傍腫瘍性神経症候群とアフェレシス(<特集>免疫性神経疾患とアフェレシス,新しい展望)

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Plasma exchange (PE) may be a useful adjunct to management of patients with the Lambert-Eaton myasthenic syndrome (LEMS) although it cannot be considered standard therapy for the other paraneoplastic neurologic syndrome at present. LEMS is an autoimmune disorder of neuromuscular junctions in which IgG autoantibodies lead to presynaptic loss in the P/Q-type voltage-gated calcium channel (VGCC), resulting in defective neuromuscular transmission. Clinically, LEMS is characterised by proximal muscle weakness initially affecting gait, autonomic symptoms (dry mouth, etc), augmentation of strength during initial voluntary activation, and depressed tendon reflexes with post-tetanic potentiation. The disorder is paraneoplastic (small celllung cancer) in about 60% of cases. PE with immunosuppressive drug therapy has been used to treat severely affected patients. PE appears to be effective and shorten the duration of the myasthenic crisis in LEMS patients. Clinical improvement induced by PE is thought to be a result of the accumulation of newly synthesized P/Q-VGCC due to a decrease in the rate of antibody-mediated P/Q-type VGCC loss. From our antibody data, the titre of anti-P/Q-type VGCC antibody decreased after plasmapheresis, but increased to the pretreatment levels 7 days after the last plasmapheresis without immunosuppressive drug therapy. After adding the immunosuppressive drug, the patient's clinical state and CMAP 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 autoantibody for the treatment of LEMS. Finally, considering our experiences and the literature, we discuss the indication of apheresis in paraneoulastic cerebellar degeneration and stiff-person syndrome.
日本アフェレシス学会の論文
2004-10-31

傍腫瘍性神経症候群とアフェレシス(<特集>免疫性神経疾患とアフェレシス,新しい展望)

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