Abstract
Myasthenia gravis is a chronic disease characterized by weakness and abnormal fatigability of skeletal muscle. The muscles innervated by the cranial nerves are particularly affected, and usually those of the neck, trunk, and extremities. In severe cases, weakness of the muscles of respiration occurs. Smooth and cardiac muscles are not involved. The disease usually becomes generalized, but in a minority of cases it remains localized to the extraocular muscles. The symptoms are commonly ameliorated, although to a variable degree, by anti-cholinesterase (anti-ChE) compounds. This response serves as the basis for diagnosis and management of the disease.
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Literature
Akao, S., and H. Kawaishi: Case of myasthenia gravis cured by partial parathyroidectomy. Rinshö-Naika Shönika 7, 281 (1952).
Anderson, H. J., C. H. Churchill-Davidson and A. T. Richardson: Bronchial neoplasm with myasthenia: Prolonged apnoea after administration of succinylcholine. Lancet 1953 II, 1291.
Aranow, H. jr., P. F. A. Hoefer and L. P. Rowland: The long-acting anticholinesterase drugs in the management of myasthenia gravis. J. chron. Dis. 6, 113–125 (1958).
Bergh, N. P.: Reaction of patients with myasthenia gravis to different agents causing neuromuscular block. Scand. J. clin. Lab. Invest. 5, 11 (1953).
Bergh, N. P.: Thymectomy in Treatment of myasthenia gravis. Acta chir. scand. (Suppl.) 1753, 1 (1953).
Bickerstaff, E. R., and A. L. Woolf: The intramuscular nerve endings in myasthenia gravis. Brain 83, 10–23 (1960).
Blalock, A., A. M. Harvey, F. R. Ford and J. L. Lilienthal jr.: The treatment of myasthenia gravis by removal of the thymus gland. J. Amer. med. Ass. 117, 1529 (1941).
Botelho, S. Y.: Alterations in muscle tension without similar changes in electrical activity in patients with myasthenia gravis. J. clin. Invest. 34, 1403 (1955a).
Bergh, N. P.: Comparison of simultaneously recorded electrical and mechanical activity in myasthenia gravis patients and in partially curarized normal humans. Amer. J. Med. 19, 693 (1955 b).
Brooks, V. B.: The action of botulinum toxin on motor nerve filaments. J. Physiol. (Lond.) 123, 501 (1954).
Burns, B. D., and W. D. M. Paton: Depolarization of the motor end-plate by decamethonium and acetylcholine. J. Physiol. (Lond.) 115, 41 (1951).
Churchill-Davidson, H. C., and A. T. Richardson: Neuromuscular transmission in myasthenia gravis. J. Physiol. (Lond.) 122, 252–263 (1953).
Coers, C., and J. E. Desmedt: Evidence for characteristic malformation of the neuromuscular junction in myasthenia gravis. Acta neurol. belg. 59, 539–561 (1959).
Comroe, J. H. Jr., J. Todd, G. D. Gammon, I. H. Leopold, G. B. Koelle, O. Bodansky and A. Gilman: The effect of di-isopropyl-fluorophosphate (DFP) upon patients with myasthenia gravis. Amer. J. med. Sei. 212, 641–651 (1946).
Desmedt, J. E.: Neurochemical lesion in myasthenia gravis. Fed. Proc. 18, 36 (1959).
Dulce, N. J.: Behandlung der myasthenia gravis und psychischer Störungen bei der Chorea minor mit Glutaminsäure. Münch, med. Wschr. 96, 1235 (1954).
Dunlop, D. M.: Thymectomy in myasthenia gravis. Trans, med. chir. Soc. Edinb. 132, 59 (1953).
Eaton, L. M., O. P. Clagett and J. A. Bastron: Thymus and its relationship to diseases of nervous system. Study of 374 cases of myasthenia gravis and comparison of 87 patients undergoing thymectomy with 225 controls. A. Res. Nerv. a. Ment. Dis., Proc. 32, 107 (1953).
Elam, J. O., D. G. Green, M. A. Schneider, H. M. Ruben, A. S. Gordon, R. F. Hustead, D. W. Benson, J. A. Clements and A. Ruben: Head-tilt method of oral resuscitation. J. Amer. med. Ass. 173, 812–815 (1960).
Elam, J. O., D. G. Green, E. S. Brown and J. A. Clements: Oxygen and carbon dioxide exchange and energy cost of expired air resuscitation. J. Amer. med. Ass. 167, 328–334 (1958).
