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Myasthenia gravis associated with polymyositis (4 cases)
$23
41
MYASTHENIA GRAVIS ASSOCIATED WITH POLYMYDSITIS (4 c a s e s ) . P.~paIek, G.Valach, F.Cibul~ik C11nlca1 C e n t r e f o r NG, B r a t i s l a v a , Slovak republic In Slovak republic, wlth population of 5,3 m i l l i o n , 453 myasthenia gravis (t4[;) patients have been registered to 3an. 1. 1994. Out of them 399 have been a l i v e with c l i n i c a l symptoms or in remlsion - i n d i c a t i n g the prevalence of 75,] per ntt111on. The average aenual incidence rate in prospective study f o r a period of 10 years (3an. 1. 1984 - Dec. 31. 1993) was 6,2 per mi111en. 146 had been aseoelated with polymyositis (PN) in 4 patients. The diagnosis of PH had been conflrmed by diagnostic c r i t e r i a stated by P. Hugdson and 3.P. Peter in 1984. A l l 4 patients f u l f i l l e d the c l i n i c a l , EH[;, laberatory and h l s t e l o g i c a l c r i t e r i a . Case 1: woman, 47, developed acute PH. A f t e r 3 months prednlsone therapy she was in c11nica1 renttsien. 5 years l a t e r she had an onset of f l u c t u a t i n g d i p l o pia, bulbar symptoms and f a t i g u e ~ b i l i t y in extremity moscles. The diagnosis of HG was confirmed. Inmunosuppressive therapy wlth prednisene and azathioprine resulted in cemlsien. Case 2: woman, 59, suffered from acute fulmlnant muscle weaknes, fatlgue and severe pain in muscles. She needed a r t i f i c i a l cesplratien and feedlng by nasog a s t r i c tube. The diagnosis HG and PI4 had been confirmed. High-dose intrsvenous methylprednlsolene pulses ( l g ) every other day were adlninlstersd in comblnatien with azathioprine, prednlsene and plasmapherssis. A f t e r long-term maintenance therapy of 100 mg azathioprlno she i s in remisien. Case 3: man, 52, developed an acute HG with severe PH. Plasmapheresis and immunosuppresslve therapy resulted in marked imprsv~,ent. Case 4: woman, 31, wlth ME; was seccessfuly treated with thymectomy - c11nlcal cemision. 4 years l a t e r she developed subacute form of PH, Nhich was completly cured with prednlsene.
42
MYASTHENIA
GRAVIS
AND
HEPATITIS
C
VIRUS
B. Vernet-der Garabedian, J.M. Pawlotsky$, F.J. Authier R~mir~$, F. Darthuy$, J. Duval$, D. Dhumeaux~ & J.F. Bach.
INFECTION
°, C. PelletS,
J.
INSERM U25, H6pital Necker, Paris, France; $ D~partement de Bact~riologie-Virologie,* Neurologie et ~ H6patho-gastroent6rologie, H6pital H. Mondor, Universit~ PXII, Cr6teil, France. Chronic hepatitis C virus (HCV) infection has been shown to be associated with a number of immunological abnormalities including various autoimmune disorders. Myasthenia gravis (MG) is an autoimmune disease affecting the motor endplate characterized by the presence of autoantibodies directed against the acetylcholine receptor (AChR) in about 90% of cases. The aim of this work was to study wether a relationship could exist between MG and HCV infection. A series of 130 MG sera from 128 patients representative of MG population with typical symptoms of MG and anti-AChR antibody titers ranging from 0 to 1500 nM were tested for the presence of anti-HCV antibodies. Two tests were used for the detection of anti-HCV antibodies: a third generation ELISA and a third generation immunoblot assay (ELISA3 and RIBA3 respectively from Ortho Diagnostic Systems). On the other hand, anti-AChR antibodies were tested in the serum of 60 patients with chronic HCV infection by a radioimmunoprecipitation assay using human AChR labeled with alpha bungarotoxin. None of the 60 patients with chronic hepatitis C had anti-AChR antibodies. Ten out of the 128 patients with MG had anti-HCV antibodies detected by ELISA. Among these 10, 7 were positive in the RIBA3 test and 2 "indeterminate", leading to an overall prevalence of anti-HCV antibodies of 7%. Markers of HCV infection are frequent in patients with MG, since they are found in 7% of them whereas the prevalence of such markers in the French population is about 1%. Two hypothesis may explain this finding: patients with MG could be at increased risk for nosocomial transmission of HCV; HCV could play a role in the occurrence of some cases of MG as reported for mixed cryoglobulinemia or lichen planus.
