Myasthenia gravis: Diagnosis and follow-up of 100 consecutive patients

被引:129
作者
Beekman, R
Kuks, JBM
Oosterhuis, HJGH
机构
[1] Department of Neurology, University Hospital, 9700 RB Groningen
关键词
myasthenia gravis; early signs; delay in diagnosis diagnostic tests; effect of therapies;
D O I
10.1007/s004150050059
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
One hundred consecutive patients with myasthenia gravis (MG) referred between 1985 and 1989 were analysed for epidemiological characteristics, evolution of early signs, delay in diagnosis, yield of diagnostic tests and effects of treatment. The female to male ratio was 1.6:1.0. Sixteen patients had a thymoma. Ocular MG occurred in 14. Associated autoimmune diseases were found in 15 patients. In 34% of the women and 10% of the men the diagnosis was delayed for more than 2 years. In the first 3 months progression was more rapid in men than in women. Anti-acetylcholine receptor antibodies were found in 94% of the patients with generalized MG and in 29% of the ocular patients. The neostigmine or the edrophonium test was positive in 84% of the generalized and in 60% of the ocular patients. Electromyography was diagnostic in 71% of the generalized and in 42% of the ocular patients tested. Thymectomy was performed in 56 patients (12 with thymomas). Fifty-one per cent were treated with one or more immunosuppressive drugs, at any time. After a mean follow-up of 9.6 years after onset remissions had occurred in 43%, considerable improvement in 25%, moderate improvement in 20% and 12% remained unchanged. There were no deaths due to MG. Thirty-six per cent remained dependent on immunosuppressive drugs. Medication-free remission was most frequent (35%) in the early-onset ( < 50 years) group. Side-effects of pyridostigmine were noted in 34% of 99 patients, of prednisone in 65% of 49 patients, and of azathioprine in 54% of 28 patients, but these necessitated stopping the drug in only 1%, 10% and 14% respectively.
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页码:112 / 118
页数:7
相关论文
共 18 条
[1]   IMMUNOSUPPRESSIVE TREATMENTS - THEIR EFFICACY ON MYASTHENIA-GRAVIS PATIENTS OUTCOME AND ON THE NATURAL COURSE OF THE DISEASE [J].
CORNELIO, F ;
ANTOZZI, C ;
MANTEGAZZA, R ;
CONFALONIERI, P ;
BERTA, E ;
PELUCHETTI, D ;
SGHIRLANZONI, A ;
FIACCHINO, F .
ANNALS OF THE NEW YORK ACADEMY OF SCIENCES, 1993, 681 :594-602
[2]   COMPARATIVE-STUDY ON THE PREVALENCE OF MYASTHENIA-GRAVIS IN THE PROVINCES OF BOLOGNA AND FERRARA, ITALY [J].
DALESSANDRO, R ;
GRANIERI, E ;
BENASSI, G ;
TOLA, MR ;
CASMIRO, M ;
MAZZANTI, B ;
GAMBERINI, G ;
CANIATTI, L .
ACTA NEUROLOGICA SCANDINAVICA, 1991, 83 (02) :83-88
[3]   THE RELATIONSHIP OF AGE TO OUTCOME IN MYASTHENIA-GRAVIS [J].
DONALDSON, DH ;
ANSHER, M ;
HORAN, S ;
RUTHERFORD, RB ;
RINGEL, SP .
NEUROLOGY, 1990, 40 (05) :786-790
[4]   MEDICAL PROGRESS - MYASTHENIA-GRAVIS [J].
DRACHMAN, DB .
NEW ENGLAND JOURNAL OF MEDICINE, 1994, 330 (25) :1797-1810
[5]   THE NATURAL COURSE OF MYASTHENIA-GRAVIS AND EFFECT OF THERAPEUTIC MEASURES [J].
GROB, D ;
BRUNNER, NG ;
NAMBA, T .
ANNALS OF THE NEW YORK ACADEMY OF SCIENCES, 1981, 377 (DEC) :652-669
[6]  
Kuks J B, 1991, Neuromuscul Disord, V1, P423, DOI 10.1016/0960-8966(91)90005-D
[7]   ANTIBODIES TO SKELETAL-MUSCLE IN MYASTHENIA-GRAVIS .1. DIAGNOSTIC-VALUE FOR THE DETECTION OF THYMOMA [J].
KUKS, JBM ;
LIMBURG, PC ;
HORST, G ;
DIJKSTERHUIS, J ;
OOSTERHUIS, HJGH .
JOURNAL OF THE NEUROLOGICAL SCIENCES, 1993, 119 (02) :183-188
[8]   ANTI-ACETYLCHOLINE RECEPTOR ANTIBODIES IN MYASTHENIA-GRAVIS .1. RELATION TO CLINICAL-PARAMETERS IN 250 PATIENTS [J].
LIMBURG, PC ;
THE, TH ;
HUMMELTAPPEL, E ;
OOSTERHUIS, HJGH .
JOURNAL OF THE NEUROLOGICAL SCIENCES, 1983, 58 (03) :357-370
[9]   A MULTICENTER FOLLOW-UP-STUDY OF 1152 PATIENTS WITH MYASTHENIA-GRAVIS IN ITALY [J].
MANTEGAZZA, R ;
BEGHI, E ;
PAREYSON, D ;
ANTOZZI, C ;
PELUCHETTI, D ;
SGHIRLANZONI, A ;
COSI, V ;
LOMBARDI, M ;
PICCOLO, G ;
TONALI, P ;
EVOLI, A ;
RICCI, E ;
BATOCCHI, AP ;
ANGELINI, C ;
MICAGLIO, GF ;
MARCONI, G ;
TAIUTI, R ;
BERGAMINI, L ;
DURELLI, L ;
CORNELIO, F .
JOURNAL OF NEUROLOGY, 1990, 237 (06) :339-344
[10]   DIAGNOSTIC SENSITIVITY OF THE LABORATORY TESTS IN MYASTHENIA-GRAVIS [J].
OH, SJ ;
KIM, DE ;
KURUOGLU, R ;
BRADLEY, RJ ;
DWYER, D .
MUSCLE & NERVE, 1992, 15 (06) :720-724