Lee Sun-Uk, Baek Seol-Hee, Kim Hyo-Jung, Choi Jeong-Yoon, Kim Byung-Jo, Kim Ji-Soo
Department of Neurology, Korea University Medical Center, Seoul, South Korea.
Research Administration Team, Seoul National University Bundang Hospital, Seongnam, South Korea.
J Neurol. 2023 Jan;270(1):486-492. doi: 10.1007/s00415-022-11394-3. Epub 2022 Sep 29.
Ophthalmoplegia is the diagnostic hallmark of anti-GQ1b antibody syndrome. This study aimed to define the patterns of acute comitant strabismus in patients with anti-GQ1b antibody syndromes. We retrospectively analyzed the ocular motor findings in 84 patients with anti-GQ1b antibody-associated ophthalmoplegia during the acute phases. Of the 84 patients, 11 (13%) showed acute comitant strabismus. Compared to those without, patients with acute comitant strabismus frequently showed abnormal ocular motor findings that included gaze-evoked (n = 8), spontaneous (n = 4) and positional nystagmus (n = 4), saccadic hypermetria (n = 3), head-shaking nystagmus (n = 2), pulse-step mismatch (n = 1), and impaired visual cancellation of the vestibulo-ocular reflex (n = 1, p < 0.001). On the contrary, iridoplegia (p = 0.029) and ptosis (p = 0.001) were more commonly observed in patients with paralytic (incomitant) strabismus than in those with acute comitant strabismus. Comitant strabismus can manifest during the acute phase of anti-GQ1b antibody syndromes in association with other central ocular motor abnormalities. These findings implicate that the cerebellum and/or brainstem can be the primary target of the anti-GQ1b antibodies.
眼肌麻痹是抗GQ1b抗体综合征的诊断标志。本研究旨在明确抗GQ1b抗体综合征患者急性共同性斜视的模式。我们回顾性分析了84例抗GQ1b抗体相关眼肌麻痹患者急性期的眼球运动检查结果。在这84例患者中,11例(13%)表现为急性共同性斜视。与无急性共同性斜视的患者相比,有急性共同性斜视的患者经常出现异常的眼球运动检查结果,包括凝视诱发性眼球震颤(n = 8)、自发性眼球震颤(n = 4)和位置性眼球震颤(n = 4)、扫视性远视(n = 3)、摇头性眼球震颤(n = 2)、脉冲-阶梯失配(n = 1)以及前庭眼反射视觉抑制受损(n = 1,p < 0.001)。相反,与急性共同性斜视患者相比,麻痹性(非共同性)斜视患者更常出现虹膜麻痹(p = 0.029)和上睑下垂(p = 0.001)。共同性斜视可在抗GQ1b抗体综合征急性期出现,并伴有其他中枢性眼球运动异常。这些发现提示小脑和/或脑干可能是抗GQ1b抗体的主要作用靶点。