Comprehensive Physiology Wiley Online Library

Diseases of Skeletal Muscle

Full Article on Wiley Online Library



Abstract

The sections in this article are:

1 History
2 Clinical Presentation of Muscle Disease
2.1 Weakness
2.2 Fatigue
2.3 Myoglobinuria
2.4 Unusual Function
3 Investigation of Patients with Muscle Disease
3.1 Clinical History and Examination
3.2 Blood Biochemistry
3.3 Electromyography
3.4 Muscle Biopsy
3.5 Nuclear Magnetic Resonance
3.6 Measurement of Muscle Cross‐Sectional Area
3.7 Tests of Muscle Function
3.8 Isolated Human Muscle Preparations
3.9 Exercise and Fasting as Provocation Tests
3.10 Sudies of Protein Turnover
4 Analysis of Muscle Weakness
4.1 Dystrophies
4.2 Inflammatory Myopathies
4.3 Atrophy and Hypertrophy
4.4 Factors Affecting Protein Turnover in Muscle
4.5 Neuropathies
5 Analysis of Abnormal Fatigability
5.1 Muscle Fatigue in Normal Subjects
5.2 Myasthenia Gravis
5.3 Disorders of Energy Metabolism
5.4 Disorders of Mitochondrial Oxidative Metabolism
5.5 Disorders of Fat Metabolism
5.6 Changes in Muscle Function
6 Disordered Function
6.1 Myotonic Syndromes
6.2 Periodic Paralysis
6.3 Malignant Hyperpyrexia
6.4 Muscle Pain, Cramps, and Contractures
7 Drug‐Induced Myopathies
8 Mechanisms Underlying Muscle Disease
8.1 Vascular and Humoral Influences
8.2 Influence of Nerve on Muscle
8.3 Disorders of the Cell Membrane
8.4 Muscle Protease Activity
8.5 Inflammatory Disorders of Muscle
8.6 Overuse and Disuse as Causes of Muscle Damage
9 Prospects For Treatment of Muscle Disease
Figure 1. Figure 1.

Computerized tomography in a male West Indian (age 44 yr) with limb‐girdle dystrophy. Top: cross sections in midthigh region. There is partial replacement of quadriceps muscle and almost complete replacement of posterior thigh (hamstring) muscles with fat. Bottom: cross sections in region of maximum calf circumference. There is hypertrophy of anterior tibial muscles, preservation of gastrocnemius, and replacement of soleus muscle by fat.

Figure 2. Figure 2.

Abnormal response to exercise of a patient with defect of mitochondrial metabolism. ▪, Normal subject; work done = 196 W (70% max), □, Patient with a defect in mitochondrial pyruvate metabolism; work done = 65 W (59% max). Note that despite doing less absolute and relative work, patient had a much greater rise in plasma lactate, which was slow to recover. [From Edwards et al. ]

Figure 3. Figure 3.

Dystrophic changes in muscle: cross sections of quadriceps muscle stained with hematoxylin‐eosin. A: normal (male, 22 yr). B: early dystrophy (male, 9 yr); note internal nuclei and variations in fiber size. The overall difference in fiber size between A and B reflects age difference between the 2 subjects and is not abnormal. C: advanced dystrophy (male, 17 yr); note loss of contractile material, which is replaced by fat and fibrous tissue. Scale bar = 50 μm.

Figure 4. Figure 4.

Cross section of quadriceps muscle in patient with polymyositis (female, 48 yr); note marked differences in fiber size, internal nuclei, and infiltration with macrophages. Scale bar = 50 μm.

Figure 5. Figure 5.

Atrophic changes. Cross sections of quadriceps muscle stained for myosin ATPase at pH 9.4. Type I fibers, light staining; type II fibers, dark staining. A: normal male. B: male patient showing type II fiber atrophy after steroid therapy. C: normal female. D: female with Cushing's syndrome showing severe type II fiber atrophy. Scale bar = 50 μm.

Figure 6. Figure 6.

Consequences of muscle denervation. Low‐power views of cross sections of quadriceps muscle, stained for myosin ATPase at pH 9.4. A: normal muscle; note the even distribution of type I and II fibers. B: muscle from a patient with a neuropathy. A number of small atrophied fibers can be seen; these are a consequence of denervation. Main feature is pronounced fiber grouping, which is due to reinnervation of fibers by sprouting of nearby surviving motor nerve axons. Scale bar = 500 μm.

Figure 7. Figure 7.

Force generated by percutaneous stimulation of quadriceps muscle at different frequencies. Muscle was stimulated with 5 impulses at 1 Hz and then for 2 s successively at 10, 20, 50, and 100 Hz. A: fresh, rested muscle. B: same muscle tested after 10‐min rest after a 20‐min step test. Note that although maximum tetanic force is nearly fully recovered, force generated at low frequencies is much reduced.



Figure 1.

Computerized tomography in a male West Indian (age 44 yr) with limb‐girdle dystrophy. Top: cross sections in midthigh region. There is partial replacement of quadriceps muscle and almost complete replacement of posterior thigh (hamstring) muscles with fat. Bottom: cross sections in region of maximum calf circumference. There is hypertrophy of anterior tibial muscles, preservation of gastrocnemius, and replacement of soleus muscle by fat.



Figure 2.

Abnormal response to exercise of a patient with defect of mitochondrial metabolism. ▪, Normal subject; work done = 196 W (70% max), □, Patient with a defect in mitochondrial pyruvate metabolism; work done = 65 W (59% max). Note that despite doing less absolute and relative work, patient had a much greater rise in plasma lactate, which was slow to recover. [From Edwards et al. ]



Figure 3.

Dystrophic changes in muscle: cross sections of quadriceps muscle stained with hematoxylin‐eosin. A: normal (male, 22 yr). B: early dystrophy (male, 9 yr); note internal nuclei and variations in fiber size. The overall difference in fiber size between A and B reflects age difference between the 2 subjects and is not abnormal. C: advanced dystrophy (male, 17 yr); note loss of contractile material, which is replaced by fat and fibrous tissue. Scale bar = 50 μm.



Figure 4.

Cross section of quadriceps muscle in patient with polymyositis (female, 48 yr); note marked differences in fiber size, internal nuclei, and infiltration with macrophages. Scale bar = 50 μm.



Figure 5.

Atrophic changes. Cross sections of quadriceps muscle stained for myosin ATPase at pH 9.4. Type I fibers, light staining; type II fibers, dark staining. A: normal male. B: male patient showing type II fiber atrophy after steroid therapy. C: normal female. D: female with Cushing's syndrome showing severe type II fiber atrophy. Scale bar = 50 μm.



Figure 6.

Consequences of muscle denervation. Low‐power views of cross sections of quadriceps muscle, stained for myosin ATPase at pH 9.4. A: normal muscle; note the even distribution of type I and II fibers. B: muscle from a patient with a neuropathy. A number of small atrophied fibers can be seen; these are a consequence of denervation. Main feature is pronounced fiber grouping, which is due to reinnervation of fibers by sprouting of nearby surviving motor nerve axons. Scale bar = 500 μm.



Figure 7.

Force generated by percutaneous stimulation of quadriceps muscle at different frequencies. Muscle was stimulated with 5 impulses at 1 Hz and then for 2 s successively at 10, 20, 50, and 100 Hz. A: fresh, rested muscle. B: same muscle tested after 10‐min rest after a 20‐min step test. Note that although maximum tetanic force is nearly fully recovered, force generated at low frequencies is much reduced.

