Wirth Magdalena A, Fierz Fabienne C, Valko Yulia, Weber Konrad P
Department of Ophthalmology, University Hospital Zurich, University of Zurich, Zurich, Switzerland.
Department of Ophthalmology, University of British Columbia, Vancouver, BC, Canada.
Front Neurol. 2020 Aug 13;11:861. doi: 10.3389/fneur.2020.00861. eCollection 2020.
Timely and accurate diagnosis of myasthenia gravis, particularly in patients with fluctuating, isolated ocular involvement, remains challenging. Serological antibody testing and repetitive nerve stimulation of peripheral muscles usually have low sensitivity in these patients. Edrophonium testing may cause adverse events, single-fiber electromyography (SFEMG) is time-consuming and both tests are often unavailable outside specialized institutions. Repetitive ocular vestibular evoked myogenic potential (roVEMP) stimulation has recently been introduced to facilitate the diagnosis of myasthenia gravis. Similar to repetitive nerve stimulation, roVEMPs detect muscle decrements with the benefit of being non-invasive and allowing for direct measurement of the extraocular muscles. This review summarizes the clinical evidence of the diagnostic value of roVEMP for myasthenia. Prospective clinical trials have demonstrated high sensitivity and specificity. RoVEMPs are of particular interest in challenging myasthenia subgroups with isolated ocular involvement, negative serology, and/or negative conventional electrophysiological results. Optimal roVEMP repetition rates of 20-30 Hz have been identified. This promising novel diagnostic tool merits further attention and investigation to establish its value as a clinical test for myasthenia.
重症肌无力的及时准确诊断,尤其是对于病情波动、仅有眼部受累的患者而言,仍然具有挑战性。血清学抗体检测和外周肌肉重复神经电刺激在这些患者中通常敏感性较低。依酚氯铵试验可能会引起不良事件,单纤维肌电图(SFEMG)耗时较长,并且这两种检测在专业机构以外的地方通常无法进行。重复性眼前庭诱发肌源性电位(roVEMP)刺激最近被引入以辅助重症肌无力的诊断。与重复神经电刺激类似,roVEMP可检测肌肉递减情况,其优势在于无创且能够直接测量眼外肌。本综述总结了roVEMP对重症肌无力诊断价值的临床证据。前瞻性临床试验已证明其具有高敏感性和特异性。roVEMP对于具有孤立性眼部受累、血清学阴性和/或传统电生理结果阴性的重症肌无力亚组尤其有意义。已确定20 - 30Hz的最佳roVEMP重复率。这种有前景的新型诊断工具值得进一步关注和研究,以确立其作为重症肌无力临床检测方法的价值。