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静脉注射免疫球蛋白在副肿瘤性神经系统疾病治疗中的应用

Intravenous immunoglobulins in the therapy of paraneoplastic neurological disorders.

作者信息

Blaes F, Strittmatter M, Merkelbach S, Jost V, Klotz M, Schimrigk K, Hamann G F

机构信息

Department of Neurology, University of the Saarland, Homburg, Germany.

出版信息

J Neurol. 1999 Apr;246(4):299-303. doi: 10.1007/s004150050350.

Abstract

The treatment of paraneoplastic neurological syndromes (e.g., tumor therapy, immunosuppressive therapy, plasmapheresis) rarely leads to an improvement in the neurological symptoms. We treated four patients suffering from paraneoplastic neurological syndromes with intravenous immunoglobulins. All four had high titers of antineuronal antibodies in serum and CSF. Two of the patients, one suffering from paraneoplastic cerebellar degeneration and the other from paraneoplastic brain stem encephalitis and polyneuropathy, received intravenous immunoglobulin treatment within 3 weeks of the onset of neurological symptoms. Both patients showed clinical improvement within 2 weeks after the initiation of therapy. They also showed a decline in the intrathecal antibody synthesis of the antineuronal antibody. Two other patients, who had suffered from paraneoplastic neuropathy for 3 and 6 months showed no improvement with the intravenous immunoglobulin therapy. In these cases there was no effect on intrathecal antibody synthesis. When started early, intravenous immunoglobulins may be of therapeutical value in treating paraneoplastic neurological syndromes. Specific intrathecal antibody synthesis may be a better measure of clinical course that autoantibody serum titers.

摘要

副肿瘤性神经系统综合征的治疗(如肿瘤治疗、免疫抑制治疗、血浆置换)很少能使神经症状得到改善。我们用静脉注射免疫球蛋白治疗了4例副肿瘤性神经系统综合征患者。所有4例患者血清和脑脊液中的抗神经元抗体滴度均很高。其中2例患者,1例患有副肿瘤性小脑变性,另1例患有副肿瘤性脑干脑炎和多发性神经病,在神经症状出现后3周内接受了静脉注射免疫球蛋白治疗。这2例患者在开始治疗后2周内临床症状均有改善。他们鞘内抗神经元抗体的合成也有所下降。另外2例患有副肿瘤性神经病3个月和6个月的患者,静脉注射免疫球蛋白治疗后没有改善。在这些病例中,对鞘内抗体合成没有影响。如果早期开始使用,静脉注射免疫球蛋白在治疗副肿瘤性神经系统综合征方面可能具有治疗价值。特定的鞘内抗体合成可能比自身抗体血清滴度更能衡量临床病程。

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