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Effect of thymectomy after 40 years of age in myasthenia gravis a retrospective nordic multicentre study
THERAPY 95
EFFECT OF THYMECTOMY AFTER 40 YEARS OF AGE IN MYASTHENIA GRAVIS A RETROSPECTIVE NORDIC MULTICENTRE STUDY.
G. Matell(1), S. Bjelak(1), I. Bj~msen(2), P. Br0gger Christensen(3), M. F~irkldl~l(6), H. Hofstad(4), C. Lindberg(5), K. M011er(6), P.O. Osterman(7), R. Pirskanen(1), F.E. Somnier(8), I. Tsidopouius(9). Departments of Neurology at 1) S0dersjukhuset Stockholm(S) 2) Rikshospitalet Oslo(N), 3) Aarhus Kommunehospital(DK), 4) Haukeland Hospital, Bergen(N), 5) Sahlgrenska sjukhuset G0teborg(S), 6) University Hospital Helsinld(SF), 7) Uppsala Akademiska Sjukhus(S), 8) Rigshospitalet Copenhagen(DK), 9) Odense University Hospital(Dk). Purpose: Thymectomy (TX) is widely accepted for treatment of generalized MG below the age of 40 resulting in improvement of 70-90 % of patients without thymoma, we have made TX after the age of 40, we have made an attempt to evaluate TX in late onset MG. Material and methods: Non-thymoma MG patients from 8 nordic centra (cf author list) with MG onset after 35 years of age (total 559) were reviewed. Selection criteda:TX after 40 years of age, follow up available 2 and/or 5 years postoperatively (n=216) were compared with nonoperated (CTR, n=156). A total of 187 pat were excluded( 64 TX, 67 CTR, 25 deaths, unknown 31). MG status (ocular and general) scores, anticholinesterase dosage, immunosuppression modalities 2 and 5 years after TX (equivalent age for CTR) were recorded. Results: TX patients were operated on at an average age of 57 (40-80) years. At 5 year follow-
up 68% were improved, 26% remained unchanged and 6% had worsened. Of 22 deaths in TX patients, 3 were due to MG or its treatment. In CTR patients 42% improved, 41% remained unchanged and 18 % worsened.Seven of 30 CTR deaths were ascribed to MG or its treatment. Conclusions: TX seems to have a therapeutic effect after 40 years of age. Some of the improvement may have been achieved by immunosuppression given. Thus, thymectomy in MG patients older than 40 years may be considered.
96
FIRST INTERIM ANALYSIS OF A RANDOMISED CLINICAL TRIAL COMPARING PLASMA EXCHANGE AND INTRAVENOUS I M M U N O G L O B U L I N IN M Y A S T H E N I A G R A V I S . Ph. GAJDOS*, CI. CHASTANG**, B CLAIR*, Ch. TRANCHANT+, F. BOLGERT++, B. EYMARD++ for the Myasthenia Gravis clinical Study group. H6pital Raymond Poincar6*, Garches. H6pital Saint Louis**, Paris. Hospices Civils Strasbourg +. H6pital de la Salp6tri~re++. Paris. France. Plasma exchange (PE) is a reference treatment of acute exacerbations of Myasthenia Gravis (MG). Intravenous immunoglobulin (IVIG) has been proposed as an alternative but the efficacy of W I G has not been established. The goals of this study are 1) to compare the outcome of PE and W I G , 2) to establish the dose effect relationship of two dosages of WIG. Method : Patients were included if they present an acute exacerbation of MG. They were randomised to receive either 1) 3 PE every 2 days, 2) IVIG 0,4 g/kg/d for 5 days or 3 days. The endpoints were the difference in the muscular score between the 1st and the 15th day (delta score) and side effects. Results : 66 patients had been included = 30 received PE and 36 IVIG (17 for 5 days and 19 for 3 days). Patients were well-balanced for main characteristics of MG at the time of randomisation. Considering the number of patients included in IVIG arms this interim analysis only compares the results of PE versus W I G considered as one gl oup. Evolution of delta score is given below : D2 D4 D6 D9 D12 D15 Delta PE 2.6 7.2 10.7 14.9 15.1 13.1 Score n--30 + 8.9 + 10.9 -+ 13.3 + 13.5 + 13.0 + 15.3 m IVIG 2.9 8.1 12.8 13.5 15.8 14.2 + SD n=36 + 7.7 + 11.9 + 12.6 + 13.8 + 14.1 _+ 13.0 0.98 0.89 0.70 0.56 0.94 0.58 * Kruskal Wallis test Five side effects (2 hematoma, 1 hemolysis, 2 chills-fever) were observed in the PE group versus none in the W I G group. Conclusion : At this time, despite the power, IVIG appears as effective as PE. Patient accrual will continue. $51
EFFECT OF THYMECTOMY AFTER 40 YEARS OF AGE IN MYASTHENIA GRAVIS A RETROSPECTIVE NORDIC MULTICENTRE STUDY.
