Department of Neurology, Leiden University Medical Center, K5Q-114, P.O. Box 9600, 2300 RC Leiden, The Netherlands.
Muscle Nerve. 2010 Aug;42(2):283-8. doi: 10.1002/mus.21642.
Autoantibodies to muscle-specific kinase (MuSK) can cause myasthenia gravis (MG). The pathophysiological mechanism remains unknown. We report in vitro electrophysiological and histological studies of the neuromuscular junction in a MuSK MG patient. Low levels of presynaptic acetylcholine release and small miniature endplate potentials were found. This combination of pre- and postsynaptic abnormalities was supported by histology, revealing partially denervated postsynaptic areas, and some degeneration of postsynaptic folds. Results suggest that anti-MuSK antibodies reduce the stability of muscle-nerve contact.
抗肌肉特异性激酶（MuSK）自身抗体可引起重症肌无力（MG）。其病理生理机制尚不清楚。我们报告了 MuSK-MG 患者神经肌肉接点的体外电生理学和组织学研究。发现突触前乙酰胆碱释放水平较低，微小终板电位较小。组织学结果支持这种突触前和突触后异常的组合，显示部分去神经后的突触后区域和一些突触后褶皱的退化。结果表明，抗 MuSK 抗体降低了肌肉-神经接触的稳定性。
