PMID: 1202896Dec 8, 1975Paper

Segmental fibre breakdown and defects of the plasmalemma in diseased human muscles

Acta Neuropathologica
H Schmalbruch

Abstract

Thick round fibres common in cross sections of muscle biopsies from patients with muscular dystrophy are due to contracted and swollen segments of otherwise normal muscle fibres. This contracture leads to segmental fibre breakdown, which is identical with Zenker's waxy degeneration. In biopsies from 90 patients suspected of neuromuscular disease, segmental contracture was seen in all or nearly all patients with infantile muscular dystrophy, necrotic myopathy or acute alcoholic myopathy. It was present in half of the patients with polymyositis or myotonic dystrophy. In resticted forms of muscular dystrophy it was rare as it was in neurogenic atrophy. In 9 clinically normal patients it was absent. In electron micrographs of the initial stage sarcomeres were moderately shortened, the sarcoplasmic reticulum was distended and the mitochondria were normal. In the plasmalemma holes were found, through which glycogen granules were lost into the interstitial tissue. In later stages myofibrils were overcontracted and homogenized; in large areas the plasmalemma was absent. Based on these findings a hypothesis for the development of waxy degeneration is proposed: locally defects of the plasmamembrane cause segmental contracture, glycogen g...Continue Reading

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Citations

Oct 12, 2000·Magnetic Resonance in Medicine : Official Journal of the Society of Magnetic Resonance in Medicine·V StraubK P Campbell
Jan 1, 1992·Acta Neuropathologica·M J CullenJ B Harris
Jan 1, 1989·Acta Neuropathologica·A ShahV Subramani
Jul 1, 1982·Journal of the Neurological Sciences·Y Wakayama, S Ohbu
Feb 1, 1983·Journal of the Neurological Sciences·G E Jones, J A Witkowski
Jun 1, 1984·Journal of the Neurological Sciences·P S WrightC Kent
May 31, 1989·Biochimica Et Biophysica Acta·A Martonosi
Jan 1, 1997·Pediatric Neurology·R FadicB P Lotz
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Jun 23, 2006·Clinical and Experimental Pharmacology & Physiology·Jeffry B Lansman, Alfredo Franco-Obregón
Jan 1, 1979·Annals of the New York Academy of Sciences·K WrogemannM C Blanchaer
Mar 27, 2010·Journal of Biomedicine & Biotechnology·Yoshihiro Wakayama
Oct 23, 1997·The Journal of Cell Biology·V StraubK P Campbell
Jan 1, 1979·Scandinavian Journal of Thoracic and Cardiovascular Surgery·R Nordlander, O Nyquist
Jul 6, 2007·Neuromuscular Disorders : NMD·Mario MarottaManuel Roig-Quilis
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Jan 1, 1985·Muscle & Nerve·N Kagawa, K Hizawa
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Feb 19, 2000·Microscopy Research and Technique·A A HackE M McNally

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