References
 1. Adrian, R. H. The effect of internal and external potassium concentration on the membrane potential of frog muscle. J. Physiol. London 133: 631–658, 1956.
 2. Adrian, R. H., and S. H. Bryant. On the repetitive discharge in myotonic muscle fibres. J. Physiol. London 240: 505–515, 1974.
 3. Adrian, R. H., and M. W. Marshall. Action potentials reconstructed in normal and myotonic muscle fibres. J. Physiol. London 258: 125–143, 1976.
 4. Adrian, R. H., and L. D. Peachey. Reconstruction of the action potential of frog sartorius muscle. J. Physiol. London 235: 103–131, 1973.
 5. Aitken, R. S., E. N. Allott, L. I. M. Castelden, and M. Walker. Observations on a case of familial periodic paralysis. Clin. Sci. 3: 47–57, 1937.
 6. Albuquerque, E. X., J. E. Rash, R. F. Mayer, and R. Sutterfield. An electrophysiological and morphological study of the neuromuscular junction in patients with myasthenia gravis. Exp. Neurol. 51: 536–563, 1976.
 7. Albuquerque, E. X., and S. Thesleff. A. comparative study of membrane properties of innervated and chronically denervated fast and slow skeletal muscles of the rat. Acta Physiol. Scand. 73: 471–480, 1968.
 8. Allott, E. N., and B. McArdle. Further observations on familial periodic paralysis. Clin. Sci. 3: 229–239, 1938.
 9. Amphlett, G. W., S. V. Perry, H. Syska, M. D. Brown, and G. Vrbova. Cross innervation and the regulatory protein system of rabbit soleus muscle. Nature London 257: 602–604, 1975.
 10. Andersen, D. M. Familial cirrhosis of the liver with storage of abnormal glycogen. Lab. Invest. 5: 11–20, 1956.
 11. Angelini, C., and A. G. Engel. Subcellular distribution of acid and neutral α‐glucosidase in normal, acid maltase deficient and myophosphorylase deficient human skeletal muscle. Arch. Biochem. 156: 350–355, 1973.
 12. Angelini, C., S. Lucre, and F. Cantarutti. Carnitine deficiency of skeletal muscle: report of a treated case. Neurology 26: 633–637, 1976.
 13. Asmussen, E. Observations on experimental muscular soreness. Acta Rheumatol. Scand. 2: 109–116, 1956.
 14. Austin, K. L., and M. A. Denborough. Drug treatment of malignant hyperpyrexia. Anaesth. Intensive Care 5: 207–213, 1977.
 15. Ballantyne, J. P., and S. Hansen. Neurogenic influence in muscular dystrophies. In: Pathogenesis of Human Muscular Dystrophies, edited by L. P. Rowland. Amsterdam: Excerpta Med., 1978, p. 187–199.
 16. Ballard, F. J., F. M. Tomas, and L. M. Stern. Increased turnover of muscle contractile proteins in Duchenne muscular dystrophy as assessed by 3‐methylhistidine and creatinine excretion. Clin. Sci. 56: 347–352, 1979.
 17. Baloh, R., P. A. Cancilla, K. Kalyanaraman, T. Munsat, C. M. Pearson, and R. Rich. Regeneration of human muscle. A morphologic and histochemical study of normal and dystrophic muscle after injury. Lab. Invest. 26: 319–328, 1972.
 18. Bank, W. J., S. Di Mauro, and L. P. Rowland. Renal failure in McArdle's disease. N. Engl. J. Med. 287: 1102, 1972.
 19. Barchi, R. L. Molecular structure and biophysical aspects of the sarcolemma. In: Current Topics in Nerve and Muscle Research, edited by A. J. Aguayo and G. Karpati. Amsterdam: Excerpta Med., 1979, p. 3–15. (Int. Congr. Ser. 455.)
 20. Bergström, J. Muscle electrolytes in man: determined by neutron activation analysis on needle biopsy specimens: a study in normal subjects, kidney patients and patients with chronic diarrhoea. Scand. J. Clin. Lab. Invest. Suppl. 68: 1–110, 1962.
 21. Bezanilla, F., and C. M. Armstrong. Negative conductance caused by entry of sodium and cesium ions into potassium channels of squid axon. J. Gen. Physiol. 60: 588–608, 1972.
 22. Bigland‐Ritchie, B., D. A. Jones, G. P. Hosking, and R. H. T. Edwards. Central and peripheral fatigue in sustained maximum voluntary contractions of human quadriceps muscle. Clin. Sci. Mol. Med. 54: 609–614, 1978.
 23. Bigland‐Ritchie, B., D. A. Jones, and J. J. Woods. Excitation frequency and muscle fatigue: electrical responses during human voluntary and stimulated contractions. Exp. Neurol. 64: 414–427, 1979.
 24. Blass, J. P. Pyruvate dehydrogenase deficiencies. In: Inherited Disorders of Carbohydrate Metabolism, edited by D. Burman, J. B. Halton, and C. A. Pennock. Lancaster, UK: MTP, 1980, p. 239–268.
 25. Bodensteiner, J. B., and A. G. Engel. Intracellular calcium accumulation in Duchenne dystrophy and other myopathies: a study of 567,000 muscle fibers in 114 biopsies. Neurology 28: 439–446, 1978.
 26. Bohmer, T., H. Bergrem, and K. Eiklid. Carnitine deficiency induced during intermittent haemodialysis for renal failure. Lancet 1: 126–128, 1978.
 27. Border, J. R., U. P. Burns, C. Rumph, and W. G. Schenk. Carnitine levels in severe infection and starvation; a possible key to the prolonged catabolic state. Surgery 68: 175–179, 1970.
 28. Borenstein, S., and J. E. Desmedt. Local cooling in myasthenia: improvement of neuromuscular failure. Arch. Neurol. Chicago 32: 152–157, 1975.
 29. Bouwsma, G., and G. K. Van Wijngaarden. Spinal muscular atrophy and hypertrophy of the calves. J. Neurol. Sci. 44: 275–279, 1980.
 30. Bowman, W. C., and M. W. Nott. Action of sympathomimetic amines and their antagonists on skeletal muscle. Pharm. Rev. 21: 27–72, 1969.
 31. Bradley, W. G. Adynamia episodica hereditaria. Clinical, pathological and electrophysiological studies in an affected family. Brain 92: 345–378, 1969.
 32. Bradley, W. G. Ultrastructural changes in adynamia episodica hereditaria and normokalaemic familial periodic paralysis. Brain 92: 379–390, 1969.
 33. Bradley, W. G. The neuropathies. In: Disorders of Voluntary Muscle, edited by J. N. Walton. Edinburgh: Churchill Livingstone, 1974, p. 804–851.
 34. Bradley, W. G., P. Hudgson, D. Gardner‐Medwin, and J. N. Walton. Myopathy associated with abnormal lipid metabolism in skeletal muscle. Lancet 1: 495–498, 1969.
 35. Bradley, W. G., M. Jenkinson, D. C. Park, P. Hudgson, D. Gardner‐Medwin, R. J. T. Pennington, and J. N. Walton. A myopathy associated with lipid storage. J. Neurol. Sci. 16: 137–154, 1972.
 36. Bradley, W. G., and P. K. Thomas. The pathology of peripheral nerve disease. In: Disorders of Voluntary Muscle, edited by J. N. Walton. Edinburgh: Churchill Livingstone, 1974, p. 234–275.
 37. Brandt, N. J., F. Buchthal, F. Ebbesen, Z. Kamieniecka, and C. Krarup. Post‐tetanic mechanical tension and evoked action potentials in McArdle's disease. J. Neurol. Neurosurg. Psychiatry 40: 920–925, 1977.
 38. Bremer, J. Carnitine and its role in fatty acid metabolism. Trends Biochem. Sci. 2: 207–209, 1977.
 39. Bressler, R., and B. Wittles. The effect of diphtheria toxin on carnitine metabolism in the heart. Biochim. Biophys. Acta 104: 39–45, 1965.
 40. Britt, B. A. Etiology and pathophysiology of malignant hyperthermia. Federation Proc. 38: 44–48, 1979.
 41. Britt, B. A., and W. Kalow. Malignant hyperthermia: etiology unknown. Can. Anaesth. Soc. J. 17: 316–330, 1970.
 42. Brooke, M. H. A Clinician's View of Neuromuscular Diseases. Baltimore, MD: Williams & Wilkins, 1977.
 43. Brooke, M. H., J. E. Carroll, J. E. Davies, and J. M. Hagberg. The prolonged exercise test. Neurology 29: 636–643, 1979.
 44. Brooks, J. E. Hyperkalemic periodic paralysis. Intracellular electromyographic studies. Arch. Neurol. Chicago 20: 13–18, 1969.
 45. Brown, G. L., and A. M. Harvey. Congenital myotonia in the goat. Brain 62: 341–363, 1939.
 46. Bryant, S. H. Muscle membrane of normal and myotonic goats in normal and low external chloride (Abstract). Federation Proc. 21: 312, 1962.
 47. Bryant, S. H. Cable properties of external intercostal muscle fibres from myotonic and nonmyotonic goats. J. Physiol. London 204: 539–550, 1969.
 48. Bryant, S. H. The electrophysiology of myotonia; with a review of congenital myotonia of goats. In: New Developments in Electromyography and Clinical Neurophysiology. New Concepts of the Motor Unit Neuromuscular Disorders, Electromyographic Kinesiology, edited by J. E. Desmedt. Basel: Karger, 1973, vol. 1, p. 420–450.
 49. Bryant, S. H. Myotonia in the goat. Ann. NY Acad. Sci. 317: 314–325, 1979.
 50. Bryant, S. H., and D. Camerino. Chloride conductance of denervated gastrocnemius fibers from normal goats. J. Neurobiol. 7: 229–240, 1976.
 51. Bryant, S. H., and A. Morales‐Aguilera. Chloride conductance in normal and myotonic muscle fibers and the action of monocarboxylic aromatic acids. J. Physiol. London 219: 367–383, 1971.
 52. Buchthal, F., L. Engbaeck, and I. Gamstorp. Some aspects of the pathophysiology of adynamia episodica hereditaria. Dan. Med. Bull. 5: 167–169, 1958.
 53. Bulcke, J. A., J. Demeirsman, and J. L. Termote. The influence of skeletal muscle atrophy on needle electromyography. As demonstrated by computed tomography. Electromyogr. Clin. Neurophysiol. 19: 269–279, 1979.
 54. Buller, A. J., J. C. Eccles, and R. M. Ecoles. Interactions between motoneurones and muscles in respect of the characteristic speeds of their responses. J. Physiol. London 150: 417–439, 1960.
 55. Buller, A. J., W. F. H. M. Mommaerts, and K. Seraydarian. Enzymic properties of myosin in fast and slow twitch muscles of the cat following cross‐innervation. J. Physiol. London 205: 581–597, 1969.
 56. Burack, R., R. H. T. Edwards, M. Green, and N. L. Jones. The response to exercise before and after treatment of myxedema with thyroxine. J. Pharmacol. Exp. Ther. 176: 212–219, 1971.
 57. Burke, D., N. F. Skuse, and A. K. Lethlean. Isometric contraction of the abductor digiti minimi muscle in man. J. Neurol. Neurosurg. Psychiatry 37: 825–834, 1974.
 58. Busch, W. A., M. H. Stromer, D. E. Goll, and A. Suzuki. Ca2+‐specific removal of Z lines from rabbit skeletal muscle. J. Cell Biol. 52: 367–381, 1972.
 59. Camerino, D., and S. H. Bryant. Effects of denervation and colchicine treatment on the chloride conductance of rat skeletal muscle fibers. J. Neurobiol. 7: 221–228, 1976.
 60. Campbell, E. J. M., R. H. T. Edwards, D. K. Hill, D. A. Jones, and M. K. Sykes. Perception of effort during partial curarisation. J. Physiol. London 263: 186P–187P, 1976.
