Lambert-Eaton筋力症候群とアフェレシス

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We studied the efficacy of plasmapheresis, analyzing the clinical score, electrophysiological finding, and the titre of anti-P/Q-type VGCC antibody. Two patients with Lambert-Eaton myasthenic syndrome (LEMS) were treated with plasmapheresis, high-dose gammaglobulin, and immunosuppressive drug therapy. The first case was a 72-year-o1d woman suffering from muscle weakness of the lower extremities. She first underwent 4 plasma exchanges and subsequently showed a transient improvement of gait disturbance. She was then treated with high-dose gammaglobulin after undergoing another 3 plasma exchages. As a result, a marked alleviation of the gait disturbance and an increase in the amplitude of compound muscle action potential (CMAP) were obtained. Antibody titres are inversely related to clinical score and CMAP amplitude. The second case was a 73-year-old man who developed gait disturbance and blepharoptosis. He was treated with 1 plasma exchange, 3 double filtration plasmapheleses, and immunosuppressive drug (prednisolone and azathiopurine). The titre of anti-P/Q-type VGCC antibody decreased after plasmaphereses, but increased to the pretreatment levels 7 days after the last plasmapheresis. After adding the immunosuppressive drugs, his clinical state and CAMP improved markedly and autoantibody titres decreased. These results suggest that it is important not only to remove the autoantibodies but also to suppress the synthesis of autoantibodies for the treatment of LEMS. Considering our experiences and other literature, we discuss the indication of apheresis treatment for LEMS.
日本アフェレシス学会の論文
1999-06-30

Lambert-Eaton筋力症候群とアフェレシス

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