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以孤立性颈伸肌无力为表现的抗穆斯科林抗体阳性重症肌无力

Musk-antibody positive myasthenia gravis presenting with isolated neck extensor weakness.

作者信息

Casasnovas C, Povedano M, Jaumà S, Montero J, Martínez-Matos J A

机构信息

Neuromuscular Unit, Department of Neurology, Hospital Universitari de Bellvitge-IDIBELL, Feixa Llarga S/N 08907, L'Hospitalet de Llobregat, Barcelona, Spain.

出版信息

Neuromuscul Disord. 2007 Jul;17(7):544-6. doi: 10.1016/j.nmd.2007.03.007. Epub 2007 May 29.

Abstract

Dropped head sign is characterized by the gradual forward sagging of the head due to weakness of neck extensor muscles. This may be a prominent sign of several neuromuscular disorders and may be an isolated feature of myasthenia gravis (MG). We describe a patient with isolated neck extensor weakness, eletrophysiological findings suggesting myasthenia gravis and positive MuSK antibodies. This case supports that finding anti-MuSK antibodies may be extremely helpful in dropped head patients and negative acetylcholine receptor antibodies especially if needle EMG does not reveal myopathic or neurogenic patterns.

摘要

低头征的特点是由于颈部伸肌肌无力导致头部逐渐向前下垂。这可能是几种神经肌肉疾病的显著体征,也可能是重症肌无力(MG)的孤立特征。我们描述了一名患有孤立性颈部伸肌无力的患者,其电生理检查结果提示重症肌无力且抗肌肉特异性激酶(MuSK)抗体呈阳性。该病例支持这样的观点,即对于低头征患者,尤其是在针极肌电图未显示肌病或神经源性模式时,检测抗MuSK抗体可能非常有帮助,而乙酰胆碱受体抗体阴性。

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