 61. Campbell, H., and E. Bramwell. Myasthenia gravis. Brain 23: 277–336, 1900.
 62. Carpenter, S., and G. Karpati. Duchenne muscular dystrophy: plasma membrane loss initiates muscle cell necrosis unless it is repaired. Brain 102: 147–161, 1979.
 63. Carrier, H. N., and G. Berthillier. Carnitine levels in normal children and adults and in patients with diseased muscles. Muscle Nerve 3: 326–334, 1980.
 64. Carroll, J. E., M. H. Brooke, D. C. De Vivo, K. K. Kaiser, and J. M. Hagberg. Biochemical and physiologic consequences of carnitine palmityltransferase deficiency. Muscle Nerve 1: 103–110, 1978.
 65. Carroll, J. E., M. H. Brooke, D. C. De Vivo, J. B. Shumate, R. Kratz, S. P. Ringel, and J. M. Hagberg. Carnitine deficiency': lack of response to carnitine therapy. Neurology 30: 618–626, 1980.
 66. Carroll, J. E., D. C. De Vivo, M. H. Brooke, G. J. Planer, and J. H. Hagberg. Fasting as a provocative test in neuromuscular diseases. Metabolism 28: 683–687, 1979.
 67. Carroll, J. E., J. M. Hagberg, M. H. Brooke, and J. B. Shumate. Bicycle ergometry and gas exchange measurements in neuromuscular diseases. Arch. Neurol. Chicago 36: 457–461, 1979.
 68. Carson, M. J., and C. M. Pearson. Familial hyperkalemic periodic paralysis with myotonic features. J. Pediatr. 64: 853–865, 1964.
 69. Chalovich, J. M., C. T. Burt, M. J. Danon, T. Glonek, and M. Bárány. Phosphodiesters in muscular dystrophies. Ann. NY Acad. Sci. 317: 649–669, 1979.
 70. Charviére, M., and G. B. Duchenne. Emporte pièce histologique. Bull. Acad. Natl. Med. Paris 30: 1050–1051, 1865.
 71. Chelmicka‐Schorr, E. E., B. G. W. Arnason, K. E. Astrom, and Z. Darzynkiewico. Treatment of mouse muscular dystrophy with the protease inhibitor pepstatin. J. Neuropathol. Exp. Neurol. 37: 263–269, 1978.
 72. Chui, L. A., T. L. Munsat, and J. R. Craig. Effect of ethanol on lactic acid production by excised normal muscle. Muscle Nerve 1: 57–61, 1978.
 73. Conway, E. J. Nature and significance of concentration relations of potassium and sodium ions in skeletal muscle. Physiol. Rev. 37: 84–132, 1957.
 74. Conway, E. J., and D. Hingerty. Relations between potassium and sodium levels in mammalian muscle and blood plasma. Biochem. J. 42: 372–376, 1948.
 75. Costill, D. L., W. J. Fink, and M. L. Pollock. Muscle fiber composition and enzyme activities of elite distance runners. Med. Sci. Sports 8: 96–100, 1976.
 76. Cox, R. A., and C. L. Hoppel. Biosynthesis of carnitine and 4‐N‐trimethylaminobutyrate from lysine. Biochem. J. 136: 1075–1082, 1973.
 77. Cresshull, I., M. J. Dawson, R. H. T. Edwards, D. G. Gadian, G. K. Gordon, D. Shaw, and D. R. Wilkie. Human muscle analysed by 31P nuclear magnetic resonance in intact subjects. J. Physiol. London: 317: 18P, 1981.
 78. Creutzfeldt, O. B., B. C. Abbot, W. M. Fowler, and E. M. Pearson. Muscle membrane potentials in episodic adynamia. Electroencephalogr. Clin. Neurophysiol. 15: 508–519, 1963.
 79. Cull‐Candy, S. G., R. Miledi, and A. Trautmann. End‐plate currents and acetylcholine noise at normal and myasthenic human end‐plates. J. Physiol. London 287: 247–265, 1979.
 80. Cullen, M. J., and J. J. Fulthorpe. Stages in fibre breakdown in Duchenne muscular dystrophy. An electron‐microscopic study. J. Neurol. Sci. 24: 179–200, 1975.
 81. Dahlback, D., D. Elmqvist, T. R. Johns, S. Rudner, and S. Thesleff. An electrophysiologic study of the neuromuscular junction in myasthenia gravis. J. Physiol. London 156: 336–343, 1961.
 82. Dastur, D. K., B. M. Gagrat, N. H. Wadia, M. M. Desai, and E. P. Bharucha. Nature of muscular change in osteomalacia: light‐ and electron‐microscope observations. J. Pathol. 117: 211–228, 1975.
 83. Dau, P. C. Plasmapheresis therapy in myasthenia gravis. Muscle Nerve 3: 468–482, 1980.
 84. Dawkins, R. L. Experimental myositis associated with hypersensitivity to muscle. J. Pathol. Bacteriol. 90: 619–625, 1965.
 85. Dawson, M. J., D. G. Gadian, and D. R. Wilkie. Mechanical relaxation rate and metabolism studied in fatiguing muscle by NMR. J. Physiol. London 299: 465–484, 1980.
 86. Dayton, W. R., D. E. Goll, M. G. Zeece, R. M. Robson, and W. J. Reville. A Ca2+‐activated protease possibly involved in myofibrillar protein turnover. Purification from porcine muscle. Biochemistry 15: 2150–2158, 1976.
 87. Dayton, W. R., W. J. Reville, D. E. Goll, and M. H. Stromer. A Ca2+‐activated protease possibly involved in myofibrillar protein turnover. Partial characterization of the purified enzyme. Biochemistry 15: 2159–2167, 1976.
 88. De Coursey, T. E., S. G. Younkin, and S. H. Bryant. Neural control of chloride conductance in rat extensor digitorum longus muscle. Exp. Neurol. 61: 705–709, 1978.
 89. De Graeff, J., and L. D. F. Lameijer. Periodic paralysis. Am. J. Med. 39: 70–80, 1965.
 90. De Lateur, B. J., and R. M. Giaconi. Effect on maximal strength of submaximal exercise in Duchenne muscular dystrophy. Am. J. Phys. Med. 58: 26–36, 1979.
 91. Demos, J. Measure des temps de circulation chez 79 myopathies étude statistique des résultats; rôle du degré de l'atteinte musculaire clinique du mode evolutif de la maladie du sexe du malade des saisons. Rev. Fr. Étud. Clin. Biol. 6: 876–887, 1961.
 92. Denborough, M. A., and R. R. H. Lovell. Anaesthetic deaths in a family. Lancet 2: 45, 1960.
 93. Denny‐Brown, D., and S. Nevin. The phenomenon of myotonia. Brain 64: 1–18, 1941.
 94. Desmedt, J. E. The neuromuscular disorder in myasthenia gravis. 1. Electrical and mechanical response to nerve stimulation in hand muscles. In: New Developments in Electromyography and Clinical Neurophysiology. New Concepts of the Motor Unit, Neuromuscular Disorders, Electromyographic Kinesiology, edited by J. E. Desmedt. Basel: Karger, 1973, vol. 1, p. 241–304.
 95. Desmedt, J. E. The neuromuscular disorder in myasthenia gravis. 2. Presynaptic cholinergic metabolism, myasthenia‐like syndromes and a hypothesis. In: New Developments in Electromyography and Clinical Neurophysiology. New Concepts of the Motor Unit, Neuromuscular Disorders, Electromyographic Kinesiology, edited by J. E. Desmedt. Basel: Karger, 1973, vol. 1, p. 305–342.
 96. Desmedt, J. E., and S. Borenstein. Diagnosis of myasthenia gravis by nerve stimulation. Ann. NY Acad. Sci. 274: 174–188, 1976.
 97. Desmedt, J. E., B. Emeryk, P. Renoirte, and K. Hainant. Disorder of muscle contraction processes in sex‐linked (Duchenne) muscular dystrophy, with correlative electromyographic study of myopathic involvement in small hand muscles. Am. J. Med. 45: 853–872, 1968.
 98. Di Donato, S., F. Cornelio, L. Pacini, D. Peluchetti, M. Rimoldi, and S. Spreafico. Muscle carnitine palmityltransferase deficiency: a case with enzyme deficiency in cultured fibroblasts. Ann. Neurol. 4: 465–467, 1978.
 99. Di Mauro, S. Metabolic myopathies. In: Handbook of Clinical Neurology. Diseases of the Muscle, edited by P. J. Vinken and G. W. Bruyn. Amsterdam: Elsevier, 1979, vol. 41, p. 175–234.
 100. Di Mauro, S., S. Arnold, A. Miranada, and L. P. Rowland. McArdle disease: the mystery of reappearing Phosphorylase activity in muscle culture—a fetal isoenzyme. Ann. Neurol. 3: 60–66, 1978.
 101. Di Mauro, S., and P. M. M. Dimauro. Muscle carnitine palmityltransferase deficiency and myoglobinuria. Science 182: 929–931, 1973.
 102. Di Mauro, S., and A. B. Eastwood. Disorders of glycogen and lipid metabolism. Adv. Neurol. 17: 123–142, 1977.
 103. Di Mauro, S., L. Z. Stern, M. Mehler, R. B. Nagle, and C. Payne. Adult‐onset acid maltase deficiency: a postmortem study. Muscle Nerve 1: 27–36, 1978.
 104. Di Mauro, S., C. Trevisan, and A. Hays. Disorders of lipid metabolism in muscle. Muscle Nerve 3: 369–388, 1980.
 105. Donaldson, S. K. B., L. Hermansen, and L. Bolles. Differential, direct effects of H+ on Ca2+‐activated force of skinned fibres from the soleus, cardiac and adductor magnus muscles of rabbits. Pfluegers Arch. 376: 55–65, 1978.
 106. Dons, B., K. Bollerup, F. Bond‐Petersen, and S. Hancke. The effect of weight‐lifting exercise related to muscle fibre composition and muscle cross‐sectional area in humans. Eur. J. Appl. Physiol. Occup. Physiol. 40: 95–106, 1979.
 107. Drachman, D. B., C. W. Angus, R. N. Adams, and I. Kao. Effect of myasthenic patients' immunoglobulin on acetylcholine receptor turnover: selectivity of degradation process. Proc. Natl. Acad. Sci. USA 75: 3422–3426, 1978.
 108. Drachman, D. B., C. W. Angus, R. N. Adams, J. D. Michelson, and G. J. Hoffman. Myasthenia antibodies cross‐link acetylcholine receptors to accelerate degradation. N. Engl. J. Med. 298: 1116–1122, 1978.
 109. Drachman, D. B., and F. Witzke. Trophic regulation of acetylcholine sensitivity of muscle: effect of electrical stimulation. Science 176: 514–516, 1972.
 110. Dreyfus, J. C., and G. Schapira. Biochemistry of progressive muscular dystrophy. In: Biochemistry of Hereditary Myopathies. Springfield, IL: Thomas, 1962.
 111. Dreyfus, J. C., G. Schapira, and F. Schapira. Biochemical study of muscle in progressive muscular dystrophy. J. Clin. Invest. 33: 794–797, 1954.
 112. Dubowitz, V. Muscle Disorders in Childhood. Philadelphia, PA: Saunders, 1978.
 113. Dubowitz, V., and M. H. Brooke. Muscle Biopsy: Modern Approach. Philadelphia, PA: Saunders, 1973. (Major Problems in Neurology, Ser. 2.)
 114. Dubowitz, V., and J. Heckmatt. Management of muscular dystrophy. Br. Med. Bull. 36: 139–144, 1980.
 115. Dubowitz, V., and A. G. E. Pearce. Enzymic activity of normal and diseased human muscle: a histochemical study. J. Pathol. Bacteriol. 81: 365–378, 1961.
 116. Duchenne, G. B. De électrisation localisée et son application à la pathologie et à la thérapeutique (2nd ed.). Paris: Baillière, 1861.
 117. Duchenne, G. B. Physiologie des mouvements demontrée à l'aide de l'expérimentation électrique et de l'observation clinique et applicable a l'étude des paralysies et deformation (1867), transl, by E. B. Kaplan. Philadelphia, PA: Saunders, 1959.
