From the Department of Neurology (S.-U.K.), Korea University Medical Center, Seoul; Department of Neurology (J.-Y.C., J.-S.K.), Seoul National University College of Medicine; Research Administration Team (H.-J.K.), Dizziness Center (J.-Y.C., J.-S.K.), Clinical Neuroscience Center, and Department of Neurology (J.-Y.C., J.-S.K.), Seoul National University Bundang Hospital, Seongnam; and Department of Neurology (J.K.K.), Dong-A University College of Medicine, Busan, South Korea.
Neurology. 2019 Sep 10;93(11):e1085-e1092. doi: 10.1212/WNL.0000000000008107. Epub 2019 Aug 9.
To delineate the clinical features and ocular motor findings in acute vestibular syndrome (AVS) associated with anti-GQ1b antibodies.
We reviewed 90 patients with positive serum anti-GQ1b antibody in association with various neurological syndromes at Seoul National University Bundang Hospital from 2004 to 2018. The diagnoses included typical Miller Fisher syndrome (n = 31), acute ophthalmoplegia without ataxia (n = 27), Guillain-Barre syndrome with ophthalmoplegia (n = 18), AVS (n = 11), and Bickerstaff brainstem encephalitis (n = 3). Of them, the 11 patients with AVS formed the basis of this study. We also conducted a systematic review on AVS reported in association with anti-GQ1b antibody.
Patients with AVS showed various ocular motor findings that included head-shaking nystagmus (n = 6), spontaneous nystagmus (n = 5), gaze-evoked nystagmus (n = 5), central positional nystagmus (n = 3), canal paresis (n = 2), and abnormal head-impulse tests (n = 1) without any internal or external ophthalmoplegia. Compared to those with other subtypes, patients with AVS mostly showed normal deep tendon reflexes (8 of 11 [73%], = 0.002) and normal results on nerve conduction studies (4 of 4 [100%], = 0.010). The clinical and laboratory findings resolved or improved markedly in all patients within 6 months of symptom onset. Systematic review further identified 7 patients with AVS and positive serum anti-GQ1b antibody who showed various ocular motor findings, including gaze-evoked nystagmus, saccadic dysmetria, central positional nystagmus, and ocular flutter or opsoclonus.
Anti-GQ1b antibody may cause acute vestibulopathy by involving either the central or peripheral vestibular structures. AVS may constitute a subtype of anti-GQ1b antibody syndrome.
描述与抗-GQ1b 抗体相关的急性前庭综合征 (AVS) 的临床特征和眼动表现。
我们回顾了 2004 年至 2018 年在首尔国立大学盆唐医院因各种神经综合征而血清抗-GQ1b 抗体阳性的 90 例患者。这些诊断包括典型的 Miller Fisher 综合征 (n=31)、无共济失调的急性眼肌麻痹 (n=27)、伴有眼肌麻痹的格林-巴利综合征 (n=18)、AVS (n=11) 和 Bickerstaff 脑干脑炎 (n=3)。其中，11 例 AVS 患者构成了本研究的基础。我们还对与抗-GQ1b 抗体相关的 AVS 进行了系统回顾。
AVS 患者表现出各种眼动表现，包括摇头性眼震 (n=6)、自发性眼震 (n=5)、凝视诱发眼震 (n=5)、中枢位置性眼震 (n=3)、管腔麻痹 (n=2) 和异常头脉冲试验 (n=1)，而无任何内或外眼肌麻痹。与其他亚型相比，AVS 患者大多表现出正常的深腱反射 (11 例中的 8 例 [73%]，=0.002) 和正常的神经传导研究结果 (4 例中的 4 例 [100%]，=0.010)。所有患者的症状发作后 6 个月内临床和实验室发现均明显缓解或改善。系统回顾进一步确定了 7 例 AVS 患者和阳性血清抗-GQ1b 抗体，他们表现出各种眼动表现，包括凝视诱发的眼震、扫视运动障碍、中枢位置性眼震、眼球震颤或眼阵挛。
抗-GQ1b 抗体可能通过涉及中枢或外周前庭结构引起急性前庭病。AVS 可能构成抗-GQ1b 抗体综合征的一个亚型。
