Variable histomorphology of muscle in congenital muscular dystrophy

Journal of the Neurological Sciences
S DasY Ramamohan

Abstract

Congenital muscular dystrophy (CMD) is a relatively uncommon disease with a controversial nosological status. That collagen synthesis could be the primary abnormality has been suggested earlier (Fidzianska et al., 1982). Amongst eighteen cases of CMD diagnosed during the past twelve years, muscle biopsy in three cases revealed prominence of myofibre necrosis and phagocytosis, and serum CPK was markedly elevated suggesting a rapidly progressive form. In twelve cases, marked increase in endomysial collagen, pronounced fallout of myofibres and significant fibre diameter variation was seen. This was associated with myonecrosis and regenerative activity of mild degree resembling the classical form of CMD. In the remaining three cases, polyfocal, polyphasic necrosis was noticed. Fibre splitting was more frequently observed, better delineated in the enzyme histochemical preparations, affecting both fibre types, while endomysial fibrofatty tissue was only moderately increased. The histomorphology in the latter group resembled that of limb girdle dystrophy. Ultrastructural findings in all the eighteen cases correlated well with light microscopic observations. lmmunohistochemical studies done on three of the eighteen cases showed normal ...Continue Reading

References

Sep 1, 1991·Journal of the Neurological Sciences·E ArikawaK Arahata
Nov 1, 1988·Journal of the Neurological Sciences·K PatelV Dubowitz
Aug 1, 1969·Journal of Neurology, Neurosurgery, and Psychiatry·A AfifiW F McCormick
Jul 1, 1967·Journal of the Neurological Sciences·D Gardner-MedwinJ N Walton
Jul 1, 1982·Journal of the Neurological Sciences·A FidzianskaC Heckmann
Oct 1, 1993·Journal of the Neurological Sciences·Y K HayashiK Arahata

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Citations

Jun 7, 2005·Muscle & Nerve·Dominique S Tews

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