 118. Duchenne, G. B. Recherche sur la paralysie musculaire pseudohypertrophique ou paralysie myo‐sclérosique. Arch. Gen. Med. 2: 200–209, 1868.
 119. Dyken, M. L., D. M. Smith, and R. L. Peake. An electromyographic diagnostic screening test in McArdle's disease and a case report. Neurology 17: 45–50, 1967.
 120. Ebashi, S., and H. Sugita. The role of calcium in physiological and pathological processes of skeletal muscle. In: Current Topics in Nerve and Muscle Research, edited by A. J. Aguayo and G. Karpati. Amsterdam: Excerpta Med., 1979, p. 73–84. (Int. Congr. Ser. 455.)
 121. Ebashi, S., Y. Toyokuray, H. Momoi, and H. Sugita. High creatine Phosphokinase activity of sera of progressive muscular dystrophy patients. J. Biochem. Tokyo 46: 103–104, 1959.
 122. Edström, L. Selective atrophy of red muscle fibers in the quadriceps in long‐standing knee‐joint dysfunction. Injuries to the anterior cruciate ligament. J. Neurol. Sci. 11: 551–558, 1970.
 123. Edström, L., and B. Ekblom. Difference in sizes of red and white muscle fibers in vastus lateralis of muscular quadriceps femoris of normal individuals and athletes: relation to physical performance. Scand. J. Clin. Lab. Invest. 3: 155–160, 1974.
 124. Edström, L., and R. Nordemar. Differential changes in typeand type II muscle fibers in rheumatoid arthritis. A biopsy study. Scand. J. Rheumatol. 3: 155–160, 1974.
 125. Edwards, R. H. T. Studies of muscular performance in normal and dystrophic subjects. Br. Med. Bull. 36: 159–164, 1980.
 126. Edwards, R. H. T., R. C. Harris, and D. A. Jones. The biochemistry of muscle biopsy in man: clinical application. In: Recent Advances in Clinical Biochemistry, No. 2, edited by K. G. M. M. Alberti and C. Price. Edinburgh: Churchill Livingstone, 1981, vol. 2, p. 242–269.
 127. Edwards, R. H. T., D. K. Hill, and D. A. Jones. Effect of fatigue on the time course of relaxation from isometric contractions of skeletal muscle in man. J. Physiol. London 227: 26P–27P, 1972.
 128. Edwards, R. H. T., D. K. Hill, and D. A. Jones. Metabolic changes associated with slow relaxation in fatigued mouse muscle. J. Physiol. London 251: 287–301, 1975.
 129. Edwards, R. H. T., D. K. Hill, D. A. Jones, and P. A. Merton. Fatigue of long duration in human skeletal muscle after exercise. J. Physiol. London 272: 769–778, 1977.
 130. Edwards, R. H. T., C. M. Maunder, P. D. Lewis, and A. G. E. Pearse. Percutaneous needle biopsy in the diagnosis of muscle diseases. Lancet 2: 1070–1071, 1973.
 131. Edwards, R. H. T., C. M. Wiles, K. Gohil, S. Krywawych, and D. A. Jones. Energy metabolism in human myopathy. In: Disorders of the Motor Unit, edited by D. Schottland. Boston, MA: Wiley, 1981, p. 715–728.
 132. Edwards, R. H. T., C. M. Wiles, J. M. Round, M. J. Jackson, and A. Young. Muscle breakdown and repair in polymyositis: a case study. Muscle Nerve 2: 223–228, 1979.
 133. Edwards, R. H. T., D. R. Wilkie, M. J. Dawson, R. E. Gordon, and D. Shaw. Clinical use of nuclear magnetic resonance in the investigation of myopathy. Lancet 2: 725–731, 1982.
 134. Edwards, R. H. T., A. Young, G. P. Hosking, and D. A. Jones. Human skeletal muscle function: description of tests and normal values. Clin. Sci. Mol. Med. 52: 283–290, 1977.
 135. Edwards, R. H. T., A. Young, and C. M. Wiles. Needle biopsy of skeletal muscle in the diagnosis of myopathy and the clinical study of muscle function and repair. N. Engl. J. Med. 302: 261–271, 1980.
 136. Ekstedt, J. Human single fiber action potentials. Acta Physiol. Scand. Suppl. 226: 1–96, 1964.
 137. Ellis, F. R., and P. J. Halsall. Malignant hyperpyrexia. Br. J. Hosp. Med. 24: 318–327, 1980.
 138. Ellis, F. R., D. G. F. Harriman, S. Currie, and S. Cain. Screening for malignant hyperthermia in susceptible patients. In: International Symposium on Malignant Hyperthermia, 2nd: Proceedings, edited by J. A. Aldrete and B. A. Britt. New York: Grune & Stratton, 1978, p. 273–285.
 139. Ellis, K. O., and S. H. Bryant. Excitation‐contraction uncoupling in skeletal muscle by dantrolene sodium. Naunyn‐Schmiedeberg's Arch. Pharmacol. 274: 107–109, 1972.
 140. Elmqvist, D. Neuromuscular transmission defects. In: New Developments in Electromyography and Clinical Neurophysiology. New Concepts of the Motor Unit, Neuromuscular Disorders, Electromyographic Kinesiology, edited by J. E. Desmedt. Basel: Karger, 1973, vol. 1, p. 229–240.
 141. Elmqvist, D., W. W. Hofman, J. Kugelberg, and D. M. J. Quastel. An electrophysiological investigation of neuromuscular transmission in myasthenia gravis. J. Physiol. London 174: 414–434, 1964.
 142. Engel, A. G., and C. Angelini. Carnitine deficiency of human skeletal muscle with associated lipid storage myopathy: a new syndrome. Science 179: 899–902, 1973.
 143. Engel, A. G., and E. H. Lambert. Calcium activation of electrically inexcitable muscle fibers in primary hypokalemic periodic paralysis. Neurology 19: 851–858, 1969.
 144. Engel, A. G., E. H. Lambert, and F. M. Howard. Immune complexes IgG and C3 at the motor end‐plate in Myasthenia Gravis. Mayo Clin. Proc. 52: 267–280, 1977.
 145. Engel, A. G., J. M. Lindstrom, E. H. Lambert, and V. A. Lennon. Ultrastructural localization of the acetylcholine receptor in myasthenia gravis and in its experimental autoimmune model. Neurology 27: 307–315, 1977.
 146. Engel, A. G., and R. G. Siekert. Lipid storage myopathy responsive to prednisone. Arch. Neurol. Chicago 27: 174–181, 1972.
 147. Engel, W. K. Investigative approach to the muscular dystrophies. In: Treatment of Neuromuscular Disease, edited by R. C. Griggs and R. T. Moxley III. New York: Raven, 1977, p. 197–226. (Advances in Neurology Ser. 17.)
 148. Engel, W. K., and M. H. Brooke. Histochemistry of the myotonic disorders. In: Progressive Muskeldystrophie Mytonie Myasthenie, edited by E. Kuhn. Stuttgart: Springer‐Verlag, 1966, p. 203–209.
 149. Engel, W. K., N. A. Vick, C. J. Glueck, and R. I. Levy. A skeletal muscle disorder associated with intermittent symptoms and a possible defect of lipid metabolism. N. Engl. J. Med. 282: 697–704, 1970.
 150. Fambrough, D. M., D. B. Drachman, and S. Satyamurti. Neuromuscular junction in myasthenia gravis: decreased acetylcholine receptors. Science 182: 293–295, 1973.
 151. Feng, T. P. Heat‐tension ratio in prolonged tetanic contractions. Proc. R. Soc. Med. 108: 522–537, 1931.
 152. Fetell, M. R., P. E. Duffy, and L. P. Rowland. Infiltrating lipoma: a cause of monomelic hypertrophy. Muscle Nerve 1: 75–80, 1978.
 153. Fick, A. Mechanische arbeit und Wärmeentwicklelung bei der Muskelhätigkeit. Leipzig, East Germany: Brockhaus, 1882.
 154. Flacke, W. Studies on veratrum alkaloids. XXXIII. The action of some esters of germine with acetic acid on the sartorius muscle of the frog. J. Pharmacol. Exp. Ther. 137: 62–69, 1962.
 155. Floyd, W. F., P. Kent, and F. Page. An electromyographic study of myotonia. Electroencephalogr. Clin. Neurophysiol. 7: 621–630, 1955.
 156. Gage, P. W., and R. S. Eisenberg. Action potentials, after‐potentials, and excitation‐contraction coupling in frog sartorius fibers without transverse tubules. J. Gen. Physiol. 53: 298–310, 1969.
 157. Gamstorp, I. Adynamia episodica hereditaria. Acta Paediatr. Uppsala Suppl. 108: 1–120, 1956.
 158. Gamstorp, I. A study of transient muscle weakness. Acta Neurol. Scand. 38: 3–19, 1962.
 159. Gandevia, S. C., and D. I. McCloskey. Changes in motor commands, as shown by changes in perceived heaviness, during partial curarisation and peripheral anaesthesia in man. J. Physiol. London 272: 673–689, 1977.
 160. Gandevia, S. C., D. I. McCloskey, and E. K. Potter. Alterations in perceived heaviness during digital anaesthesia. J. Physiol. London 306: 365–375, 1980.
 161. Gardner‐Medwin, D. Clinical features and classification of the muscular dystrophies. Br. Med. Bull. 36: 109–115, 1980.
 162. Glatt, H. R., and C. G. Honegger. Retrograde episodal transport for cartography of neurones. Experientia 29: 1515–1517, 1973.
 163. Gohil, K., D. A. Jones, and R. H. T. Edwards. Analysis of muscle mitochondrial function with techniques applicable to needle biopsy samples. Clin. Physiol. 1: 195–207.
 164. Goldberg, A. L., G. N. De Martino, and P. Libby. The influence of thyroid hormones and proteinease inhibitors on protein degradation in skeletal muscle. In: Current Topics in Nerve and Muscle Research, edited by A. J. Aguayo and G. Karpati. Amsterdam: Excerpta Med., 1979, p. 53–60. (Int. Congr. Ser. 455.)
 165. Gollnick, P. D., R. B. Armstrong, C. W. Saubert IV, K. Piehl, and B. Saltin. Enzyme activity and fiber composition in skeletal muscle of untrained and trained men. J. Appl. Physiol. 33: 312–319, 1972.
 166. Goodgold, J., and A. Eberstein. An electromyographic study of induced myotonia in rats. Exp. Neurol. 21: 159–166, 1968.
 167. Goodgold, J., and A. Eberstein. Electrodiagnosis of Neuromuscular Diseases. Baltimore, MD: Williams & Wilkins, 1972.
 168. Gordon, A. M., J. R. Green, and D. Lagunoff. Studies on a patient with hypokalemic familial periodic paralysis. Am. J. Med. 48: 185–195, 1970.
 169. Gordon, A. M., and L. I. Kao. Disorders of muscle membranes. The periodic paralyses. In: Physiology of Membrane Disorders, edited by T. E. Andreoli, J. F. Hoffman, and D. D. Fanestil. New York: Plenum, 1978, p. 817–829.
 170. Gowers, W. R. Pseudohypertrophic muscular paralysis. Lancet 2: 113–116, 1879.
 171. Grampp, W., J. B. Harris, and S. Thesleff. Inhibition of denervation changes in skeletal muscle by blockers of protein synthesis. J. Physiol. London 221: 743–754, 1972.
 172. Grassi, E., B. Lucci, C. Marchini, S. Ottonello, M. Parma, R. Reggiani, G. L. Rossi, and J. Tagliavini. Deformed erythrocytes in muscular dystrophies. Neurology 28: 842–844, 1978.
 173. Graystone, J. E. Creatinine excretion during growth. In: Human Growth: Body Composition, Cell Growth Energy, and Intelligence, edited by D. B. Cheek. Philadelphia, PA: Lea & Febiger, 1968, p. 182–197.
