


Author(s):  
H. D. Geissinge ◽  
L.D. Rhodes

A recently discovered mouse model (‘mdx’) for muscular dystrophy in man may be of considerable interest, since the disease in ‘mdx’ mice is inherited by the same mode of inheritance (X-linked) as the human Duchenne (DMD) muscular dystrophy. Unlike DMD, which results in a situation in which the continual muscle destruction cannot keep up with abortive regenerative attempts of the musculature, and the sufferers of the disease die early, the disease in ‘mdx’ mice appears to be transient, and the mice do not die as a result of it. In fact, it has been reported that the severely damaged Tibialis anterior (TA) muscles of ‘mdx’ mice seem to display exceptionally good regenerative powers at 4-6 weeks, so much so, that these muscles are able to regenerate spontaneously up to their previous levels of physiological activity.


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