41
MYASTHENIA GRAVIS ASSOCIATED WITH POLYMYDSITIS (4 c a s e s ) . P.~paIek, G.Valach, F.Cibul~ik C11nlca1 C e n t r e f o r NG, B r a t i s l a v a , Slovak republic In Slovak republic, wlth population of 5,3 m i l l i o n , 453 myasthenia gravis (t4[;) patients have been registered to 3an. 1. 1994. Out of them 399 have been a l i v e with c l i n i c a l symptoms or in remlsion - i n d i c a t i n g the prevalence of 75,] per ntt111on. The average aenual incidence rate in prospective study f o r a period of 10 years (3an. 1. 1984 - Dec. 31. 1993) was 6,2 per mi111en. 146 had been aseoelated with polymyositis (PN) in 4 patients. The diagnosis of PH had been conflrmed by diagnostic c r i t e r i a stated by P. Hugdson and 3.P. Peter in 1984. A l l 4 patients f u l f i l l e d the c l i n i c a l , EH[;, laberatory and h l s t e l o g i c a l c r i t e r i a . Case 1: woman, 47, developed acute PH. A f t e r 3 months prednlsone therapy she was in c11nica1 renttsien. 5 years l a t e r she had an onset of f l u c t u a t i n g d i p l o pia, bulbar symptoms and f a t i g u e ~ b i l i t y in extremity moscles. The diagnosis of HG was confirmed. Inmunosuppressive therapy wlth prednisene and azathioprine resulted in cemlsien. Case 2: woman, 59, suffered from acute fulmlnant muscle weaknes, fatlgue and severe pain in muscles. She needed a r t i f i c i a l cesplratien and feedlng by nasog a s t r i c tube. The diagnosis HG and PI4 had been confirmed. High-dose intrsvenous methylprednlsolene pulses ( l g ) every other day were adlninlstersd in comblnatien with azathioprine, prednlsene and plasmapherssis. A f t e r long-term maintenance therapy of 100 mg azathioprlno she i s in remisien. Case 3: man, 52, developed an acute HG with severe PH. Plasmapheresis and immunosuppresslve therapy resulted in marked imprsv~,ent. Case 4: woman, 31, wlth ME; was seccessfuly treated with thymectomy - c11nlcal cemision. 4 years l a t e r she developed subacute form of PH, Nhich was completly cured with prednlsene.
42
MYASTHENIA
GRAVIS
AND
HEPATITIS
C
VIRUS
B. Vernet-der Garabedian, J.M. Pawlotsky$, F.J. Authier R~mir~$, F. Darthuy$, J. Duval$, D. Dhumeaux~ & J.F. Bach.
INFECTION
°, C. PelletS,
J.
INSERM U25, H6pital Necker, Paris, France; $ D~partement de Bact~riologie-Virologie,* Neurologie et ~ H6patho-gastroent6rologie, H6pital H. Mondor, Universit~ PXII, Cr6teil, France. Chronic hepatitis C virus (HCV) infection has been shown to be associated with a number of immunological abnormalities including various autoimmune disorders. Myasthenia gravis (MG) is an autoimmune disease affecting the motor endplate characterized by the presence of autoantibodies directed against the acetylcholine receptor (AChR) in about 90% of cases. The aim of this work was to study wether a relationship could exist between MG and HCV infection. A series of 130 MG sera from 128 patients representative of MG population with typical symptoms of MG and anti-AChR antibody titers ranging from 0 to 1500 nM were tested for the presence of anti-HCV antibodies. Two tests were used for the detection of anti-HCV antibodies: a third generation ELISA and a third generation immunoblot assay (ELISA3 and RIBA3 respectively from Ortho Diagnostic Systems). On the other hand, anti-AChR antibodies were tested in the serum of 60 patients with chronic HCV infection by a radioimmunoprecipitation assay using human AChR labeled with alpha bungarotoxin. None of the 60 patients with chronic hepatitis C had anti-AChR antibodies. Ten out of the 128 patients with MG had anti-HCV antibodies detected by ELISA. Among these 10, 7 were positive in the RIBA3 test and 2 "indeterminate", leading to an overall prevalence of anti-HCV antibodies of 7%. Markers of HCV infection are frequent in patients with MG, since they are found in 7% of them whereas the prevalence of such markers in the French population is about 1%. Two hypothesis may explain this finding: patients with MG could be at increased risk for nosocomial transmission of HCV; HCV could play a role in the occurrence of some cases of MG as reported for mixed cryoglobulinemia or lichen planus.