 174. Griggs, R. C. Hypertrophy and cardiomypathy in the neuromuscular diseases. Circ. Res. 35, Suppl. 2: 145–151, 1974.
 175. Griggs R. C. The myotonic disorders and the periodic paralyses. In: Treatment of Neuromuscular Diseases, edited by R. C. Griggs and R. T. Moxley III. New York: Raven, 1977, p. 143–159. (Advances in Neurology Ser. 17.)
 176. Grimby, G., C. Broberg, I. Krotkiewska, and M. Krotkiewska. Muscle fibre composition in patients with traumatic cord lesion. Scand. J. Rehabil. Med. 8: 37–42, 1976.
 177. Gronert, G. A., J. H. Milde, and S. R. Taylor. Porcine muscle responses to carbachol, α‐ and β‐adrenoceptor agonists, halothane or hyperthermia. J. Physiol. London 307: 319–333, 1980.
 178. Gross, D., A. Grassino, W. R. D. Ross, and P. T. MacKlem. Electromyogram pattern of diaphragmatic fatigue. J. Appl. Physiol: Respirat. Environ. Exercise Physiol: 46: 1–7, 1979.
 179. Gross, M. Proximal spinal muscular atrophy. J. Neurol. Neurosurg. Psychiatry 29: 29–34, 1966.
 180. Gruener, R., L. Z. Stern, C. Payne, and L. Hannapel. Hyperthyroid myopathy. Intracellular electrophysiological measurements in biopsied human intercostal muscle. J. Neurol. Sci. 24: 339–349, 1975.
 181. Gutmann, E. (editor). The Denervated Muscle (translation). New York: Consultants Bureau, 1962, p. 13–56.
 182. Häggmark, T., and E. Eriksson. Cylinder or mobile cast brace after knee ligament surgery. A clinical analysis and morphologic and enzymatic studies of changes in the quadriceps muscle. Am. J. Sports Med. 7: 48–56, 1979.
 183. Häggmark, T., and E. Eriksson. Hypotrophy of the soleus muscle in man after Achilles tendon rupture. Discussion of findings obtained by computed tomography and morphologic studies. Am. J. Sports Med. 7: 121–126, 1979.
 184. Hall, G. M., J. N. Lucke, and D. Lister. Porcine malignant hyperthermia. V. Fatal hyperthermia in the Pietrain pig, associated with the infusion of α‐adrenergic agonists. Br. J. Anaesth. 49: 855–863, 1977.
 185. Hall, K., and R. Luft. Growth hormone and somatomedin. In: Advances in Metabolic Disorders, edited by R. Levine and R. Luft. New York: Academic, 1974, vol. 7, p. 1–36.
 186. Halliday, D., and R. O. McKeran. Measurement of muscle protein synthetic rate from serial muscle biopsies and total body protein turnover in man by continuous intravenous infusion of l‐[α‐15N]lysine. Clin. Sci. Mol. Med. 49: 581–590, 1975.
 187. Halsall, P. J., and F. R. Ellis. Screening test for the malignant hyperpyrexia phenotype using suxamethonium‐induced contracture of muscle treated with caffeine and its inhibition by dantrolene. Br. J. Anaesth. 51; 753–756, 1979.
 188. Harper, P. S. Myotonic Dystrophy. Philadelphia, PA: Saunders, 1979. (Major Problems in Neurology, Ser. 9.)
 189. Harriman, D. G. F., F. R. Ellis, A. J. Franks, and D. W. Sumner. Malignant hyperthermic myopathy in man: an investigation of 75 families. In: International Symposium on Malignant Hyperthermia, 2nd: Proceedings, edited by J. A. Aldrete and B. A. Britt. New York: Grune & Stratton, 1978, p. 67–87.
 190. Harris, R. C., R. H. T. Edwards, E. Hultman, and L. O. Nordesjo. The time course of phosphorylcreatine resynthesis during recovery of the quadriceps muscle in man. Pfluegers Arch. 367: 137–142, 1976.
 191. Harting, H. Über einen fall von intermittierender Paralysis Spinalis. Zentralbl. Med. Wiss. 13: 428–429, 1875.
 192. Hay, A. M., and J. C. Waterlow. The effect of alloxan diabetes on muscle and liver protein synthesis in the rat, measured by constant infusion of l‐[14C]lysine. J. Physiol. London 191: 111P–112P, 1967.
 193. Hayward, M. Automatic analysis of the electromyogram in healthy subjects of different ages. J. Neurol. Sci. 33: 397–413, 1977.
 194. Hayward, M., and R. G. Willison. Automatic analysis of the electromyogram in patients with chronic partial denervation. J. Neurol. Sci. 33: 415–423, 1977.
 195. Heckmatt, J. Z., V. Dubowitz, and S. Leeman. Detection of pathological change in dystrophic muscle with B‐scan ultrasound imaging. Lancet 1: 1389–1390, 1980.
 196. Henriksson, J., and J. S. Reitman. Time course of changes in human skeletal muscle succinate dehydrogenase and cytochrome oxidase activities and maximal oxygen uptake with physical activity and inactivity. Acta Physiol. Scand. 99: 91–97, 1977.
 197. Hill, D. K., M. J. McDonnell, and P. A. Merton. Direct stimulation of the adductor pollicis in man. J. Physiol. London 300: 2P–3P, 1980.
 198. Hjalmarson, A. C., D. E. Rannels, R. Kao, and H. E. Morgan. Effects of hypophysectomy, growth hormone, and thyroxine on protein turnover in heart. J. Biol. Chem. 250: 4556–4561, 1975.
 199. Hofmann, W. W., and R. A. Smith. Hypokalemic periodic paralysis: studies in vitro. Brain 93: 445–474, 1970.
 200. Horowicz, P., P. W. Gage, and R. S. Eisenberg. The role of the electrochemical gradient in determining potassium fluxes in frog striated muscle. J. Gen. Physiol. Suppl. 51: 193S–203S, 1968.
 201. Hosking, G. P., U. S. Bhat, V. Dubowitz, and R. H. T. Edwards. Measurements of muscle strength and performance in children with normal and diseased muscle. Arch. Dis. Child. 51: 957–963, 1976.
 202. Hosking, G. P., N. P. C. Cavanagh, D. P. L. Smyth, and J. Wilson. Oral treatment of carnitine myopathy [letter]. Lancet 1: 853, 1977.
 203. Hudecki, M. S., C. Pollina, A. K. Bhargava, J. E. Fitzpatrick, C. A. Privitera, and D. Schmidt. Effect of exercise on chickens with hereditary muscular dystrophy. Exp. Neurol. 61: 65–73, 1978.
 204. Hultman, E. Studies of muscle metabolism of glycogen and active phosphate in man with special reference to exercise and diet. Scand. J. Clin. Lab. Invest. Suppl. 94: 1–63, 1967.
 205. Hurwitz, L. J., D. McCormick, and I. V. Allen. Reduced muscle and α‐glucosidase (acid maltase) activity in hypothyroid myopathy. Lancet 1: 67–69, 1970.
 206. Ikai, M., and T. Fukunaga. Calculation of muscle strength per unit cross‐sectional area of human muscle by means of ultrasonic measurement. Int. Z. Angew. Physiol. 26: 26–32, 1968.
 207. Ikai, M., and T. Fukunaga. A study on training effect on strength per unit cross‐sectional area of muscle by means of ultrasonic measurement. Int. Z. Angew. Physiol. 28: 173–180, 1970.
 208. Ionasescu, V., H. Zellweger, and T. W. Conway. A new approach for carrier detection in Duchenne muscular dystrophy. Protein synthesis of muscle polyribosomes in vitro. Neurology 21: 703–709, 1971.
 209. Isaacs, H. Myopathy and malignant hyperthermia. In: International Symposium on Malignant Hyperthermia, 2nd: Proceedings, edited by J. A. Aldrete and B. A. Britt. New York: Grune & Stratton, 1978, p. 89–102.
 210. Ito, Y., R. Miledi, A. Vincent, and J. Newsom‐Davis. Acetylcholine receptors and end‐plate electrophysiology in myasthenia gravis. Brain 101: 345–368, 1978.
 211. Jacob, A., E. R. Clack, and A. E. H. Emery. Genetic study of sample of 70 patients with myasthenia gravis. J. Med. Genet. 5: 257–261, 1968.
 212. Jennekens, F. G. I., H. F. M. Busch, N. M. van Hemel, and R. A. Hodgland. Inflammatory myopathy in scapulo‐ilio‐peroneal atrophy with cardiopathy. A study of two families. Brain 98: 709–722, 1975.
 213. Johnson, R. L., C. W. Fink, and M. Ziff. Lymphotoxin formation by lymphocytes and muscle in polymyositis. J. Clin. Invest. 51: 2435–2449, 1972.
 214. Jones, D. A., B. Bigland‐Ritchie, and R. H. T. Edwards. Excitation frequency and muscle fatigue: mechanical responses during voluntary and stimulated contractions. Exp. Neurol. 64: 401–413, 1979.
 215. Jones, N. L., E. J. M. Campbell, R. H. T. Edwards, and D. G. Robertson. Clinical Exercise Testing: Guide to the Use of Exercise Physiology in Clinical Investigation. Philadelphia, PA: Saunders, 1975.
 216. Jones, P. R. M., and J. Pearson. Anthropometric determination of leg fat and muscle plus bone volumes in young male and female adults. J. Physiol. London 204: 63P–66P, 1969.
 217. Jones, R., and G. Vrbová. Can denervation hypersensitivity be prevented? J. Physiol. London 217: 67P–68P, 1971.
 218. Julien, J., C. Vital, J. M. Vallat, M. Vallat, and M. Le Blanc. Oculopharyngeal muscular dystrophy. A case with abnormal mitochondria and “fingerprint” inclusions. J. Neurol. Sci. 21: 165–169, 1974.
 219. Kadefors, R., E. Kaiser, and I. Petersen. Dynamic spectrum analysis of myo‐potentials and with special reference to muscle fatigue. Electromyography 8: 39–74, 1968.
 220. Kagen, L. J. Myoglobinemia in inflammatory myopathies. J. Am. Med. Assoc. 237: 1448–1452, 1977.
 221. Kagen, L. J., S. Moussavi, S. L. Miller, and P. Tsairis. Serum myoglobin in muscular dystrophy. Muscle Nerve 3: 221–226, 1980.
 222. Kaiser, E., and I. Petersen. Muscle action potentials studied by frequency analysis and duration of measurement. Acta Neurol. Scand. 41: 213–235, 1965.
 223. Kakulas, B. A. Destruction of differentiated muscle cultures by sensitised lymphoid cells. J. Pathol. Bacteriol. 91: 495–503, 1966.
 224. Kakulas, B. A. In vitro destruction of skeletal muscle by sensitised cells. Nature London 210: 1115–1118, 1966.
 225. Kao, I., and A. M. Gordon. Mechanism of insulin‐induced paralysis of muscles from potassium‐depleted rats. Science 188: 740–741, 1975.
 226. Kao, I., and A. M. Gordon. Alteration of skeletal muscle cellular structures by potassium depletion. Neurology 27: 855–860, 1977.
 227. Kar, N. C., and C. M. Pearson. A calcium‐activated neutral protease in normal and dystrophic human muscle. Clin. Chim. Acta 73: 293–297, 1976.
 228. Kar, N. C., and C. M. Pearson. Muscular dystrophy and activation of proteases. Muscle Nerve 1: 308–313, 1978.
 229. Kar, N. C., and C. M. Pearson. Proteolytic mechanisms in muscular dystrophies and other neuromuscular diseases. In: Degradative Processes in Heart and Skeletal Muscle, edited by K. Wildenthal. Amsterdam: Elsevier, 1980, p. 257–269.
 230. Karpati, G., S. Carpenter, A. G. Engel, G. Watters, J. Allen, S. Rothman, G. Klassen, and O. A. Mamer. The syndrome of systemic carnitine deficiency. Clinical, morphologic, biochemical, and pathophysiologic features. Neurology 25: 16–24, 1975.