G. Matell(1), S. Bjelak(1), I. Bj~msen(2), P. Br0gger Christensen(3), M. F~irkldl~l(6), H. Hofstad(4), C. Lindberg(5), K. M011er(6), P.O. Osterman(7), R. Pirskanen(1), F.E. Somnier(8), I. Tsidopouius(9). Departments of Neurology at 1) S0dersjukhuset Stockholm(S) 2) Rikshospitalet Oslo(N), 3) Aarhus Kommunehospital(DK), 4) Haukeland Hospital, Bergen(N), 5) Sahlgrenska sjukhuset G0teborg(S), 6) University Hospital Helsinld(SF), 7) Uppsala Akademiska Sjukhus(S), 8) Rigshospitalet Copenhagen(DK), 9) Odense University Hospital(Dk). Purpose: Thymectomy (TX) is widely accepted for treatment of generalized MG below the age of 40 resulting in improvement of 70-90 % of patients without thymoma, we have made TX after the age of 40, we have made an attempt to evaluate TX in late onset MG. Material and methods: Non-thymoma MG patients from 8 nordic centra (cf author list) with MG onset after 35 years of age (total 559) were reviewed. Selection criteda:TX after 40 years of age, follow up available 2 and/or 5 years postoperatively (n=216) were compared with nonoperated (CTR, n=156). A total of 187 pat were excluded( 64 TX, 67 CTR, 25 deaths, unknown 31). MG status (ocular and general) scores, anticholinesterase dosage, immunosuppression modalities 2 and 5 years after TX (equivalent age for CTR) were recorded. Results: TX patients were operated on at an average age of 57 (40-80) years. At 5 year follow-
up 68% were improved, 26% remained unchanged and 6% had worsened. Of 22 deaths in TX patients, 3 were due to MG or its treatment. In CTR patients 42% improved, 41% remained unchanged and 18 % worsened.Seven of 30 CTR deaths were ascribed to MG or its treatment. Conclusions: TX seems to have a therapeutic effect after 40 years of age. Some of the improvement may have been achieved by immunosuppression given. Thus, thymectomy in MG patients older than 40 years may be considered.
96
FIRST INTERIM ANALYSIS OF A RANDOMISED CLINICAL TRIAL COMPARING PLASMA EXCHANGE AND INTRAVENOUS I M M U N O G L O B U L I N IN M Y A S T H E N I A G R A V I S . Ph. GAJDOS*, CI. CHASTANG**, B CLAIR*, Ch. TRANCHANT+, F. BOLGERT++, B. EYMARD++ for the Myasthenia Gravis clinical Study group. H6pital Raymond Poincar6*, Garches. H6pital Saint Louis**, Paris. Hospices Civils Strasbourg +. H6pital de la Salp6tri~re++. Paris. France. Plasma exchange (PE) is a reference treatment of acute exacerbations of Myasthenia Gravis (MG). Intravenous immunoglobulin (IVIG) has been proposed as an alternative but the efficacy of W I G has not been established. The goals of this study are 1) to compare the outcome of PE and W I G , 2) to establish the dose effect relationship of two dosages of WIG. Method : Patients were included if they present an acute exacerbation of MG. They were randomised to receive either 1) 3 PE every 2 days, 2) IVIG 0,4 g/kg/d for 5 days or 3 days. The endpoints were the difference in the muscular score between the 1st and the 15th day (delta score) and side effects. Results : 66 patients had been included = 30 received PE and 36 IVIG (17 for 5 days and 19 for 3 days). Patients were well-balanced for main characteristics of MG at the time of randomisation. Considering the number of patients included in IVIG arms this interim analysis only compares the results of PE versus W I G considered as one gl oup. Evolution of delta score is given below : D2 D4 D6 D9 D12 D15 Delta PE 2.6 7.2 10.7 14.9 15.1 13.1 Score n--30 + 8.9 + 10.9 -+ 13.3 + 13.5 + 13.0 + 15.3 m IVIG 2.9 8.1 12.8 13.5 15.8 14.2 + SD n=36 + 7.7 + 11.9 + 12.6 + 13.8 + 14.1 _+ 13.0 0.98 0.89 0.70 0.56 0.94 0.58 * Kruskal Wallis test Five side effects (2 hematoma, 1 hemolysis, 2 chills-fever) were observed in the PE group versus none in the W I G group. Conclusion : At this time, despite the power, IVIG appears as effective as PE. Patient accrual will continue. $51