Department of Neurology (JN, GB, FW, XZ, and JK), University of Illinois College of Medicine Peoria, Illinois Neurologic Institute, OSF St. Francis Medical Center, Peoria, Illinois; Department of Neurology (JK and FW), Illinois Neurologic Institute OSF St. Francis Medical Center, Peoria, Illinois; and Department of Internal Medicine (SB), University of Illinois College of Medicine Peoria, OSF St. Francis Medical Center, Peoria, Illinois.
J Neuroophthalmol. 2022 Jun 1;42(2):251-255. doi: 10.1097/WNO.0000000000001498. Epub 2021 Dec 30.
The opsoclonus-myoclonus-ataxia syndrome (OMAS) represents a pathophysiology and diagnostic challenge. Although the diverse etiologies likely share a common mechanism to generate ocular, trunk, and limb movements, the underlying cause may be a paraneoplastic syndrome, as the first sign of cancer, or may be a postinfectious complication, and thus, the outcome depends on identifying the trigger mechanism. A recent hypothesis suggests increased GABAA receptor sensitivity in the olivary-oculomotor vermis-fastigial nucleus-premotor saccade burst neuron circuit in the brainstem. Therefore, OMAS management will focus on immunosuppression and modulation of GABAA hypersensitivity with benzodiazepines.
We serially video recorded the eye movements at the bedside of 1 patient with SARS-CoV-2-specific Immunoglobulin G (IgG) serum antibodies, but twice-negative nasopharyngeal reverse transcription polymerase chain reaction (RT-PCR). We tested cerebrospinal fluid (CSF), serum, and nasopharyngeal samples. After brain MRI and chest, abdomen, and pelvis CT scans, we treated our patient with clonazepam and high-dose Solu-MEDROL, followed by a rituximab infusion after her formal eye movement analysis 10 days later.
The recordings throughout her acute illness demonstrated different eye movement abnormalities. While on high-dose steroids and clonazepam, she initially had macrosaccadic oscillations, followed by brief ocular flutter during convergence the next day; after 10 days, she had bursts of opsoclonus during scotopic conditions with fixation block but otherwise normal eye movements. Concern for a suboptimal response to high-dose Solu-MEDROL motivated an infusion of rituximab, which induced remission. An investigation for a paraneoplastic etiology was negative. CSF testing showed elevated neuron-specific enolase. Serum IgG to Serum SARS-CoV2 IgG was elevated with negative RT-PCR nasopharyngeal testing.
A recent simulation model of macrosaccadic oscillations and OMAS proposes a combined pathology of brainstem and cerebellar because of increased GABAA receptor sensitivity. In this case report, we report 1 patient with elevated CSF neuronal specific enolase, macrosaccadic oscillations, ocular flutter, and OMAS as a SARS-CoV-2 postinfectious complication. Opsoclonus emerged predominantly with fixation block and suppressed with fixation, providing support to modern theories on the mechanism responsible for these ocular oscillations involving cerebellar-brainstem pathogenesis.
眼-面-共济失调-肌阵挛综合征（OMAS）代表了一种病理生理学和诊断挑战。尽管不同的病因可能具有产生眼部、躯干和肢体运动的共同机制，但根本原因可能是副肿瘤综合征，作为癌症的第一个迹象，也可能是感染后并发症，因此，结果取决于识别触发机制。最近的一个假设表明，在脑干中的橄榄-眼动小脑蚓部--fastigial 核-运动前扫视爆发神经元回路中，GABAA 受体敏感性增加。因此，OMAS 的治疗将侧重于免疫抑制和用苯二氮䓬类药物调节 GABAA 超敏反应。
我们连续记录了 1 例 SARS-CoV-2 特异性 IgG 血清抗体阳性但两次鼻咽 RT-PCR 阴性的患者的眼动。我们测试了脑脊液（CSF）、血清和鼻咽样本。在进行脑部 MRI 和胸部、腹部和骨盆 CT 扫描后，我们用氯硝西泮和大剂量 Solu-MEDROL 治疗我们的患者，然后在 10 天后她的正式眼动分析后进行利妥昔单抗输注。
在她的急性疾病期间的记录显示出不同的眼动异常。在大剂量类固醇和氯硝西泮治疗期间，她最初有巨扫视性震颤，接下来的一天在会聚时出现短暂的眼球颤动；10 天后，在暗适应条件下出现扫视性眼球震颤爆发，但其他眼部运动正常。对大剂量 Solu-MEDROL 反应不佳的担忧促使进行利妥昔单抗输注，这诱导了缓解。副肿瘤病因学的调查结果为阴性。CSF 测试显示神经元特异性烯醇化酶升高。血清 IgG 对血清 SARS-CoV2 IgG 升高，鼻咽 RT-PCR 检测为阴性。
最近的巨扫视性震颤和 OMAS 模拟模型提出，由于 GABAA 受体敏感性增加，脑干和小脑的联合病理学。在这个病例报告中，我们报告了 1 例 CSF 神经元特异性烯醇化酶升高、巨扫视性震颤、眼球颤动和 OMAS 作为 SARS-CoV-2 感染后并发症的患者。眼震主要表现为固定阻断，并随固定抑制，为涉及小脑-脑干发病机制的这些眼部震颤的负责机制的现代理论提供支持。
