Lifetime course of myasthenia gravis
David Grob MD
Department of Medicine, Maimonides Medical Center, Brooklyn, New York
Search for more papers by this authorNorman Brunner MD
Department of Medicine, Maimonides Medical Center, Brooklyn, New York
Search for more papers by this authorTatsuji Namba MD
Department of Medicine, Maimonides Medical Center, Brooklyn, New York
Search for more papers by this authorCorresponding Author
Murali Pagala PhD
Neuromuscular Research Laboratory, Department of Surgery, Maimonides Medical Center, 4802 Tenth Avenue, Brooklyn, New York 11219
Neuromuscular Research Laboratory, Department of Surgery, Maimonides Medical Center, 4802 Tenth Avenue, Brooklyn, New York 11219Search for more papers by this authorDavid Grob MD
Department of Medicine, Maimonides Medical Center, Brooklyn, New York
Search for more papers by this authorNorman Brunner MD
Department of Medicine, Maimonides Medical Center, Brooklyn, New York
Search for more papers by this authorTatsuji Namba MD
Department of Medicine, Maimonides Medical Center, Brooklyn, New York
Search for more papers by this authorCorresponding Author
Murali Pagala PhD
Neuromuscular Research Laboratory, Department of Surgery, Maimonides Medical Center, 4802 Tenth Avenue, Brooklyn, New York 11219
Neuromuscular Research Laboratory, Department of Surgery, Maimonides Medical Center, 4802 Tenth Avenue, Brooklyn, New York 11219Search for more papers by this authorAbstract
Between 1940 and 2000 a total of 1976 patients with myasthenia gravis (MG) were studied. Diagnosis was made by improvement in weakness after anticholinesterase medication. The historical developments in diagnosis and treatment of MG are reviewed. We analyzed the clinical course of MG as influenced by age, gender, thymectomy, thymomectomy, and the presence of antibodies to acetylcholine receptors (AChR). The clinical course of MG was significantly influenced by age and gender, and these need special attention in managing patients. The most severe level of weakness and high mortality occurred during the first 1 to 2 years of the disease, after which many patients experienced improvement. For treating MG patients the usefulness of thymectomy remains to be proven, and novel drugs need to be developed to increase the number as well as normal functioning of the AChRs and other components of the neuromuscular system. Muscle Nerve, 2007
REFERENCES
- 1 Aarli JA, Stefansson K, Marton LSG, Wollmann RL. Patients with myasthenia gravis and thymoma have in their sera IgG autoantibodies against titin. Clin Exp Imunol 1990; 82: 284–288.
- 2 Blalock A, Mason MF, Morgan HE, Riven SS. Myasthenia gravis and tumors of the thymic region. Report of a case in which the tumor was removed. Ann Surg 1939; 110: 544–561.
- 3 Brunner NG, Namba T, Grob D. Corticosteroids in the management of severe, generalized myasthenia gravis. Neurology 1972; 22: 603–610.
- 4
Campbell H,
Bramwell E.
Myasthenia gravis pseudoparalytica: review of 70 case reports, including nine new patients.
Brain
1900;
23:
277–336.
10.1093/brain/23.2.277 Google Scholar
- 5 Dau PC, Lindstrom JM, Cassel CK, Denys EH, Shev EE, Spitler LE. Plasmapheresis and immunosuppressive drug therapy in myasthenia gravis. N Engl J Med 1977; 297: 1134–1140.
- 6 Deymeer F, Gungor-Tuncer O, Yilmaz V, Parman Y, Serdaroglu P, Ozdemir C, et al. Clinical comparison of anti-MuSK- vs anti-AChR-positive and seronegative myasthenia gravis. Neurology 2007; 68: 609–611.
- 7 Domagk G. Eine neue Klasse von Desinfection-mitteln. Dtsch Med Wochenschr 1935; 61: 829–832.
- 8
Erb W.
Zur casuistic der bulbaren lahmungen.
Arch Psychiat
1879;
9:
325–350.
10.1007/BF02666475 Google Scholar
- 9 Fleming A. Penicillin, its practical application. Philadelphia: Blakestone; 1946. p 1–33.
- 10 Flood PR, Bjugn R, Gilhus NE, Hofstad H, Matre R, Aarlie JA. The ultrastructural localization of antigens for antibodies in myasthenia gravis. Ann N Y Acad Sci 1987; 505: 732–734.
- 11
Goldflam S.
Ueber einen scheinbar heilbaren bulbarparalytischen Symptomen-complex mit Betheiligung der Extremitaten.,
Dtsch Ztschr Nervenh
1893;
4:
312–352.
10.1007/BF01665294 Google Scholar
- 12
Grob D.
Course and management of myasthenia gravis.
JAMA
1953;
1153:
529–532.
10.1001/jama.1953.02940230001001 Google Scholar
- 13 Grob D. Myasthenia gravis. Current status of pathogenesis, clinical manifestations, and management. J Chronic Dis 1958; 8: 536–566.
- 14 Grob D. Myasthenia gravis. A review of pathogenesis and treatment. Arch Intern Med 1961; 108: 615–638.
- 15 Grob D. Clinical manifestations of myasthenia gravis. In: EX Albuquerque, AT Elderefrawi, editors. Myasthenia gravis. London: Chapman and Hall; 1983. p 319–345.
- 16 Grob D, Arsura EL, Brunner NG, Namba T. The course of myasthenia gravis and therapies affecting outcome. Ann N Y Acad Sci 1987; 505: 472–499
- 17 Grob D, Brunner NG, Namba T. The natural course of myasthenia gravis and effect of therapeutic measures. Ann N Y Acad Sci 1981; 377: 652–669.
