多发性硬化作为急性眩晕一个未被充分认识的原因：临床和病理生理学见解

文献信息

中文摘要

多发性硬化（MS）是一种自身免疫性疾病，在复发缓解阶段常表现为急性神经系统症状。虽然运动、感觉和视觉缺陷是公认的复发表现，但前庭症状如急性眩晕、头晕和不平衡相对未被充分认识，且常带来诊断挑战。本综述综合了关于MS相关急性前庭综合征及其中枢病理生理机制的基础和近期证据。幕下脱髓鞘斑块，最常位于脑桥和小脑中脚，破坏大纤维系统并产生独特的中枢眼动体征。我们强调高度特征性的眼动特征——包括核间性眼肌麻痹、双眼外斜性核间性眼肌麻痹和获得性钟摆性眼震——这些是关键的定位诊断线索。此外，我们讨论临床模拟病症，将中枢性位置性眼震和眩晕与良性阵发性位置性眩晕区分开来，后者在MS人群中高度流行且可通过床旁手法治疗。至关重要的是，本文探讨了床旁工具如HINTS检查的局限性，这些工具能识别中枢病理，但无法区分缺血性卒中和急性脱髓鞘。在急诊环境中，将先进神经影像学——如灌注MRI、中央静脉征和顺磁性边缘病变——与详细眼动评估相结合至关重要。最后，我们探讨模拟周围性前庭病的非典型束性病变，并假设MS中延迟、失同步的轴突传导可能损害前庭恢复所需的隐性扫视的精确计时，值得未来队列研究。

英文摘要

Multiple sclerosis (MS) is an autoimmune disorder that frequently presents with acute neurological symptoms during relapsing-remitting phases. While motor, sensory, and visual deficits are widely recognized manifestations of relapses, vestibular symptoms such as acute vertigo, dizziness, and imbalance are comparatively under-recognized and often pose diagnostic challenges. This review synthesizes foundational and recent evidence regarding MS-related acute vestibular syndrome and its central pathophysiologic mechanisms. Infratentorial demyelinating plaques, most frequently localized within the pons and the middle cerebellar peduncles, disrupt large fiber systems and give rise to distinct central oculomotor signs. We highlight highly characteristic ocular motor features-including internuclear ophthalmoplegia, wall-eyed bilateral INO, and acquired pendular nystagmus-that serve as crucial topodiagnostic clues. Furthermore, we address clinical mimics, distinguishing central positional nystagmus and vertigo from benign paroxysmal positional vertigo, the latter being highly prevalent and treatable via bedside maneuvers in MS populations. Crucially, this paper examines the limitations of bedside tools like the HINTS exam, which identify central pathology but fail to differentiate ischemic stroke from acute demyelination. In the emergency setting, integrating advanced neuroimaging-such as perfusion MRI, the central vein sign, and paramagnetic rim lesions-with detailed ocular motor evaluation is vital. Lastly, we explore atypical fascicular lesions mimicking peripheral vestibulopathy and hypothesize how delayed, desynchronized axonal conduction in MS may impair the precise timing of covert saccades required for vestibular recovery, warranting future cohort investigations.