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与具有末端葡糖醛酸基-3-硫酸表位的糖脂单克隆IgM抗体相关的周围神经病。

Peripheral neuropathy associated with monoclonal IgM antibody to glycolipids with a terminal glucuronyl-3-sulfate epitope.

作者信息

Fredman P, Lycke J, Andersen O, Vrethem M, Ernerudh J, Svennerholm L

机构信息

Department of Psychiatry and Neurochemistry, University of Göteborg, Mölndal Hospital, Sweden.

出版信息

J Neurol. 1993 Jun;240(6):381-7. doi: 10.1007/BF00839972.

Abstract

Twenty-nine patients with paraproteinaemia, 12 with neuropathy and 17 without a previous record of neurological symptoms were clinically characterized. All 12 neuropathy patients had a moderate to severe sensorimotor demyelinating neuropathy. The patients were examined with regard to serum antibodies to gangliosides, including GM1, GD1a, GD1b, GT1b, and LM1, and other acidic glycolipids, including LK1 and sulphatide, of human brain and peripheral nerve. Sera from 80 blood donors, 40 men and 40 women 20-60 years of age, were used as normal controls. The sera were analysed with an ELISA performed on thin-layer chromatography plates. At a dilution of 1/400 none of the control sera gave a detectable reaction and a titre of > or = 1:400 was considered as a positive test. In 11 of the 12 neuropathy patients the paraproteinaemia was of IgM type and 10 of them had a positive antibody titre against LK1 and Hex-LK1, acidic glycolipids with a terminal glucuronyl-3-sulphate group. The antibody titre against LK1 in 1 patient was 1:400 and varied between 1:5,000 and 1:3,200,000 in the other 9. One of the patients also had a positive titre, 1:64,000, to sulphatide. None of the sera from the 17 paraproteinaemia patients without a previous record of neurological symptoms contained antibodies to LK1 or to any glycolipid antigen examined, except for sulphatide. A positive titre (> or = 1:400) of antibodies to sulphatide was found in sera from 4 of these patients, the titres being < or = 3,200.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

对29例副蛋白血症患者进行了临床特征分析,其中12例有神经病变,17例既往无神经症状记录。12例神经病变患者均有中度至重度感觉运动性脱髓鞘神经病变。对患者检测了针对神经节苷脂(包括GM1、GD1a、GD1b、GT1b和LM1)以及人脑和周围神经的其他酸性糖脂(包括LK1和硫脂)的血清抗体。选取80名年龄在20至60岁之间的献血者(40名男性和40名女性)的血清作为正常对照。血清在薄层色谱板上通过酶联免疫吸附测定(ELISA)进行分析。在1/400的稀释度下,对照血清均未产生可检测到的反应,滴度≥1:400被视为阳性检测结果。12例神经病变患者中有11例副蛋白血症为IgM型,其中10例针对LK1和Hex-LK1(具有末端葡萄糖醛酸-3-硫酸基团的酸性糖脂)的抗体滴度呈阳性。1例患者针对LK1的抗体滴度为1:400,其他9例患者的滴度在1:5000至1:3200000之间变化。其中1例患者针对硫脂的滴度也呈阳性,为1:64000。17例既往无神经症状记录的副蛋白血症患者的血清中,除硫脂外,均未检测到针对LK1或任何检测的糖脂抗原的抗体。这些患者中有4例血清中针对硫脂的抗体滴度呈阳性(≥1:400),滴度≤3200。(摘要截取自250字)

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