 231. Kennelly, A. E. An approximate law of fatigue in the speeds of racing animals. Proc. Am. Acad. Arts Sci. 42: 275–331, 1906.
 232. Kreitzer, S. M., M. Ehrenpreis, E. Miguel, and J. Petrasek. Acute myoglobinuric renal failure in polymyositis. NY State J. Med. 2: 295–297, 1978.
 233. Kristensson, K., and Y. Olsson. Retrograde axonal transport of protein. Brain Res. 29: 363–365, 1971.
 234. Lanavi, A. La contractión miotónica en el hombre despríes de curarizaeión. Medicina Buenos Aires 7: 21–26, 1947.
 235. Land, J. M., and J. B. Clark. Mitochondrial myopathies. Biochem. Soc. Trans. 7: 231–245, 1979.
 236. Landouzy, L., and J. Déjèrine. De la myopathie atrophique progressive (myopathie héréditaire), débutant, dans l'enfance par la face, sans altération du système nerveux. C. R. Acad. Sci. 98: 53–55, 1884.
 237. Lane, J. M., and F. L. Mastaglia. Drug‐induced myopathies in man. Lancet 2: 562–565, 1978.
 238. Layzer, R. B. Glycolysis and glycogen. In: Pathogenesis of Human Muscular Dystrophies, edited by L. P. Rowland. Amsterdam: Excerpta Med., 1977, p. 395–403. (Int. Congr. Ser. 404.)
 239. Layzer, R. B., R. J. Havel, and M. B. McIlroy. Partial deficiency of carnitine palmityltransferase: physiologic and biochemical consequences. Neurology 30: 627–633, 1980.
 240. Layzer, R. B., R. E. Lovelace, and L. P. Rowland. Hyperkalemic periodic paralysis. Arch. Neurol. Chicago 16: 455–472, 1967.
 241. Layzer, R. B., and L. P. Rowland. Cramps. N. Engl. J. Med. 285: 31–40, 1971.
 242. Lewis, P. O., G. W. Pickering, and P. Rothschild. Observations on muscular pain in intermittent claudication. Heart 15: 359–383, 1931.
 243. Leyburn, P., and J. N. Walton. The treatment of myotonia: a controlled trial. Brain 82: 81–91, 1959.
 244. Libby, P., and A. L. Goldberg. Leupeptin, a protease inhibitor, decreases protein degradation in normal and diseased muscles. Science 199: 534–536, 1978.
 245. Libby, P., and A. L. Goldberg. The control and mechanism of breakdown in striated muscle: studies with selective inhibitors. In: Degradative Processes in Heart and Skeletal Muscle, edited by K. Wildenthal. Amsterdam: Elsevier, 1980, p. 201–222.
 246. Liljestrand, A. Fall av adynamia episodica hereditaria. Opusc. Med. 7: 183–193, 1957.
 247. Lindstrom, J. M., M. E. Seybold, V. A. Lennon, S. Whittingham, and D. D. Duane. Antibody to acetylcholine receptor in myasthenia gravis. Prevalence, clinical correlates, and diagnostic value. Neurology 26: 1054–1059, 1976.
 248. Lipicky. R. J. Studies in human myotonic dystrophy. In: Pathogenesis of Human Muscular Dystrophies, edited by L. P. Rowland. Amsterdam: Excerpta Med., 1977, p. 729–738. (Int. Congr. Ser. 404.)
 249. Lipicky, R. J., and S. H. Bryant. Sodium, potassium, and chloride fluxes in intercostal muscle from normal goats and goats with hereditary myotonia. J. Gen. Physiol. 50: 89–111, 1966.
 250. Lipicky, R. J., S. H. Bryant, and J. H. Salmon. Cable parameters, sodium, potassium, chloride, and water content, and potassium efflux in isolated external intercostal muscle of normal volunteers and patients with myotonia congenita. J. Clin. Invest. 50: 2091–2103, 1971.
 251. Lister, D., G. M. Hall, and J. N. Lucke. Porcine malignant hyperthermia. III. Adrenergic blockade. Br. J. Anaesth. 48: 831–838, 1976.
 252. Lister, D., R. A. Sair, J. A. Will, G. R. Schmid t. R. G. Cassens, W. G. Hoekstra, and E. J. Briskey. Metabolism of striated muscle of stress‐susceptible pigs breathing oxygen or nitrogen. Am. J. Physiol. 218: 102–107, 1970.
 253. Liversedge, L. A., and M. J. Campbell. The central neuronal muscular atrophies and other dysfunctions of the anterior horn cells. In: Disorders of Voluntary Muscle (3rd ed.), edited by J. N. Walton. Edinburgh: Churchill Livingstone, 1974, p. 775–803.
 254. Lomo, T., and J. Rosenthal. Control of ACh sensitivity by muscle activity in the rat. J. Physiol. London 221: 493–513, 1972.
 255. Lucy, J. A. Is there a membrane deficit in muscle and other cells? Br. Med. Bull. 36: 187–192, 1980.
 256. Luft, R., D. Ekkos, G. Palmrieri, L. Ernster, and B. Afzelius. A case of severe hypermetabolism of nonthyroid origin with a defect in maintenance of mitochondrial respiratory control. A correlated clinical biochemical and morphological study. J. Clin. Invest. 41: 1776–1804, 1962.
 257. MacDonald, R. D., N. B. Rewcastle, and J. G. Humphrey. The myopathy of hyperkalemic periodic paralysis. An electron microscopic study. Arch. Neurol. Chicago 19: 274–283, 1968.
 258. MacDonald, R. D., N. B. Rewcastle, and J. G. Humphrey. Myopathy of hypokalemic periodic paralysis. An electron microscopic study. Arch. Neurol. Chicago 20: 565–585, 1969.
 259. Mahler, R. F. Disorders of glycogen metabolism. In: Clinics in Endocrinology and Metabolism, edited by K. G. M. M. Alberti. London: Saunders, 1976, vol. 5, p. 579–598.
 260. Manchester, K. L. The hormonal control of protein metabolism. In: Protein Metabolism and Nutrition, edited by D. J. A. Cole, K. M. Borrman, P. J. Buttery, D. Lewis, R. J. Neale, and H. Swan. London: Butterworths, 1976, p. 35–47.
 261. Maruyama, K., M. L. Sunde, and R. W. Swick. Growth and muscle protein turnover in the chick. Biochem. J. 176: 573–582, 1978.
 262. Mayer, M., E. Shafrir, N. Kaiser, R. J. Milholland, and F. Rosen. Interaction of glucocorticoid hormones with rat skeletal muscle: catabolic effects and hormone binding. Metabolism 25: 157–167, 1976.
 263. McArdle, B. Myopathy due to a defect in muscle glycogen breakdown. Clin. Sci. 10: 13–25, 1951.
 264. McArdle, B. Adynamic episodica hereditaria and its treatment. Brain 85: 121–148, 1962.
 265. McComas, A. J. Neuromuscular Function and Disorders. London: Butterworths, 1977.
 266. McComas, A. J., K. Mrozek, and W. G. Bradley. The nature of the electrophysiological disorder in adynamia episodica. J. Neurol. Neurosurg. Psychiatry 31: 448–452, 1968.
 267. McComas, A. J., R. E. P. Sica, and M. E. Brandstater. Further motor unit studies in Duchenne muscular dystrophy. J. Neurol. Neurosurg. Psychiatry 40: 1147–1151, 1977.
 268. McComas, A. J., R. E. P. Sica, and S. Currie. Muscular dystrophy: evidence for a neural factor. Nature London 226: 1263–1264, 1970.
 269. McKeran, R. O., D. Halliday, and P. Purkiss. Increased myofibrillar protein catabolism in Duchenne muscular dystrophy measured by 3‐methylhistidine excretion in the urine. J. Neurol. Neurosurg. Psychiatry 40: 979–981, 1977.
 270. McKeran, R. O., G. Slavin, T. M. Andrews, P. Ward, and N. G. P. Mair. Muscle fibre type changes in hypothyroid myopathy. J. Clin. Pathol. 28: 659–663, 1975.
 271. Mense, S. S. Muscle nociceptors. J. Physiol. Paris 73: 233–240, 1977.
 272. Merton, P. A. Voluntary strength and fatigue. J. Physiol. London 123: 553–564, 1954.
 273. Meryon, E. On granular and fatty degeneration of the voluntary muscle. Med. Chir. Trans. Edinburgh 35: 73–84, 1852.
 274. Meryon, E. Practical and Pathological Researches on the Various Forms of Paralysis. London: Churchill, 1864, p. 200–215.
 275. Miledi, R. Properties of regenerating neuromuscular synapse in the frog. J. Physiol. London 154: 190–205, 1960.
 276. Miller, S. E., A. D. Roses, and S. H. Appel. Scanning electron microscopy studies in muscular dystrophy. Arch. Neurol. Chicago 33: 172–174, 1976.
 277. Millward, D. J. Protein turnover in skeletal and cardiac muscle during normal growth and hypertrophy. In: Degradative Processes in Heart and Skeletal Muscle, edited by K. Wildenthal. Amsterdam: Elsevier, 1980, p. 161–199.
 278. Millward, D. J., P. C. Bates, J. G. Brown, S. R. Rosochacki, and M. J. Rennie. Protein degradation and the regulation of protein balance in nature. In: Protein Degradation in Health and Disease. Amsterdam: Excerpta Med., 1980, p. 307–309. (Ciba Found. Symp. 75.)
 279. Millward, D. J., P. C. Bates, G. K. Grimble, J. G. Brown, M. Nathan, and M. J. Rennie. Quantitative importance of non‐skeletal muscle sources of N2‐methylhistidine in urine. Biochem. J. 90: 225–228, 1980.
 280. Mokri, B., and A. G. Engel. Duchenne dystrophy: electron microscopic findings pointing to a basic or early abnormality in the plasma membrane of the muscle fiber. Neurology 25: 1111–1120, 1975.
 281. Mommaerts, W. F. H. M., B. Illingworth, C. M. Pearson, R. J. Guillorg, and K. Seraydarian. A functional disorder of muscle associated with the absence of Phosphorylase. Proc. Natl. Acad. Sci. USA 45: 791–797, 1959.
 282. Monckton, G., and H. Marusyk. The incorporation of 3H[G]‐l‐leucine into single muscle fibres in Duchenne dystrophy and Charcot‐Marie‐Tooth disease. Can. J. Neurol. Sci. 6: 53–57, 1979.
 283. Morgan‐Hughes, J. A. Painful disorders of muscle. Br. J. Hosp. Med. 22: 362–365, 1979.
 284. Morgan‐Hughes, J. A., P. Darveniza, S. N. Kahn, D. N. Landon, R. M. Sherratt, J. M. Land, and J. B. Clark. I mitochondrial myopathy characterized by a deficiency in reducible cytochrome b. Brain 100: 617–640, 1977.
 285. Morgan‐Hughes, J. A., and W. G. P. Mair. Atypical muscle mitochondria in oculoskeletal myopathy. Brain 96: 215–224, 1973.
 286. Moser, H. von, U. Weismann, R. Richterich, and E. Rossi. Progressive muskeldystropie Haufigkeit, Klinik und Genetik der Duchenne‐Form. Schweiz. Med. Wochenschr. 94: 1610–1621, 1966.
 287. Mosso, A. Fatigue, translated by M. Drummond and W. G. Drummond. London: Allen & Unwin, 1915, p. 78–80.
 288. Moulds, R. F. W., and M. A. Denborough. A study of the action of caffeine, halothane, potassium chloride and procaine on normal human skeletal muscle. Clin. Exp. Pharmacol. Physiol. 1: 197–209, 1974.
 289. Moulds, R. F. W., and M. A. Denborough. Identification of susceptibility to malignant hyperpyrexia. Br. Med. J. 2: 245–247, 1974.
 290. Moulds, R. F. W., A. Young, D. A. Jones, and R. H. T. Edwards. A study of the contractility, biochemistry and morphology of an isolated preparation of human skeletal muscle. Clin. Sci. Mol. Med. 52: 291–297, 1977.