Fatt, P.: Biophysics of junctional transmission. Physiol. Rev. 34, 674 (1954).
Ferguson, F. R., E. C. Hutchinson and L. A. Liversedge: Myasthenia gravis; Results of medical management. Lancet 1955 II, 636.
Granirer, L. W.: Beneficial effect of postpartum plasma in a case of myasthenia gravis. Conn. med. J. 172, 934 (1953).
Grob, D.: Course and management of myasthenia gravis. J. Amer. med. Ass. 153, 529 (1953).
Grob, D.: Effect of Sarin in patients with myasthenia gravis. Progress Report to U.S. Army Chemical Corps Medical Laboratories, Army Chemical Center, Med. (1955).
Grob, D.: The manifestations and treatment of poisoning due to nerve gas and other organic phosphorus anticholinesterase compounds. Arch, intern. Med. 98, 221–239 (1956).
Grob, D.: The neuromuscular system. Chapter 30 in Clinical Physiology. 725–756. Ed. by Arthur Grollman. New York: McGraw-Hill Inc. 1957.
Grob, D.: Myasthenia gravis: Current status of pathogenesis, clinical manifestations and management. J. chron. Dis. 8, 536–566 (1958).
Grob, D., and A. M. Harvey: Observations on the effects of tetraethyl pyrophosphate (TEPP) in man and on its use in the treatment of myasthenia gravis. Bull. Johns Hopk. Hosp. 84, 533–567 (1949).
Grob, D., and A. M. Harvey: Effect of adrenocorticotropic hormone (ACTH) and cortisone administration in patients with myasthenia gravis and report of onset of myasthenia gravis during prolonged cortisone administration. Bull. Johns Hopk. Hosp. 91, 124–136 (1952).
Grob, D., and A. M. Harvey: Abnormalities in neuromuscular transmission, with special reference to myasthenia gravis. Amer. J. Med. 15, 695–709 (1953).
Grob, D., and R. J. Johns: Use of oximes in the treatment of intoxication by anticholinesterase compounds in patients with myasthenia gravis. Amer. J. Med. 24, 512–518 (1958 a).
Grob, D., and R. J. Johns: Treatment of anticholinesterase intoxication in normal subjects and myasthenia patients with oximes. J. Amer. med. Ass. 166, 1855–1858 (1958b).
Grob, D., and R. J. Johns: Use of the oxime 1,1 ’-trimethylene bis-(4-formylpyridinium bromide) dioxime (EA1814, TMB-4) in the management of intoxication by anticholinesterase compounds. Contractor’s Progress Report to U.S. Army Chemical Corps. Medical Laboratories, Army Chemical Center, Md., August (1958c).
Furtherstudies on the mechanism of the defect in neuromuscular transmission in myasthenia gravis, with particular reference to the acetylcholine-insensitive block. In H. R. Viets, ed., Myasthenia Gravis: Second Internat. Sympos. Proc. 127–149. Springfield, 111.: Chas. C. Thomas 1961.
M. Harvey: Studies in neuromuscular function. I. Introduction and methods. Bull. Johns Hop. Hosp. 99, 115–124 (1956a).
M. Harvey: Studies in neuromuscular function. III. Stimulating and depressant effects of acetylcholine and choline in normal subjects. Bull. Johns Hopk. Hosp. 99, 136–152 (1956b).
M. Harvey: Studies in neuromuscular function. IV. Stimulating and depressant effects of acetylcholine and choline in patients with myasthenia gravis, and their relationship to the defect in neuromuscular transmission. Bull. Johns Hopk. Hosp. 99, 153–181 (1956c). Studies in neuromuscular function.
M. Harvey: Effects of anticholinesterase compounds, d-tubocurarine, and decamethonium in normal subjects. Bull. Johns Hopk. Hosp. 99, 195 (1956 d).
M. Harvey: Studies in neuromuscular function. VI: Effects of anticholinesterase compounds, d-tubocurarine, and decamethonium in patients with myasthenia gravis. Bull. Johns Hopk. Hosp. 99, 219–238 (1956e).
M. Harvey: Alterations in neuromuscular transmission in myasthenia gravis, as determined by studies of drug action. Amer. J. Med. 19, 684–690 (1955).
M. Harvey: A. Liljestrand and R. J. Johns: Potassium movement in normal subjects and in patients with familial periodic paralysis. A.er. J. Med. 23, 340–375 (1957).
