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抗肌肉特异性激酶(MuSK)阳性、抗乙酰胆碱受体(AChR)阳性及血清阴性重症肌无力的临床比较

Clinical comparison of anti-MuSK- vs anti-AChR-positive and seronegative myasthenia gravis.

作者信息

Deymeer F, Gungor-Tuncer O, Yilmaz V, Parman Y, Serdaroglu P, Ozdemir C, Vincent A, Saruhan-Direskeneli G

机构信息

Department of Neurology, Istanbul University, Istanbul Medical Faculty, Capa, 34390 Istanbul, Turkey.

出版信息

Neurology. 2007 Feb 20;68(8):609-11. doi: 10.1212/01.wnl.0000254620.45529.97.

DOI:10.1212/01.wnl.0000254620.45529.97
PMID:17310034
Abstract

We compared 65 anti-acetylcholine receptor (AChR)-negative myasthenia gravis (MG) patients, including 32 anti-muscle-specific tyrosine kinase (MuSK)-positive (49%) and 33 anti-MuSK-negative (seronegative) (51%) patients, with 161 anti-AChR-positive MG patients. The anti-MuSK-positive group had a higher frequency of bulbar involvement and respiratory crises. The seronegative group was in between the anti-MuSK positive and the anti-AChR positive groups, being closer to the latter, with regard to the severity of the disease. At the end of follow-up, the outcome of the anti-MuSK-positive patients was not different from that of the anti-AChR-positive patients, although their maintenance corticosteroid dose was higher. The seronegative patients had better outcome than the other two groups.

摘要

我们将65例抗乙酰胆碱受体(AChR)阴性的重症肌无力(MG)患者与161例抗AChR阳性的MG患者进行了比较,其中抗AChR阴性的MG患者包括32例抗肌肉特异性酪氨酸激酶(MuSK)阳性(49%)和33例抗MuSK阴性(血清阴性)(51%)患者。抗MuSK阳性组延髓受累和呼吸危象的发生率更高。血清阴性组在疾病严重程度方面介于抗MuSK阳性组和抗AChR阳性组之间,且更接近后者。随访结束时,抗MuSK阳性患者的结局与抗AChR阳性患者并无差异,尽管他们维持使用皮质类固醇的剂量更高。血清阴性患者的结局优于其他两组。

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