重症筋無力症(Myasthenia gravis:MG)

1 ( myasthenia gravis :MG).. 1/74.. The Cochrane database MEDLINE . Agency for Health Care Policy and Research AHCPR .. 2 . MG. PSL. AChR. 2/74.. 1.. 2.. 3.. 4.. 5.. 1.. 2.. 3.. 4.. 5.. 1.. 2.. 3.. 4.. 5.. 1.. 2.. 3.. 4.. 1. tacrolims hydrate (FK506 ). A.. B.. C.. D.. 2. azathioprine AZP . A.. B.. C.. D.. 3. ciclosporine (CYA ). A.. 3/74. B.. C.. D.. 4. cyclophosphamide (CPA ). A.. B.. C.. D.. 5. mycophenolate mofetil . A.. B.. C.. D.. 1.. 2.. 3.. A.. B. (double filtration plasmapheresis DFPP). C. (immuno absorption IA).. 1.. 2.. 3.. 4.. MG .. MG .. MG .. MG ..Sero-negative MG .. 4/74.. 1.. (ACh) . AChR T AChR . AChR . B T . MHC AChR . positive negative selection . [8] . AChR MG seronegative MG . [1] . 2.. 3.. 1 . AChR . seronegative MG .. 4.. Osserman 2 myasthenia gravis Foundation of America MGFA score 3 4 ADL 5 7.

2 5.. 1987 [1] 10 1 . 70 5 . 1995 [2] 10 . 30 10 50 20 . 32 30 50 37-48 . MG ADL . QOL .. 5/74.. Lawrence [3] 1950 1995 MG 33 .. MG .. 4 Uono 1973 10 [4] Kuroiwa 1977 [5] Araki 1982 . [6] MG .. MG 1965 . 1/4 MG . [7] MG .. 1) .. 62 1988 pp. 227-245 . 2) . QOL .. 7 1995 pp. 19-21 . 3) Lawrence HP , James CT. Epidemiologic evidence for a changing natural history of myasthenia gravis . Neurology 1996; 47: 1233-1238. 4) Uono M. Clinical statistics of myasthenia gravis in Japan. Int J Neurol 1980; 14: 87-99. 5) Kuroiwa Y. Epidemiological aspects of myasthenia gravis in Japan. In: Satoyoshi E, ed. myasthenia gravis : pathogenesis and treatment. Tokyo: University of Tokyo Press. 1981;9-17. 6) Araki S, Uchino M, Kumamoto T. Prevalence studies of multiple sclerosis, myasthenia gravis , and myopathies in Kumamoto district, Japan.

3 Neuroepidemiology 1987; 6: 120-129. 7) Oosterhuis HJ. The natural course of myasthenia gravis : a long term follow up study. J. Neurol Neurosurg Psychiatry 1989; 52: 1121-1127. 8) Levinson AI, Wheatley LM. The thymus and the pathogenesis of myasthenia gravis . Immunopatho. 1996; 78; 1-15. 6/74.. 1932 MG 1 2 .. MG .. MEDLINE Cochran Library evidence .. 1.. pyridostigmine bromide ( ) distigmine bromide ( ) . ambenomium chloride ( ) neostigmine bromide ( ) . neostigmine bromide edrophonium chloride ( . ) pyridostigmine bromide .. pyridostigmine bromide 1 (60mg)/ . / . ambenomium chloride distigmine bromide .. 2.. 1950-1951 edrophonium chloride test (tensilon test) Osserman 3 25000 . edrophonium chloride 90-95% .. 1 (10mg) . 2mg 15 30 45 .. 3.. 7/74. 4.. ( ) AV .. Hudson 4 neostigmine.

4 Neostigmine . Tiedtand 5 neostigmine .. MG .. Munsat14 MG . MG .. drug holiday . Patten 6 .. 5.. 1) [ ] Walker MB. Treatment of myasthenia gravis with physostigmine. Lancet 1200-1201, 1934. 2) [ ] Walker MB. Case showing the effect of prostigmin on myasthenia gravis . Proceed Royal Soci Med XXVIII,448:759-760, 1935.. 3) Osserman KE, Genkins G, Critical reappraisal of the use of edrophonium (tensilon). chloride test in myasthenia gravis and significance of clinical classification. Ann NY Acad Sci 35:312-326,1966. 8/74. 4) Hudson CS, Rash JE, Tiedt TN et al. Neostigmine-induced alterations at the mammalian neuromuscular junction. II. Ultrastructure. J Phrma Exper Therap 205:341-356, 1978. 5) Tiedtand TN, Alubuquerque EX, Hudson CS et al. Neostigmine-induced alterations at the mammalian neuromuscular junction.

5 I. muscle contraction and electrophysiology. J Phrma Exper Therap 205:326-339, 1978. 6) [ ] Patten BM, Oliver KL, Engel WK. Adverse interaction between steroid hormones and anticholinesterase drugs. Neurology 24: 442-449, 1974. 9/74.. MG MG .. 1.. 1939 Blalock 21 MG MG . [1] MG 20 . MG [2] . MG .. 2.. 1975 . trans-sternal thymectomy . trans-cervical thymectomy extended thymectomy ET MG . Blalock [2] . 1960 .. ET . 286. 89 ET 20 [3] . 6 1 3 5 10 . 15 20 6 1 3 . 5 10 15 20 6. 3 10 .. MG ET . 1987 [4] 1533 832 . [3] 295 . 378 159 .. [5] .. 100-200 rad 4000 rad . [6] . 10/74. 3.. MG MG . 1 2 AChR 3 . 4 5 . MHC II 6 7 positive negative selection .. MG MG . 4.. MG . randamised controlled trial RCT 2000. Gronseth & Barohn MG 310 . 28 21 [7] . MG medication-free remission asymptomatic.

