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Failure to Confirm a Vascular Cause of Muscular Dystrophy

Failure to Confirm a Vascular Cause of Muscular Dystrophy Abstract The vascular hypothesis of the cause of muscular dystrophy suggests that ischemia is responsible for the muscle fiber necrosis. A xenon 133 clearance study of muscle blood flow in Duchenne and other muscular dystrophies showed no obvious difference between the response to exercise and arterial occlusion compared with control subjects. Radioautographic study of distribution of 4-125l-antipyrine in skeletal muscle of mice with muscular dystrophy showed no abnormal areas of ischemia. A statistical examination was also made of the grouping of damaged fibers, one of the observations on which the vascular hypothesis was based. Only 0.9% of fibers undergoing phagocytosis occurred in groups of four or more fibers in greater frequency than would have been expected by chance, and 70% of such fibers were isolated. These studies argue strongly against the vascular hypothesis of the cause of muscular dystrophy. References 1. Demos J: Mésure des temps de circulation chez 79 myopathes . Rev Fr Etud Clin Biol 6:876-887, 1961. 2. Demos J: Un nouveau probléme pose par la myopathie humaine: Les troubles des temps de circulation et leur liaison avec l'activité enzymatique serique . Soc Med Hop Paris 77:636-646, 1961. 3. Demos J: Essai d'appréciation d'une action thérapeutique éventuelle au cours de la myopathie: Etude critique de l'action du p-hydroxyphényl-butyl-amino-ethanol . Sem Hop Paris 39:572-579, 1963. 4. Demos J: Early diagnosis and treatment of rapidly developing Duchenne de Boulogne type myopathy (type DDB I) . Am J Phys Med 50:271-284, 1971. 5. Demos J: Raisons d'une amino-diphenoloxydase dans les plaquettes sanguines humaines: Existance d'un variation de cette enzyme chez les malades atteints de myopathie (Forme de Duchenne de Boulogne) . CR Acad Sci 267:677-680, 1968. 6. Demos J: La détection de porteuses saines du trait myopathique dans la myopathie humaine . Ann Genet 12:191-201, 1969. 7. Hathaway PW, Engel WK, Zellweger H: Experimental myopathy after microarterial embolization: Comparison with childhood X-linked pseudohypertrophic muscular dystrophy . Arch Neurol 22:365-378, 1970.Crossref 8. Mendell JR, Engel WK, Derrer EC: Duchenne muscular dystrophy: Functional ischaemia reproduces its characteristic lesions . Science 172:1143-1145, 1971.Crossref 9. Banker BQ: A phase and electron microscopic study of dystrophic muscle: I. The pathological changes in the 2-week-old Bar Harbor 129 dystrophic mouse . J Neuropathol Exp Neurol 26:259-275, 1967.Crossref 10. Rogers AW: Techniques of Autoradiography . Amsterdam, Elsevier Publishing Co, 1967. 