M. Harvey, and K. E. Osserman: Unpublished observations (1958).
Harvey, A. M., and J. L. Lilienthal: Observations on the nature of myasthenia gravis. The intra-arterial injection of acetylcholine, prostigmine and adrenaline. Bull. Johns Hopk. Hosp. 69, 566 (1941).
Harvay, A. M., J. L. Lilienthal Jr., D. Grob, B. F. Jones and S. A. Talbot: The administration of di-isopropyl fluorophosphate to man: IV. The effects on neuromuscular function in normal subjects and in myasthenia gravis. Bull. Johns Hopk. Hosp. 81, 267–292 (1947).
S. A. Talbot: Observations on the nature of myasthenia gravis. The effect of thymectomy on neuromuscular transmission. J. clin. Invest. 21, 579 (1942).
Harvay, A. M., and R. L. Masland: The electromyogram in myasthenia gravis. Bull. Johns Hopk. Hosp. 69, 1 (1941).
Harvay, A. M., and M. R. Whitehill: Prostigmine as aid in diagnosis of myasthenia gravis. J. Amer. med. Ass. 108, 1329 (1937).
Harvay, A. M., Quinine as adjuvant to prostigmine in diagnosis of myasthenia gravis. Preliminary report. Bull. Johns Hopk. Hosp. 61, 216 (1937).
Herzfeld, E., O. Kraupp, K. Pateisky u. C. Stumpf: Pharmakologische und klinische Wirkungen des Cholinesterashemmkörpers Hexamethylen-bis-(N-methyl-carbaminoyl-l-me-thyl-3-oxypyridiniumbromid) (BC 51) (Pharmacological and clinical effects of the cholinesterase inhibitor hexamethylene-bis-(N-methylcarbaminoyl-l-methyl-3-oxypyridinium bromide) (BC 51). Wien. kin. Wschr. 69, 245–248 (1957).
Hoefer, P. F. A., H. Aronow and L. P. Rowland: Therapy of myasthenia gravis. Neurology 3, 691 (1953).
Hutter, O. F.: Effect of choline on neuromuscular transmission in the cat. J. Physiol. (Lond.) 117, 241 (1952).
Irwin, R. U., and M. J. Smith III: Cholinesterase inhibition by galanthamine and lycoramine. Biochem. Pharmacol. 3, 147–148 (1960).
Johns, R. J.: Factors influencing muscle membrane potential in man, as recorded with intracellular microelectrodes. J. Pharmacol, exp. Ther. 122, 36a (1958).
Johns, R. J., D. Grob and A. M. Harvey: Electromyographic changes in myasthenia gravis. Amer. J. Med. 19, 679–683 (1955).
Johns, R. J.: Studies in neuromuscular function. II. Effects of nerve stimulation in normal subjects and in patients with myasthenia gravis. Bull. Johns Hopk. Hosp. 99, 125–135 (1956).
Jorgensen, J. B., and F. Therkelsen: Thymectomy in the treatment of myasthenia gravis. Acta chir. scand. 107, 414 (1954).
Katz, B., and S. Thesleff: A study of the “desensitization” produced by acetylcholine at the motor endplate. J. Physiol. (Lond.) 138, 63 (1957).
Keynes, G.: Investigation into thymic disease and tumor formation. Brit. J. Surg. 42, 449 (1955).
Kuffler, S. W.: Specific excitability of the endplate region in normal and denervated muscle. J. Neurophysiol. 6, 99–110 (1943).
Lammers, W., and D. Most van Spijk: Unsuccessful attempt to demonstrate a paralytic factor in serum of myasthenia gravis patients. Nature (Lond.) 173, 1192 (1954).
MacDermot, V.: The changes in the motor endplate in myasthenia gravis. Brain 83, 24–35 (1960).
McSweyn, N. F., and H. Schwartz: Thymectomy in myasthenia gravis. Canad. med. Ass. J. 70, 311 (1954).
Merrill, G. G.: Neostigmine toxicity: Report of fatality following diagnostic test for myasthenia. J. Amer. med. Ass. 137, 362 (1948).
Mertens, H. G.: Über den Verlauf der Myasthenie nach Carotissinus-denervierung. Nervenarzt 26, 150 (1955).
