Suppr超能文献

以垂头综合征为突出临床特征的伴有胸腺增生的肌肉特异性激酶(MuSK)阳性重症肌无力。

Dropped head syndrome as prominent clinical feature in MuSK-positive Myasthenia Gravis with thymus hyperplasia.

作者信息

Spengos Konstantinos, Vassilopoulou Sofia, Papadimas Georgios, Tsivgoulis Georgios, Karandreas Nikos, Zambelis Thomas, Manta Panagiota

机构信息

Department of Neurology, Eginition Hospital, University of Athens School of Medicine, Vas. Sofias 74, 11528 Athens, Greece.

出版信息

Neuromuscul Disord. 2008 Feb;18(2):175-7. doi: 10.1016/j.nmd.2007.09.007. Epub 2007 Nov 28.

Abstract

MuSK-positive Myasthenia Gravis is in most cases clinically characterized by a progressive course with severe oculobulbar involvement or prominent neck, shoulder and respiratory muscle weakness. It is also distinguished from other forms of myastehnia through its lack of germinal centers or lymphocytic infiltrates in the thymic tissue. We present the case of a MuSK-positive female myasthenic patient with over four years slowly progressive weakness of the neck extensor muscles in the presence of thymus hyperplasia and discuss its uncommon and markedly focal clinical and electrophysiological features, as well as the excellent course under medication with pyridostigmine and prednisone, especially after thymectomy.

摘要

在大多数情况下,MuSK 阳性重症肌无力的临床特征是病情呈进行性发展,伴有严重的眼球运动和延髓肌受累,或颈部、肩部及呼吸肌无力明显。它还因其胸腺组织中缺乏生发中心或淋巴细胞浸润而有别于其他形式的重症肌无力。我们报告一例 MuSK 阳性女性重症肌无力患者,该患者在胸腺增生的情况下,颈部伸肌缓慢进行性无力超过四年,并讨论其罕见且明显局灶性的临床和电生理特征,以及使用吡啶斯的明和泼尼松治疗后的良好病程,特别是胸腺切除术后的情况。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验