11. Hudlicka O: Muscle Blood Flow: Its Relation to Muscle Metabolism and Function . Amsterdam, Swets & Zeitlinger, 1973. 12. Molyneux L, Turnbull J, Walder DN: A portable apparatus for recording the rate of clearance of radioactive sodium from human calf muscle . J Sci Inst 35:259-261, 1958.Crossref 13. Bradley WG, Jaros E: Axoplasmic flow in axonal neuropathies: II. Axoplasmic flow in mice with motor neuron disease and muscular dystrophy . Brain 96:247-258, 1973.Crossref 14. Plackett RL: The truncated Poisson distribution . Biometrics 8:485-488, 1953.Crossref 15. Lindbjerg IF: Variations in fat content of various skeletal muscles . Scand J Clin Lab Invest Suppl 93:14-17, 1967. 16. Talso PJ, Lahr TN, Spafford N, et al: A comparison of the volume distribution of antipyrine, N-acetyl-4-amino-antipyrine and 131I-labelled 4-iodo-antipyrine in human beings . J Lab Clin Med 46:619-623, 1955. 17. Schultze B: Autoradiography at the cellular level , in Pollister AW (ed): Physical Techniques in Biological Research , ed 2. New York, Academic Press, 1969, vol 3, Part B. 18. Bell GH, Davidson JN, Scarbrough H: Textbook of Physiology and Biochemistry , ed 7. Edinburgh, Livingstone, 1968. 19. Spector WS (ed): Handbook of Biological Data . Philadelphia, WB Saunders, 1956, p 277. 20. Kunze K: Das Sauerstoffdruckfeld im normalen und pathologisch veränderten Muskel. Berlin, Springer-Verlag, 1969. 21. Romanul FCA: Capillary supply and metabolism of muscle fibers . Arch Neurol 12:497-509, 1965.Crossref 22. Engel WK: Nouvelle hypothese sur la pathogenie de la dystrophie musculaire pseudohypertrophique de Duchenne . Rev Neurol 124:291-298, 1971. 23. Mendell JR, Engel WK, Derrer EC: Increased plasma enzyme concentrations in rats with functional ischaemia of muscle provide a possible model of Duchenne muscular dystrophy . Nature 239:523-524, 1972.Crossref 24. Denny-Brown D: Experimental studies pertaining to hypertrophy, regeneration and degeneration . Res Publ Assoc Nerv Ment Dis 38:147-196, 1960. 25. Isaacs ER, Bradley WG, Henderson G: Longitudinal fibre splitting in muscular dystrophy: A serial cinematographic study . J Neurol Neurosurg Psychiatry 36:813-819, 1973.Crossref 26. Jerusalem F, Engel AG, Gomez MR: Duchenne dystrophy: I. Morphometric study of the muscle microvasculature . Brain 97:115-122, 1974.Crossref 27. Paulson OB, Engel AG, Gomez MR: Muscle blood flow in Duchenne type muscular dystrophy, limb-girdle dystrophy, polymyositis, and in normal controls . J Neurol Neurosurg Psychiatry 37:685-690, 1974.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1975 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1975.00490490070007
Publisher site
See Article on Publisher Site