Nowell, P. T., and A. Wilson: Isolation of quaternary ammonium compounds from the extracts of thymus glands. In H. R. Viets, ed., Myasthenia Gravis: Second Internat. Sympos. Proc., 238–256. Springfield, Hl.: Chas. C. Thomas 1961.
Osserman, K. E., E. S. Cohen and G. Genkins: Phospholine iodide: An anticholinesterase drug of new structure. Preliminary report in the treatment of myasthenia gravis. In H. R. Viets, ed., Myasthenia Gravis: Second Internat. Sympos. Proc., 581–594. Springfield, 111.: Chas. C. Thomas 1961.
Osserman, K. E.,, and L. I. Kaplan: Studies in myasthenia gravis. Present status of therapy with octamethyl pyrophosphoramide (OMPA). Ann. intern. Med. 41, 108 (1954).
Osserman, K. E., E. S. Cohen and G. Genkins: Studies in myasthenia gravis: Use of edrophonium chloride (Tensilon) in differentiating myasthenic from cholinergic weakness. Arch. Neurol. Psychiat. (Chicago) 70, 385 (1953).
Osserman, K. E., E. S. Cohen and G. Genkins: Rapid diagnostic test for myasthenia gravis: Increased muscle strength, without fasciculation, after intravenous administration of edrophonium (tensilon) chloride. J. Amer. med. Ass. 150, 265 (1952).
Osserman, K. E., P. Teng and L. I. Kaplan: Studies in myasthenia gravis. Preliminary report on therapy with Mestinon bromide. J. Amer. med. Ass. 155, 961 (1954).
Pateisky, K., E. Herzfelt and C. Stumpf: Der Effekt von Polymethylen-bis (N-methyl-carbaminolyl-m-Trimethylammoniumphenolen) BC 40, BC 47, BC 48 auf Cholinesterase-aktivat und Muskeltätigkeit bei Myasthenia gravis pseudoparalytica. Wien. klin. Wschr. 69, 2–7 (1957).
Pateisky, K., O. Kraupp u. C. Stumpf: Klinische Erfahrungen mit Hexamethylen-bis-(N-methyl-carbaminoyl-m-trimethylammoniumphenol) (BC 40) bei Myasthenia gravis pseudoparalytica. Wien. klin. Wschr. 67, 578 (1955).
Pelikan, E. W., J. E. Tether and K. R. Unna: Sensitivity of myasthenia gravis patients to tubocurarine and decamethonium. Neurology 3, 284 (1953).
Randt, C. T.: Myasthenia gravis. Med. Clin. N. Amer. 37, 535 (1953).
Rider, J. A., S. Schulman, R. B. Richter, H. D. Moeller and K. P. DuBois: Treatment of myasthenia gravis with octamethyl pyrophosphoramide. J. Amer. med. Ass. 145, 967 to 972 (1951).
Rowland, L. P., M. C. Korengold, I. A. Jaffe, L. Berg and G. M. Shy: Prostigmine induced muscle weakness in myasthenia gravis patients. Neurology 5, 89 (1955).
Roe. J. H., R. J. Johns and D. Grob: Unpublished observations (1955).
Safar, P.: Mouth-to-mouth airway. Anesthesiology 18, 904 (1957).
Roe. J. H., Ventilatory efficacy of mouth-to-mouth artificial respiration. J. Amer. med. Ass. 167, 335–341 (1958).
Roe. J. H., L. A. Escarrago and J. O. Elam: A comparison of the mouth-to-mouth and mouth-to-airwav methods of artificial respiration with the chest-pressure arm-lift methods. New Engl. J. Med. 258, 671–677 (1958).
Schaumann, W., and C. Job: Differential effects of quaternary Cholinesterase inhibitor, phos-pholine, and its tertiary analogue, compound 217-AO, on central control of respiration and on neuromuscular transmission. The antagonism by 217-AO of the respiratory arrest caused by morphine. J. Pharmacol, exp. Ther. 123, 114–120 (1958).
Schulman, S., J. A. Rider and R. B. Richter: Use of octamethyl pyrophosphoramide in the treatment of myasthenia gravis. J. Amer. med. Ass. 152, 1707 (1953).
Schwab, R. S.: WIN-8077 in the treatment of sixty myasthenia gravis patients. A twelve month report. Amer. J. Med. 19, 734 (1955).
Schwab, R. S., and C. Leland: Sex and age in myasthenia gravis as critical factors in incidence and remission. J. Amer. med. Ass. 153, 1270 (1953).
