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血浆置换和大剂量静脉注射免疫球蛋白治疗艾萨克斯综合征1例的临床评估

Clinical evaluation of plasma exchange and high dose intravenous immunoglobulin in a patient with Isaacs' syndrome.

作者信息

Ishii A, Hayashi A, Ohkoshi N, Oguni E, Maeda M, Ueda Y, Ishii K, Arasaki K, Mizusawa H, Shoji S

机构信息

Department of Neurology, University of Tsukuba, Japan.

出版信息

J Neurol Neurosurg Psychiatry. 1994 Jul;57(7):840-2. doi: 10.1136/jnnp.57.7.840.

Abstract

The clinical effects of plasma exchange and high dose intravenous immunoglobulin (IVIg) were evaluated in a 41 year old woman with Isaacs' syndrome. After double filtration plasma exchange, symptoms almost disappeared for two to three weeks and the recorded continuous muscle action potentials were considerably decreased. Symptoms recurred within a few months. On the other hand, IVIg worsened the symptoms of the disorder: during and after IVIg at a dose of 0.2 g/kg/day (total 50 g), widespread myokymia, pseudomyotonia, and muscle cramps gradually increased. Symptoms improved after another course of plasma exchange.

摘要

对一名患有艾萨克斯综合征的41岁女性评估了血浆置换和大剂量静脉注射免疫球蛋白(IVIg)的临床效果。双重滤过血浆置换后,症状几乎消失了两到三周,记录的持续性肌肉动作电位显著降低。症状在几个月内复发。另一方面,IVIg使该疾病的症状恶化:在以0.2 g/kg/天(共50 g)的剂量使用IVIg期间及之后,广泛的肌束震颤、假肌强直和肌肉痉挛逐渐加重。在另一疗程的血浆置换后症状有所改善。

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