6 Improved survived 21 . relative rate [8-36] . 1 Evoli et al. [12] Werneck et al. [11] Grob et al. [19 20 21] . 18 MG medication-free asymptomatic . improved relative rate 2 .. 3 MG. MG improved 4 . MG improved . trans-cervical trans-sternal and extended trans-sternal MG .. MG . well-designed prospective controlled study . 5.. 1970. 5-15 [21 25 26] 1970 . 1 MG . [36] 33 . 11 . [36] Video-assisted thoracoscopy thymectomy [37] . 11/74.. MG .. 1 .. 1) Blalock A, Mason MF, Morgan HJ, et al. myasthenia gravis and tumors of the thymic region. Report of a case in which the tumor was removed. Ann Surg110:544-561,1939. 2) Blalock A. Thymectomy in the treatment of myasthenia gravis . Report of twenty cases. J Thorac Surg 13:316-339, 1944. 3) Masaoka A, Yamakawa Y, Niwa H, et al Extended thymectomy for myasthenia gravis patients: a 20- year review.

7 Ann Thorac Surg. 62(3):853-859, 1996. 4) .. 62 ,227-245, 1988. 5) Bril V, Kojic J, Dhanani A. The long-term clinical outcome of myasthenia gravis in patients with thymoma. Neurology. 51(4): 1198-1200, 1998. 6) Monden Y, Nakahara K, Nanjo S, et al. Invasive thymoma with myasthenia gravis Cancer, 54:2513-2518, 1984. 7) Gronseth GS, Barohn RJ. Practice parameter: thymectomy for autoimmune myasthenia gravis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 55:7-15, 2000. 8) (III cohort study) Christensen PB, Jensen TS, Tsiropoulos I, et al. Mortality and survival in myasthenia gravis : a Danish population based study. J Neurol Neurosurg Psychiatry 64:78-83, 1998. 9) ( b) Evoli A, Batocchi AP, Lino M, Tonali P.

8 Thymectomy for early-onset myasthenia gravis : a case-control study. Neuromuscul Disord 8:270, 1998. 10) ( cohort study) Beekman R, Kuks JBM, Oosterhuis HJGH. myasthenia gravis : diagnosis and follow-up of 100 consecutive patients. J Neurol 244:112-118, 1997. 11) ( b) Werneck LC, Cunha FMF, Scola RH. myasthenia gravis a study comparing thymectomy, and conservative treatment. Neurology 46:A334. Abstract, 1996. 12) ( cohort study) Evoli A, Batocchi AP, Tonali P, Palmisani MT, Lino M. Thymectomy for late-onset myasthenia gravis . Neurology 46:A310. Abstract, 1996. 13) ( cohort study) Beghi E, Antozzi C, Batocchi AP, et al. Prognosis of myasthenia gravis : a multicenter follow-up study of 844 patients. J Neurol Sci 106:213-220, 1991. 12/74. 14) ( cohort study) Valli G, Jann S, Premoselli S, Scarlato G.

9 myasthenia gravis treatment: twelve years experience on 110 patients. Ital J Neurol Sci 8:593-601,1987. 15) ( cohort study) Mantegazza R, Beghi E, Pareyson D, et al. A multicentre follow-up study of 1152 patients with myasthenia gravis in Italy. J Neurol 237:339-344, 1990. 16) ( cohort study) Scoppetta C, Tonali P, Evoli A, et al. Treatment of myasthenia gravis . Report of 139 patients. J Neurol 222:11-21, 1979. 17) ( cohort study) Donaldson DH, Ansher M, Horan S, Rutherford RB, Ringel SP. The relationship of age to outcome in myasthenia gravis . Neurology 40:786-790, 1990. 18) ( cohort study) Papatestas AE, Genkins G, Kornfeld P, et al. Effects of thymectomy in myasthenia gravis . Ann Surg 206:79-88, 1987. 19) ( cohort study) Grob D, Arsura EL, Brunner NG, Namba T. The course of myasthenia gravis and therapies affecting outcome.

10 Ann NY Acad Sci 505:472-499, 1987. 20) ( cohort study) Grob D, Brunner NG, Namba T. The natural course of myasthenia gravis and effect of therapeutic measures. Ann NY Acad Sci 377:652-669, 1981. 21) ( cohort study) Grob D. Course and management of myasthenia gravis . JAMA. 153:529-532, 1953. 22) ( cohort study) Scadding GK, Havard CWH, Lange MJ, Domb I. The long term experience of thymectomy for myasthenia gravis . J Neurol Neurosurg Psychiatry 48:401-406, 1985. 23) ( cohort study) Rodriguez M, Gomez MR, Howard FM, Taylor WF. myasthenia gravis in children: long-term follow-up. Ann Neurol 13:504-510, 1983. 24) ( cohort study) Oosterhuis HJ. Observations of the natural history of myasthenia gravis and the effect of thymectomy. Ann NY Acad Sci 377:678-690, 1981. 25) ( b) Buckingham JM, Howard FM, Bernatz PE, et al.