Schwab, R. S., C. K. Marshall and W. T. Timberlake: WIN 8077 in treatment of myasthenia gravis, use of N,N’-bis (2-diethyl-aminoethyl) oxamide bis-2-chlorobenzyl chloride in 50 patients. J. Amer. med. Ass. 158, 625–628 (1955).
Schwab, R. S., K. E. Osserman and J. E. Tether: Treatment of myasthenia gravis. J. Amer. med. Ass. 165, 671–674 (1957).
Schwarz, H.: Urecholine in myasthenia gravis. Canad. med. Ass. J. 72, 346 (1955).
Schwarz, H.: Curare-like factor in serum of myasthenia gravis patients. Canad. med. Ass. J. 67, 238 (1952).
Seibert, P.: Zur Behandlung der Myasthenia gravis. Klinische Erfahrungen über die Behandlung der Myasthenia gravis pseudoparalytica mit Pyridostigmin. Dtsch. med. Wschr. 78, 805 (1953).
Simpson, J. A.: Discussion on myasthenia. The value of thymectomy. Proc. roy. Soc. Med. 49, 795 (1956).
Simpson, J. A.: Myasthenia gravis: A new hypothesis. Scot. med. J. 5, 419–436 (1960).
Strauss, A. J. L., B. C. Seegal, K. S. Hsu, P. M. Burkholder, W. L. Nastuk and K. E. Osserman: Immunofluorescence demonstration of a muscle binding complement-fixing serum globulin fraction in myasthenia gravis. Proc. Soc. exp. Biol. (N. Y.) 105, 184–191 (1960).
Tether, J. R.: Treatment of myasthenia gravis with mestinon bromide. J. Amer. med. Ass. 160, 156 (1956).
Tether, J. E.: Intravenous neostigmine in diagnosis of myasthenia gravis. Ann. intern. Med. 29, 1132 (1948).
Thevenard, A.: Les effets de Innervation sin-carotidienne sur la myasthenia bulbospinal essai d’interprétation. Rev. neurol. 90, 107 (1954).
Torda, C., and H. G. Wolf: Depression of acetylcholine synthesis by serum from working muscle, healthy subjects and myasthenia gravis patients. Proc. Soc. exp. Biol. (N. Y.) 59, 13 (1945).
Tsukiyama, K., A. Nakai, R. Mine and T. Kitani: Studies on a myasthenic substance present in the serum of patient with myasthenic gravis. Med. J. Osaka Univ. 10, 159–170 (1959).
Maanen, E. F.: Neuromuscular blocking agents. Amer. J. Med. 19, 669 (1955).
Viets, H. R.: Thymectomy for myasthenia gravis. J. Amer. med. Ass. 151, 1248 (1953).
Viets, H. R.: Diagnosis of myasthenia gravis in patients with dysphagia. J. Amer. med. Ass. 134, 988 (1947).
Viets, H. R., and R. S. Schwab: Prostigmin in the diagnosis of myasthenia gravis. New Engl. J. Med. 213, 1280 (1935).
Westebb erg, M. R.: Clinical evaluation of ambenomium (Mysuran) chloride. Arch. Neurol. Psychiat. (Chicago) 75, 91 (1956).
Westebberg, M. R., and K. R. Magee: Treatment review. Myasthenia gravis. Neurology 5, 729 (1955).
F. E. Shideman: Effect of 3-hydroxy phenyldimethylethylammonium chloride (Tensilon) in myasthenia gravis. Univ. Mich. med. Bull. 17, 311 (1951).
F. E. Shideman, and J. T. Lures: The clinical use of hexaethyltetraphosphate in myasthenia gravis. Univ. Mich. med. Bull. 14, 15 (1948).
Wilson, A., G. A. Man and H. Georhegan: Cholinesterase activity of blood and muscle in myasthenia gravis. Quart. J. Med. 20, 13 (1951).
Wilson. A., G. A., and H. B. Stoner: Myasthenia gravis. A consideration of its causation in a study of 14 cases. Quart. J. Med. 13, 1 (1944).
Zaimis, E. J.: Motor endplate differences as a determining factor in the mode of action of neuromuscular blocking substances. J. Physiol. (Lond.) 122, 238 (1953).
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Grob, D. (1963). Therapy of Myasthenia Gravis. In: Koelle, G.B. (eds) Cholinesterases and Anticholinesterase Agents. Handbook of Experimental Pharmacology, vol 15. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-99875-1_23
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