Summary
Lesions in skeletal muscle resulting from ischemia caused by peripheral vascular disease in two patients were essentially identical to the early muscle lesions of Duchenne muscular dystrophy (DMD) patients and carriers of that disease, as well as to the early muscle lesions of experimental animals with aorta ligations plus a small dose of 5-hydroxytryptamine (serotonin). The one similarity is harmonious with, although does not necessarily advance, the ischemia hypothesis of DMD, and the other supports the ischemia mechanism proposed in the animal model.
Zusammenfassung
Es werden Veränderungen am Skeletmuskel bei zwei Patienten mit peripheren Gefäßerkrankungen beschrieben. Diese sind im wesentlichen identisch mit den frühen histologischen Veränderungen bei Duchenne'scher Dystrophie sowie bei Konduktorinnen dieser Krankheit und ebenfalls gleichartig mit denjenigen, die experimentell durch Ligatur der Aorta und zusätzlich kleinen Dosen von Serotonin am Versuchstier bewirkt werden können. Die erstgenannte Ähnlichkeit its vereinbar — obwohl nicht notwendigerweise beweisend — mit der Ischämietheorie der progressiven Muskeldystrophie. Die zweitgenannte Ähnlichkeit stützt die Annahme eines ischämischen Mechanismus, wie er für die Ergebnisse des Tierversuches vorgeschlagen wurde.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Boysen, G., Engel, A. G.: Effects of microembolization on the skeletal muscle blood flow. Acta Neurol. Scand. 52, 71–80 (1975)
Engel, W. K., Cunningham, G. G.: Rapid examination of muscle tissue—an improved trichrome method for rapid diagnosis of muscle biopsy fresh-frozen sections. Neurology 13, 919–923 (1963)
Engel, W. K.: Diseases of the neuromuscular junction and muscle. In: C. Adams (ed.), Neurohistochemistry, pp. 622–672. Amsterdam: Elsevier Press 1965
Engel, W. K.: Muscle biopsy. Clin. Orthop. 39, 80–105 (1965)
Engel, W. K., Brooke, M. H.: Muscle biopsy as a clinical diagnostic aid. In: W. S. Fields (ed.), Neurological Diagnostic Techniques, pp. 90–146. Springfield (Ill.): Charles C. Thomas 1966
Engel, W. K., Cunningham, G. G.: Alkaline phosphatase-positive abnormal muscle fibers of humans. J. Histochem. Cytochem. 18, 55–57 (1970)
Engel, W. K.: Nouvelle hypothese sur la pathogenie de la dystrophie musculaire pseudohypertrophique de Duchenne. Rev. Neurol. (Paris) 124, 291–298 (1971)
Engel, W. K.: Duchenne muscular dystrophy: A histologically based ischemia hypothesis and comparison with experimental ischemia myopathy. In: The Striated Muscle, pp. 453–472. Baltimore: The Williams and Wilkins Co. 1973
Engel, W. K.: Workshop on the aetiology of Duchenne muscular dystrophy: The vascular hypothesis. In: W. G. Bradley, D. Gardner-Medwin, J. N. Walton (eds.), Recent Advances in Myology, pp. 166–177. New York: American Elsevier Publishing Co., Inc. 1975
Engel, W. K., Derrer, E. C.: Drugs blocking the muscle-damaging effects of 5-HT and noradrenaline in aorta-ligated rats. Nature 254, 151–152 (1975)
Engel, W. K.: Integrative histochemical approach to the defect of Duchenne muscular dystrophy. In: L. P. Rowland (ed.), Pathogenesis of the Human Muscular Dystrophies. New York: American Elsevier Publishing Co. (in press)
Hathaway, P. W., Engel, W. K., Zellweger, H.: Experimental myopathy after microarterial embolization. Arch. Neurol. 22, 365–378 (1970)
Jerusalem, F., Engel, A. G., Gomez, M. R.: Duchenne dystrophy. I. Morphometric study of the muscle microvasculature. Brain 97, 115–122 (1974)
Kunze, K., Berk, H., Bruckel, B.: Alteration of blood flow in diseased muscle. In: B. A. Kakulas (ed.), Clinical Studies in Myology, Part 2, pp. 198–203. New York: American Elsevier Publishing Co., Inc.
Mendell, J. R., Engel, W. K., Derrer, E. C.: Duchenne muscular dystrophy: Functional ischemia reproduces its characteristic lesions. Science 172, 1143–1145 (1971)
Mendell, J. R., Engel, W. K., Derrer, E. C.: Increased plasma enzyme concentrations in rats with functional ischemia of muscle provide a possible model of Duchenne muscular dystrophy. Nature 239, 522–524 (1972)
O'Brien, M. D., Bradley, W. G., Murchison, D., Johnson, M., Walder, D. N.: An esperimental investigation of the vascular hypothesis of the aetiology of muscular dystrophy. IIIrd Int. Cong. on Muscle Diseases. Newcastle-upon-Tyne, Great Britain, Excerpta Medica Int. Cong. Series No. 334, 109–110 (1974)
Paulson, O. B., Engel, A. G., Gomez, M. R.: Muscle blood flow in Duchenne dystrophy, limb-girdle dystrophy, polymyositis and in normal controls. J. Neurol. Neurosurg. Psychiat. 37, 685–690 (1974)
Reznik, M., Engel, W. K.: Ultrastructural and histochemical correlation of experimental muscle regeneration. J. Neurol. Sci. 11, 167–185 (1970)
Siegel, B. A., Engel, W. K., Derrer, E. C.: Localization of 99mTc-diphosphonate in acutely injured muscle: Relationship to muscle calcium deposition. Neurology 27, 230–238 (1977)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Engel, W.K., Hawley, R.J. Focal lesions of muscle in peripheral vascular disease. J. Neurol. 215, 161–168 (1977). https://doi.org/10.1007/BF00312474
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00312474