- 18 Grob D, Harvey AM. Abnormalities of neuromuscular transmission with special reference to myasthenia gravis. Am J Med 1953; 15: 695–709.
- 19 Grob D, Johns RJ, Harvey AM. Studies in neuromuscular function in normal subjects and patients with myasthenia gravis. Bull Johns Hopkins Hosp 1956; 99: 115–238.
- 20 Grob D, Namba T. Corticotropin in generalized myasthenia gravis. Effect of short intensive courses. JAMA 1966; 198: 703–707.
- 21 Hoch W, McConville J, Helmes S, Newsom-Davis J, Melms A, Vincent A. Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies. Nat Med 2001; 7: 365–368.
- 22
Iwasa K.
Striational autoantibodies in myasthenia gravis mainly react with ryanodine receptor.
Muscle Nerve
1997;
20:
753–756.
10.1002/(SICI)1097-4598(199706)20:6<753::AID-MUS16>3.0.CO;2-V CAS PubMed Web of Science® Google Scholar
- 23 Jaretzki A, Bethea M, Wolff M, Olarte MR, Lovelace RE, Penn AS, et al. A rational approach to total thymectomy in the treatment of myasthenia gravis. Ann Thorac Surg 1977; 24: 120–130.
- 24 Jolly F. Ueber Myasthenia gravis pseudoparalytica, Berlin klin Wchuschr 1895; 32: 1–7.
- 25 Keesey JC. Clinical evaluation and management of myasthenia gravis. Muscle Nerve 2004; 29: 484–505.
- 26 Kennedy FS, Moersch FP. Myasthenia gravis. A clinical review of 87 cases observed between 1915 and the early part of 1932. CMAJ 1937; 37: 216–223.
- 27 Lassen HCA. Preliminary report of the 1952 epidemic of poliomyelitis in Copenhagen with special reference to the treatment of acute respiratory insufficiency. Lancet 1953; 1: 37–41.
- 28 Lindstrom JM, Seybold ME, Lennon VA, Whittingham S, Duane DD. Antibody to acetylcholine receptor in myasthenia gravis. Neurology 1976; 26: 1054–1059.
- 29 Mertens HG, Balzereit F, Leipert M. The treatment of severe myasthenia gravis with immunosuppressive agents. Eur Neurol 1969; 2: 321–339.
- 30 Mygland A, Tysnes OB, Matre R, Volpe P, Aarlie JA, Gilhus NE. Ryanodine receptor antibodies in myasthenia gravis patients with a thymoma. Ann Neurol 1992; 32: 589–591.
- 31 Nakata M, Kuwabara S, Kawaguchi N, Takahashi H, Misawa S, Kanai K, et al. Is excitation-contraction coupling impaired in myasthenia gravis? Clin Neurophysiol 2007; 118: 1144–1148.
- 32 Namba T, Nakata Y, Grob D. The role of humoral and cellular immune factors in neuromuscular block in myasthenia gravis. N Y Acad Sci 1976; 274: 493–515.
- 33
Oppenheim H.
Ueber einen Fall von chronischer progressiver Bulbarparalyse ohne anatomischen Befund.
Virchow's Arch Path Anat
1887;
108:
522–530.
10.1007/BF02281477 Google Scholar
- 34 Pagala MKD, Namba T, Grob D. Desensitization to acetylcholine at motor end plates in normal humans, patients with myasthenia gravis, and experimental models of myasthenia. Ann N Y Acad Sci 1981; 377: 567–582.
- 35 Pagala MKD, Nandakumar NV, Venkatachari SAT, Ravindran K, Namba T, Grob D. Responses of intercostal muscle biopsies from normal subjects and patients with myasthenia gravis. Muscle Nerve 1990; 13: 1012–1022.
- 36 Pagala M, Nandakumar NV, Venkatachari SAT, Ravindran K, Amaladevi B, Namba T, et al. Mechanisms of fatigue in normal intercostal muscle and muscle from patients with myasthenia gravis. Muscle Nerve 1993; 16: 911–921.
- 37 Papatestas AE, Alpert KE, Osserman KE, Osserman RS, Kark AE. Studies in myasthenia gravis: effects of thymectomy. Results in 1985 patients with nonthymomatous and thymomatous myasthenia gravis. Am J Med 1971; 50: 465–474.
- 38 Romi F, Aarli JA, Gilhus NE. Myasthenia gravis patients with ryanodine receptor antibodies have distinctive clinical features. Eur J Neurol 2007; 14: 617–620.
- 39 Sanders DB, El-Salem K, Massey JM, McConville J, Vincent A. Clinical aspects of MuSK antibody positive seronegative MG. Neurology 2003; 60: 1978–1980.
- 40 Simpson JA. An evaluation of thymectomy in myasthenia gravis. Brain 1958; 81: 112–144.
- 41 Skeie GO, Romi F, Aarlie JA, Bentsen PT, Gilhus NE. Pathogenesis of myositis and myasthenia associated with titin and ryanodine receptor antibodies. Ann NY Acad Sci 2003; 998: 343–350.
- 42 Strauss AJL, Seegal BC, Hsu KC, Burkholder PM, Nastuk WL, Osserman KE. Immunofluorescence demonstration of a muscle binding, complement-fixing serum globulin fraction in myasthenia gravis. Proc Soc Exper Biol Med 1960; 105: 184–191.
- 43 Walker MB. Treatment of myasthenia gravis with physostigmine. Lancet 1934; 1: 1200–1201.
- 44 Willis T. De Anima Brutorum. Oxford: Theatro Sheldiano; 1672. p 404.