Abstract

Abstract The vascular hypothesis of the cause of muscular dystrophy suggests that ischemia is responsible for the muscle fiber necrosis. A xenon 133 clearance study of muscle blood flow in Duchenne and other muscular dystrophies showed no obvious difference between the response to exercise and arterial occlusion compared with control subjects. Radioautographic study of distribution of 4-125l-antipyrine in skeletal muscle of mice with muscular dystrophy showed no abnormal areas of ischemia. A statistical examination was also made of the grouping of damaged fibers, one of the observations on which the vascular hypothesis was based. Only 0.9% of fibers undergoing phagocytosis occurred in groups of four or more fibers in greater frequency than would have been expected by chance, and 70% of such fibers were isolated. These studies argue strongly against the vascular hypothesis of the cause of muscular dystrophy. References 1. Demos J: Mésure des temps de circulation chez 79 myopathes . Rev Fr Etud Clin Biol 6:876-887, 1961. 2. Demos J: Un nouveau probléme pose par la myopathie humaine: Les troubles des temps de circulation et leur liaison avec l'activité enzymatique serique . Soc Med Hop Paris 77:636-646, 1961. 3. Demos J: Essai d'appréciation d'une action thérapeutique éventuelle au cours de la myopathie: Etude critique de l'action du p-hydroxyphényl-butyl-amino-ethanol . Sem Hop Paris 39:572-579, 1963. 4. Demos J: Early diagnosis and treatment of rapidly developing Duchenne de Boulogne type myopathy (type DDB I) . Am J Phys Med 50:271-284, 1971. 5. Demos J: Raisons d'une amino-diphenoloxydase dans les plaquettes sanguines humaines: Existance d'un variation de cette enzyme chez les malades atteints de myopathie (Forme de Duchenne de Boulogne) . CR Acad Sci 267:677-680, 1968. 6. Demos J: La détection de porteuses saines du trait myopathique dans la myopathie humaine . Ann Genet 12:191-201, 1969. 7. Hathaway PW, Engel WK, Zellweger H: Experimental myopathy after microarterial embolization: Comparison with childhood X-linked pseudohypertrophic muscular dystrophy . Arch Neurol 22:365-378, 1970.Crossref 8. Mendell JR, Engel WK, Derrer EC: Duchenne muscular dystrophy: Functional ischaemia reproduces its characteristic lesions . Science 172:1143-1145, 1971.Crossref 9. Banker BQ: A phase and electron microscopic study of dystrophic muscle: I. The pathological changes in the 2-week-old Bar Harbor 129 dystrophic mouse . J Neuropathol Exp Neurol 26:259-275, 1967.Crossref 10. Rogers AW: Techniques of Autoradiography . Amsterdam, Elsevier Publishing Co, 1967. 11. Hudlicka O: Muscle Blood Flow: Its Relation to Muscle Metabolism and Function . Amsterdam, Swets & Zeitlinger, 1973. 12. Molyneux L, Turnbull J, Walder DN: A portable apparatus for recording the rate of clearance of radioactive sodium from human calf muscle . J Sci Inst 35:259-261, 1958.Crossref 13. Bradley WG, Jaros E: Axoplasmic flow in axonal neuropathies: II. Axoplasmic flow in mice with motor neuron disease and muscular dystrophy . Brain 96:247-258, 1973.Crossref 14. Plackett RL: The truncated Poisson distribution . Biometrics 8:485-488, 1953.Crossref 15. Lindbjerg IF: Variations in fat content of various skeletal muscles . Scand J Clin Lab Invest Suppl 93:14-17, 1967. 16. Talso PJ, Lahr TN, Spafford N, et al: A comparison of the volume distribution of antipyrine, N-acetyl-4-amino-antipyrine and 131I-labelled 4-iodo-antipyrine in human beings . J Lab Clin Med 46:619-623, 1955. 17. Schultze B: Autoradiography at the cellular level , in Pollister AW (ed): Physical Techniques in Biological Research , ed 2. New York, Academic Press, 1969, vol 3, Part B. 18. Bell GH, Davidson JN, Scarbrough H: Textbook of Physiology and Biochemistry , ed 7. Edinburgh, Livingstone, 1968. 19. Spector WS (ed): Handbook of Biological Data . Philadelphia, WB Saunders, 1956, p 277. 20. Kunze K: Das Sauerstoffdruckfeld im normalen und pathologisch veränderten Muskel. Berlin, Springer-Verlag, 1969. 21. Romanul FCA: Capillary supply and metabolism of muscle fibers . Arch Neurol 12:497-509, 1965.Crossref 22. Engel WK: Nouvelle hypothese sur la pathogenie de la dystrophie musculaire pseudohypertrophique de Duchenne . Rev Neurol 124:291-298, 1971. 23. Mendell JR, Engel WK, Derrer EC: Increased plasma enzyme concentrations in rats with functional ischaemia of muscle provide a possible model of Duchenne muscular dystrophy . Nature 239:523-524, 1972.Crossref 24. Denny-Brown D: Experimental studies pertaining to hypertrophy, regeneration and degeneration . Res Publ Assoc Nerv Ment Dis 38:147-196, 1960. 25. Isaacs ER, Bradley WG, Henderson G: Longitudinal fibre splitting in muscular dystrophy: A serial cinematographic study . J Neurol Neurosurg Psychiatry 36:813-819, 1973.Crossref 26. Jerusalem F, Engel AG, Gomez MR: Duchenne dystrophy: I. Morphometric study of the muscle microvasculature . Brain 97:115-122, 1974.Crossref 27. Paulson OB, Engel AG, Gomez MR: Muscle blood flow in Duchenne type muscular dystrophy, limb-girdle dystrophy, polymyositis, and in normal controls . J Neurol Neurosurg Psychiatry 37:685-690, 1974.Crossref

Journal

Archives of NeurologyAmerican Medical Association

Published: Jul 1, 1975

References