From the Department of Neurology (S.-U.L., J.-Y.C., J.-S.K.), Seoul National University College of Medicine, Seoul, South Korea; Dizziness Center (S.-U.L., J.-Y.C., J.-S.K.), Clinical Neuroscience Center, and Research Administration Team (H.-J.K.), Seoul National University Bundang Hospital, Seongnam, South Korea.
Neurology. 2018 Jun 12;90(24):e2135-e2145. doi: 10.1212/WNL.0000000000005689. Epub 2018 May 23.
To define a disorder characterized by recurrent spontaneous vertigo (RSV) of unknown etiology and interictal headshaking nystagmus (HSN).
We characterized HSN in 35 patients with RSV-HSN compared to that recorded in randomly selected patients with compensated vestibular neuritis (VN), vestibular migraine (VM), and Ménière disease (MD).
The estimated time constant (TC) of the primary phase of HSN was 12 seconds (95% confidence interval [CI] 12-13) in patients with RSV-HSN, which was larger than those in patients with VN (5 seconds, 95% CI 4-5), VM (5 seconds, 95% CI 5-6), or MD (6 seconds, 95% CI 5-6). TCs of the horizontal vestibulo-ocular reflex were also larger during the rotatory chair test in patients with RSV-HSN. Among the 35 patients with RSV-HSN, 7 showed vigorous long-lasting HSN with a peak slow-phase velocity >50.0°/s. In 5 patients (5 of 7, 71%) with vigorous HSN, HSN could have been induced even with headshaking for only 2 to 5 seconds. Long-term prognosis was favorable, with a resolution or improvement of the symptoms in more than half of the patients during the median follow-up of 12 (range 2-58) years from symptom onset. None developed VM, MD, or cerebellar dysfunction during the follow-up.
The clinical features and characteristics of HSN in our patients indicate a hyperactive and asymmetric velocity-storage mechanism that gives rise to intermittent attacks of spontaneous vertigo probably when marginal compensation of underlying pathology is disrupted by endogenous or exogenous factors.
定义一种以反复发作的自发性眩晕（RSV）为特征的疾病，其病因不明，发作间期伴有摇头性眼震（HSN）。
我们对 35 例 RSV-HSN 患者的 HSN 特征进行了描述，并与随机选择的前庭神经炎（VN）、前庭性偏头痛（VM）和梅尼埃病（MD）患者的记录进行了比较。
RSV-HSN 患者的 HSN 主要相的估计时间常数（TC）为 12 秒（95%置信区间 [CI] 12-13），大于 VN 患者（5 秒，95%CI 4-5）、VM 患者（5 秒，95%CI 5-6）或 MD 患者（6 秒，95%CI 5-6）。在旋转椅试验中，RSV-HSN 患者的水平前庭眼反射的 TC 也较大。在 35 例 RSV-HSN 患者中，7 例表现出剧烈的、持久的 HSN，其峰值慢相速度＞50.0°/s。在 5 例（71%）有剧烈 HSN 的患者中，即使仅摇头 2 至 5 秒，也可能诱发 HSN。长期预后良好，超过一半的患者在症状出现后的中位随访 12 年（范围 2-58 年）期间症状得到缓解或改善。在随访期间，无患者出现 VM、MD 或小脑功能障碍。
我们患者的 HSN 临床特征和特征表明存在一种过度活跃和不对称的速度储存机制，当潜在病理的边缘代偿因内源性或外源性因素而被破坏时，可能会导致间歇性自发性眩晕发作。
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