 291. Moxham, J., A. J. R. Morris, S. Spiro, R. H. T. Edwards, and M. Green. The contractile properties and fatigue of the diaphragm in man. Clin. Sci. 58: 6P, 1979.
 292. Moxham, J., C. M. Wiles, D. Newham, and R. H. T. Edwards. Sternomastoid muscle function and fatigue in man. Clin. Sci. 59: 463–468, 1980.
 293. Moxley, R. T., III. Metabolic studies in muscular dystrophy, a role for insulin. In: Treatment of Neuromuscular Diseases, edited by R. C. Griggs and R. T. Moxley III. New York: Raven, 1977, p. 161–173. (Advances in Neurology Ser. 17.)
 294. Munck, A. Glucocorticoid inhibition of glucose uptake by peripheral tissues: old and new evidence, molecular mechanisms, and physiological significance. Perspect. Biol. Med. 14: 265–269, 1971.
 295. Munsat, T. L. Pharmacologic therapy of dystrophy in man and animals. Ann. NY Acad. Sci. 317: 400–408, 1979.
 296. Naess, K., and A. Storm‐Mathison. Fatigue of sustained tetanic contractions. Acta Physiol. Scand. 34: 351–366, 1956.
 297. Needham, D. M. Machina Carnis: the Biochemistry of Muscular Contraction in Its Historical Development. Cambridge, UK: Cambridge Univ. Press, 1972.
 298. Nelson, T. E. Excitation contraction coupling, a common etiological pathway for malignant hyperthermia susceptible muscle. In: International Symposium on Malignant Hyperthermia, 2nd: Proceedings, edited by J. A. Aldrete and B. A. Britt. New York: Grune & Stratton, 1978, p. 23–36.
 299. Nelson, T. E., E. W. Jones, J. H. Venable, and D. D. Kerr. Malignant hyperthermia of Poland China swine: studies of a myogenic etiology. Anesthesiology 36: 52–56, 1972.
 300. Newsom‐Davis, J., A. J. Pinching, A. Vincent, and S. G. Wilson. Function of circulating antibody to acetylcholine receptor in myasthenia gravis: investigation by plasma exchange. Neurology 28: 266–272, 1978.
 301. O'doherty, D. S., D. Schellinger, and V. Raptopoulos. Computed tomographic patterns of pseudohypertrophic muscular dystrophy: preliminary results. J. Comput. Assist. Tomography 1: 482–486, 1977.
 302. Offerijus, F. G. J., D. Westerink, and A. F. Willebrands. The relationship of potassium deficiency to muscular paralysis of insulin. J. Physiol. London 141: 377–384, 1958.
 303. Otsuka, M., and I. Ohtsuki. Mechanism of muscular paralysis by insulin with special reference to periodic paralysis. Am. J. Physiol. 219: 1178–1182, 1970.
 304. Pathen, B. M., J. M. Shabot, J. Alperin, and R. F. Dodson. Hepatitis associated with lipid storage myopathy. Ann. Intern. Med. 87: 417–421, 1977.
 305. Patrick, J., and J. Lindstrom. Autoimmune response to acetylcholine receptor. Science 180: 871–872, 1973.
 306. Patterson, S., and L. Klenerman. The effect of pneumatic tourniquets on the ultrastructure of skeletal muscle. J. Bone Jt. Surg. 61B: 178–183, 1979.
 307. Pearson, C. M. Development of arthritis, periarthritis and periostitis in rats given adjuvants. Proc. Soc. Exp. Biol. Med. 91: 95–101, 1956.
 308. Pearson, C. M. Mechanisms involved in polymyositis and dermatomyositis. In: Infection and Immunology in the Rheumatic Diseases, edited by D. C. Dumonde. Oxford, UK: Black‐well Scientific, 1976, p. 489–493.
 309. Pearson, C. M., and K. Kalyanaraman. The periodic paralyses. In: The Metabolic Basis of Inherited Disease (3rd ed.), edited by J. B. Stanbury, J. B. Wyngaarden, and D. S. Fredrickson. New York: McGraw‐Hill, 1972, p. 1181–1203.
 310. Pennington, R. J. T. Clinical biochemistry of muscular dystrophy. Br. Med. Bull. 36: 123–126, 1980.
 311. Perkoff, G. T., P. Hardy, and E. Velez‐Garcia. Reversible acute muscular syndrome in chronic alcoholism. N. Engl. J. Med. 274: 1277–1285, 1966.
 312. Pernow, B. B., R. J. Havel, and D. B. Jennings. The second wind phenomenon in McArdle's syndrome. Acta Med. Scand. Suppl. 472: 294–307, 1967.
 313. Peter, J. B., and D. S. Campion. Animal models of myotonia. In: Pathogenesis of Human Muscular Dystrophies, edited by L. P. Rowland. Amsterdam: Bxcerpta Med., 1977, p. 739–746. (Int. Congr. Ser. 404.)
 314. Pfau, A. Interpretation of whole body potassium measurement. In: Human Body Composition, edited by J. Brozek. Oxford, UK: Pergamon, 1965, p. 57–60.
 315. Plishker, G. A., and S. H. Appel. An overview of erythrocyte abnormalities in muscle diseases. In: Current Topics in Nerve and Muscle Research, edited by A. J. Aguayo and G. Karpati. Amsterdam: Excerpta Med., 1979, p. 39–50. (Int. Congr. Ser. 455.)
 316. Porte, D., Jr., D. W. Crawford, D. B. Jennings, C. Aber, and B. McIlroy. Cardiovascular and metabolic responses to exercise in a patient with McArdle's syndrome. N. Engl. J. Med. 275: 406–412, 1966.
 317. Ramirez, B. U., and D. Pette. Effects of long‐term electrical stimulation on sarcoplasmic reticulum of fast rabbit muscle. FEBS Lett. 49: 188–190, 1974.
 318. Reddy, M. K., J. D. Etlinger, M. Rabinowitz, D. A. Fischman, and R. Zak. Removal of Z‐lines and alpha‐actin from isolated myofibrils by a calcium‐activated neutral protease. J. Biol. Chem. 250: 4278–4284, 1975.
 319. Reeds, P. J., A. A. Jackson, D. Picou, and N. Poulter. Muscle mass and composition in malnourished infants and children and changes seen after recovery. Pediatr. Res. 12: 613–618, 1978.
 320. Reid, C. The mechanism of voluntary muscle fatigue. Q. J. Exp. Physiol. 19: 17–28, 1928.
 321. Rennie, M. J., R. H. T. Edwards, and D. J. Millward. Increased protein degradation in muscle disease: cause or effect. Muscle Nerve 5: 85–86, 1982.
 322. Rennie, M. J., R. H. T. Edwards, D. J. Millward, S. C. Wolman, D. Holliday, and D. E. Matthews. Effects of Duchenne muscular dystrophy on muscle protein synthesis. Nature London 296: 165–167, 1982.
 323. Rennie, M. J., S. Rosochacki, M. Nathan, P. C. Bates, R. H. T. Edwards, and D. J. Millward. Intracellular, plasma and urine 3‐methylhistidine as an index of muscle wasting and repair. In: Amino Acid Analysis in Clinical Chemistry and Medical Research, edited by J. M. Rattenbury. Chichester, UK: Horwood, 1980, p. 210–224.
 324. Richer, K., A. Haas, G. Hertel, and H. G. Mertens. Transient muscular weakness in severe recessive myotonia congenita. Improvement of isometric muscle force by drugs relieving myotonic stiffness. J. Neurol. 218: 253–262, 1978.
 325. Riecker, G., and H. D. Bolte. Membran potentiale einzlaner Skeletmuskelzellen bei hypokaliamischer periodischer Muskel paralyse. Klin. Wochenschr. 44: 804–807, 1966.
 326. Riggs, J. E., R. C. Griggs, and R. T. Moxley III. Acetazolamide‐induced weakness in paramyotonia congenita. Ann. Intern. Med. 86: 169–173, 1977.
 327. Roelofs, R. I., W. K. Engel, and P. B. Chauvin. Histochemical Phosphorylase activity in regenerating muscle fibers from myophosphorylase‐deficient patients. Science 111: 795–797, 1972.
 328. Rose, A. L., and J. N. Walton. Polymyositis: a survey of 89 cases with particular reference to treatment and prognosis. Brain 89: 747–768, 1966.
 329. Rotthauwe, H. W., W. Mortier, and H. Beyer. Neuer Typ einer recessiv X‐chromosomal vererbten Muskeldystrophie: Scapulo‐humero‐distale Muskeldystrophie mit frühzeitigen Kontrakturen und Herzrhythmusstörungen. Humangenetik 16: 181–200, 1972.
 330. Rowland, L. P. Biochemistry of muscle membranes in Duchenne muscular dystrophy. Muscle Nerve 3: 3–20, 1980.
 331. Rowland, L. P., S. Araki, and P. Carmel. Contracture in McArdle's disease. Stability of adenosine triphosphate during contracture in phosphorylase‐deficient human muscle. Arch. Neurol. Chicago 13: 541–544, 1965.
 332. Rowland, L. P., C. Clark, and M. Olarte. Therapy for dermatomyositis and polymyositis. In: Treatment of Neuromuscular Diseases, edited by R. C. Griggs and R. T. Moxley III. New York: Raven, 1977, p. 63–97. (Advances in Neurology Ser. 17.)
 333. Rüdel, R. The mechanism of pharmacologically induced myotonia. In: Membranes and Disease, edited by L. Bolis, J. F. Hoffman, and A. Leaf. New York: Raven, 1976, p. 207–213.
 334. Rüdel, R., and J. Senges. Mammalian skeletal muscle: reduced chloride conductance in drug‐induced myotonia and induction of myotonia by low‐chloride solution. Naunyn‐Schmiedeberg's Arch. Pharmacol. 274: 337–347, 1972.
 335. Rüdman, D., C. W. Sewell, and J. D. Ansley. Deficiency of carnitine in cachectic serotic patients. J. Clin. Invest. 60: 716–723, 1967.
 336. Sahgal, V., V. Subramani, R. Hughes, A. Shah, and H. Singh. On the pathogenesis of mitochondrial myopathies. An experimental study. Acta Neuropathol. 46: 177–183, 1979.
 337. Salmons, S., and G. Vrbová. The influence of activity on some contractile characteristics of mammalian fast and slow muscles. J. Physiol. London 201: 535–549, 1969.
 338. Saltin, B., K. Nazar, D. L. Costill, E. Stein, E. Jansson, B. Essén, and P. D. Gollnick. The nature of the training response; peripheral and central adaptations of one‐legged exercise. Acta Physiol. Scand. 96: 289–305, 1976.
 339. Sargeant, A. J., C. T. M. Davies, R. H. T. Edwards, C. Maunder, and A. Young. Functional and structural changes after disease of human muscle. Clin. Sci. Mol. Med. 52: 337–342, 1977.
 340. Satyamurti, S., D. B. Drachman, and F. Slone. Blockade of acetylcholine receptors: a model of myasthenia gravis. Science 187: 955–957, 1975.
 341. Schmid, R., and R. E. Mahler. Chronic progressive myopathy with myoglobinuria, demonstration of a glycogenolytic defect in the muscle. J. Clin. Invest. 38: 2044–2058, 1959.
 342. Schotland, D. L., E. Bonilla, and M. Van Meter. Duchenne dystrophy: alteration in muscle plasma membrane structure. Science 196: 1005–1007, 1977.
 343. Schotland, D. L., E. Bonilla, and Y. Wakayama. Pathogenesis of muscle cell damage in the dystrophies, morphologic aspects including freeze fracture studies. In: Current Topics in Nerve and Muscle Research, edited by A. J. Aguayo and G. Karpati. Amsterdam: Excerpta Med., 1979, p. 29–38. (Int. Congr. Ser. 455.)
 344. Schotland, D. L., E. Bonilla, and Y. Wakayama. Application of the freeze fracture technique to the study of human neuromuscular disease. Muscle Nerve 3: 21–27, 1980.
 345. Schotland, D. L., S. Di Mauro, E. Bonilla, A. Scarpa, and C. P. Lee. Neuromuscular disorder associated with a defect in mitochondrial energy supply. Arch. Neurol. Chicago 33: 475–479, 1976.
 346. Schwarz, H. A., G. Slavin, and B. Ansell. Histological and morphometric studies in polymyositis and dermatomyositis. Ann. Rheum. Dis. 39: 186, 1980.
 347. Serratrice, G., H. Rouz, R. Aquaron, and A. M. Recordier. Serum and muscle activities (glycolytic and transferase) in human cortisone myopathies. In: Muscle Diseases, edited by J. N. Walton, N. Canal, and G. Scarlato. Amsterdam: Excerpta Med., 1970, p. 489–496.
 348. Shy, G. M., T. Wanko, P. T. Rowley, and A. G. Engel. Studies in familial periodic paralysis. Exp. Neurol. 3: 53–121, 1961.
 349. Siegel, I. M. The management of muscular dystrophy: a clinical review. Muscle Nerve 1: 453–460, 1978.
 350. Simpson, J. A. Myasthenia gravis: a personal view of pathogenesis and mechanism, part 1. Muscle Nerve 1: 45–56, 1978.
 351. Simpson, J. A. Myasthenia gravis: a personal view of pathogenesis and mechanism, part 2. Muscle Nerve 1: 151–156, 1978.
 352. Sîrca, A., and M. Susec‐Michieli. Selective type II fibre muscular atrophy in patients with osteoarthritis of the hip. J. Neurol. Sci. 44: 149–159, 1980.
 353. Sloper, J. C., T. A. Partridge, P. D. Smith, and D. Manghani. The pathogenesis of experimental allergic myositis. In: Infection and Immunology in the Rheumatic Diseases, edited by D. C. Dumonde. Oxford, UK: Blackwell Scientific, 1979, p. 495–501.
 354. Smith, J. B., and A. Patel. The Wohlfart‐Kugelberg‐Welander disease; review of the literature and report of a case. Neurology 15: 469–473, 1965.
 355. Sréter, F. A., J. Gergely, S. Salmons, and F. Romanul. Synthesis by fast muscle of myosin light chains characteristic of slow muscle in response to long‐term stimulation. Nature London New Biol. 241: 17–19, 1973.
 356. Stålberg, E., J. Ekstedt, and A. Broman. Neuromuscular transmission in myasthenia gravis studied with single‐fibre electromyography. J. Neurol. Neurosurg. Psychiatry 37: 540–547, 1974.
 357. Stålberg, E., J. V. Trontelj, and M. Janko. Single fibre EMG findings in muscular dystrophy (Abstract). In: Symposium on Structure and Function of Normal and Diseased Muscle and Peripheral Nerve. Kazimierz, Poland: 1972.
 358. Stephens, J. A., and A. Taylor. Fatigue of maintained voluntary muscle contraction in man. J. Physiol. London 220: 1–18, 1972.
 359. Stracher, A., E. B. McGowan, and S. A. Shafio. Muscular dystrophy: inhibition of degeneration in vivo with protease inhibitors. Science 200: 50–51, 1978.
 360. Swash, M., M. S. Schwartz, and M. K. Sargeant. Pathogenesis of longitudinal splitting of muscle fibers in neurogenic disorders and in polymyositis. Neuropathol. Appl. Neurobiol. 4: 99–115, 1978.
 361. Tarui, S., G. Okuno, Y. Ikura, Y. Tanaka, T. Tanaka, M. Suda, and M. Nishikawa. Phosphofructokinase deficiency in skeletal muscle. A new type of glycogenosis. Biochem. Biophys. Res. Commun. 19: 517–523, 1965.
 362. Tevaarwerk, G. J. M., K. P. Strickland, C. H. Lin, and A. J. Hudson. Studies on insulin resistance and insulin receptor binding in myotonia dystrophica. J. Clin. Endocrinol. Metab. 49: 216–222, 1979.
 363. Thesleff, S. Supersensitivity of skeletal muscle produced by Botulinum toxin. J. Physiol. London 151: 598–607, 1960.
 364. Thomas, P. K., D. B. Calne, and C. F. Elliott. X‐linked scapuloperoneal syndrome. J. Neurol. Neurosurg. Psychiatry 35: 208–215, 1972.
 365. Thorstensson, A., G. Grimby, and J. Karlsson. Force‐velocity relation and fiber composition in human knee extensor muscles. J. Appl. Physiol. 40: 12–16, 1976.
 366. Tornvall, G. Assessment of physical capabilities with special reference to the evaluation of maximum voluntary isometric strength and maximal working capacity. Acta Physiol. Scand. Suppl. 201: 1–102, 1963.
 367. Tower, S. Trophic control of non‐nervous tissues by the nervous system: a study of muscle and bone innervated from an isolated and quiescent region of spinal cord. J. Comp. Neurol. 67: 241–267, 1937.
 368. Toyka, K. V., D. B. Drachman, D. E. Griffin, A. Pestronk, J. A. Winkelstein, K. H. Fischbeck, and I. Kao. Myasthenia gravis. Study of humoral immune mechanisms by passive transfer to mice. N. Engl. J. Med. 296: 125–131, 1977.
 369. Toyka, K. V., D. B. Drachman, A. Pestronk, and I. Kao. Myasthenia gravis: passive transfer from man to mouse. Science 190: 397–399, 1975.
 370. Trenkle, A. Hormonal and nutritional interrelationships and their effects on skeletal muscle. J. Anim. Sci. 38: 1142–1152, 1974.
 371. Tsukagoshi, H., T. Nakanishi, K. Kondo, and T. Tsubaki. Hereditary proximal neurogenic muscular atrophy in adult. Arch. Neurol. Chicago 12: 597–603, 1965.
 372. Ulbricht, W. The effect of veratridine on excitable membranes of nerve and muscle. Ergeb. Physiol. Biol. Chem. Exp. Pharmakol. 61: 18–71, 1969.
 373. Uthne, K., C. R. Reagan, L. P. Gimpel, and J. L. Kostyo. Effects of human somatomedin preparations on membrane transport and protein synthesis in the isolated rat diaphragm. J. Clin. Endocrinol., Metab. 39: 548–554, 1974.
 374. Victor, M., R. Hayes, and R. D. Adams. Oculopharyngeal muscular dystrophy. N. Engl. J. Med. 267: 1267–1272, 1962.
 375. Viets, H. R. A historical review of myasthenia gravis from 1672–1900. J. Am. Med. Assoc. 153: 1273–1280, 1953.
 376. Vrbová, G. The influence of the motor nerve on the characteristic properties of skeletal muscle. In: The Biochemistry of Myasthenia Gravis and Muscular Dystrophy, edited by G. G. Lunt and R. M. Marchbanks. New York: Academic, 1978, p. 3–21.
 377. Walker, M. B. Treatment of myasthenia gravis with physostigmine. Lancet 1: 1200–1201, 1934.
 378. Walker, M. B. A case showing the effect of prostigmin on myasthenia gravis. Proc. R. Soc. Med. 28: 759–761, 1935.
 379. Waller, A. D. The sense of effort: an objective study. Brain 14: 179–249, 1891.
 380. Walton, J. N., and F. J. Natrass. On the classification, natural history and treatment of the myopathies. Brain 11: 169–231, 1954.
 381. Wang, P., T. Clausen, and H. Orskov. Salbutamol inhalations suppress attacks of hyperkalemia in familial periodic paralysis. Monogr. Hum. Genet. 10: 62–65, 1978.
 382. Warthin, A. S. The myocardial lesions of diphtheria. J. Infect. Dis. 35: 32–66, 1924.
 383. Waterlow, J. C., P. J. Garlick, and D. J. Millward. Protein Turnover in Mammalian Tissues and in the Whole Body. Amsterdam: Elsevier, 1978.
 384. Watson, W. E. Centripetal passage of labelled molecules along mammalian motor axons. J. Physiol. London 196: 122P–123P, 1968.
 385. Weeds, A. G., D. R. Trentham, C. J. C. Kean, and A. J. Buller. Myosin from cross‐reinnervated cat muscles. Nature London 247: 135–139, 1974.
 386. Westgaard, R. H. Influence of activity on the passive electrical properties of denervated soleus muscle fibres in the rat. J. Physiol. London 251: 683–697, 1975.
 387. Wiles, C. M. The Determinants of the Relaxation Rate of Human Muscle in Vivo. London: Univ. of London, 1980. Dissertation.
 388. Wiles, C. M., and R. H. T. Edwards. Weakness in myotonic syndromes. Lancet 2: 598–601, 1977.
 389. Wiles, C. M., D. A. Jones, and R. H. T. Edwards. Fatigue in human metabolic myopathy. In: Human Muscle Fatigue: Physiological Mechanisms, edited by R. Ponter and J. Whelan. London: Putman Med., 1981, p. 264–282. (Ciba Found. Symp.)
 390. Wiles, C. M., A. Young, D. A. Jones, and R. H. T. Edwards. Muscle relaxation rate, fibre‐type composition and energy turnover in hyper‐ and hypothyroid patients. Clin. Sci. 57: 375–384, 1979.
 391. Willems, J. C., L. A. H. Manneis, J. M. F. Trubels, J. H. Verrkaps, A. E. R. H. Meyer, D. Van Dam, and V. Van Haeslit. Leigh's encephalomyopathy in a patient with cytochrome c oxidase deficiency in muscle tissue. Pediatrics 60: 850–857, 1977.
 392. Williams, C. H., M. D. Shanklin, H. B. Hedrick, M. Muhrer, D. Stubbs, G. F. Krause, C. G. Payne, J. D. Benedict, D. P. Hutcheson, and J. F. Lasley. The fulminant hyperthermia‐stress syndrome: genetic aspects, hemodynamic and metabolic measurements in susceptible and normal pigs. In: International Symposium on Malignant Hyperthermia, 2nd: Proceedings, edited by J. A. Aldrete and B. A. Britt. New York: Grune & Stratton, 1978, p. 113–140.
 393. Willner, J. H., S. Ginsburg, and S. Dimauro. Active transport of carnitine into skeletal muscle. Neurology 28: 721–724, 1978.
 394. Willner, J. H., D. S. Wood, C. Cerri, and B. Britt. Increased myophosphorylase in malignant hyperthermia. N. Engl. J. Med. 303: 138–140, 1980.
 395. Winer, N., D. M. Klachko, R. D. Baer, P. L. Langley, and T. W. Barnes. Myotonic response induced by inhibitors of cholesterol biosynthesis. Science 153: 312–313, 1966.
 396. Wingard, D. W. Malignant hyperthermia: a human stress syndrome (Letter)? Lancet 2: 1450–1451, 1974.
 397. Wood, D. S. Human skeletal muscle: analysis of Ca2+ regulation in skinned fibers using caffeine. Exp. Neurol. 58: 218–230, 1978.
 398. Wood, D. S., M. M. Sorenson, A. B. Eastwood, W. E. Charash, and J. P. Reuben. Duchenne dystrophy: abnormal generation of tension and Ca2+ regulation in single skinned fibers. Neurology 28: 447–457, 1978.
 399. Young, A., D. P. Brenton, and R. H. T. Edwards. Analysis of muscle weakness in osteomalacia. Clin. Sci. Mol. Med. 54: 31P, 1978.
 400. Young, A., I. Hughes, P. Russell, and M. J. Parker. Measurement of quadriceps muscle wasting. Ann. Rheum. Dis. 38: 571, 1979.
 401. Young, V. R., and H. N. Munro. Muscle protein turnover in human beings in health and disease. In: Degradative Processes in Heart and Skeletal Muscle, edited by K. Wildenthal. Amsterdam: Elsevier, 1980, p. 271–291.

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How to Cite

R. H. T. Edwards, D. A. Jones. Diseases of Skeletal Muscle. Compr Physiol 2011, Supplement 27: Handbook of Physiology, Skeletal Muscle: 633-672. First published in print 1983. doi: 10.1002/cphy.cp100120