Kherallah Bassil, Samaha Elias, Bach Sarah E, Guede Cindy, Kattah Jorge C
Department of Neurology, University of Illinois College of Medicine, Peoria, IL, United States.
Department of Pathology, University of Illinois College of Medicine, Peoria, IL, United States.
Front Neurol. 2022 Aug 26;13:960584. doi: 10.3389/fneur.2022.960584. eCollection 2022.
We define acute vestibular syndrome (AVS) as a sudden onset vertigo, nausea, vomiting, and head motion intolerance, more frequently associated with an acute peripheral and unilateral vestibulopathy. About 10-20% of all cases with central vestibulopathy are secondary to stroke. We report three patients evaluated over the past decade with an acute AVS along with subtle downbeat nystagmus (DBN), followed by dysarthria and progressive truncal and limb ataxia, as well as increasing DBN intensity.
All patients underwent neurologic examination, video-oculography, MRI, serum cancer markers, spinal fluid examination, paraneoplastic panel testing, and oncologic workup. With a consolidated diagnosis of cancer/paraneoplastic syndrome, we treated with plasma exchange (PLEX), high-dose steroids, surgery, and oncologic investigation. We additionally provided oncotherapy in one out of three patients.
All three patients had an acute AVS, downbeat nystagmus DBN, and inability to perform tandem gait. Two of three patients had a normal head impulse test (HIT). As acute vertigo, nausea, and vomiting subsided, a progressive cerebellar syndrome ensued characterized by persistent DBN, impaired horizontal and vertical pursuit, impaired VOR suppression, truncal and limb ataxia, and dysarthria. All patients had normal MRI brain studies excluding stroke. CSF studies demonstrated lymphocytic pleocytosis and elevated protein. One patient had confirmed ovarian cancer with high CA-125 serum levels; another had undifferentiated cancer of unknown primary with high CA-125 and one patient with esophageal cancer. All had a positive PCA-1 antibody titer, also known as anti-Yo antibody. In one patient with expeditious immunosuppression, the ataxia progression slowed for 18 months, whereas the other two patients with delayed initiation of treatment had more rapidly progressive ataxia.
Paraneoplastic encephalitis related to PCA-1 antibody (Anti-Yo) targets Purkinje cells and cells in the granular layer of the cerebellar cortex. Clinically, our patients had a central AVS characterized by DBN and followed with progressive ataxia and unremarkable neuroimaging studies. Rapid initiation of treatment may offer a greater chance to prevent further neurologic decline. Any patient with an AVS as well as DBN and normal MRI should have an expeditious workup to rule out metabolic, toxic, and infectious causes just prior to considering prompt treatment with high-dose steroids and plasma exchange (PLEX) to mitigate the risk of rapidly progressive and irreversible neurologic decline.
我们将急性前庭综合征（AVS）定义为突然发作的眩晕、恶心、呕吐和头部运动不耐受，更常见于急性外周性和单侧前庭病变。所有中枢性前庭病变病例中约10 - 20%继发于中风。我们报告了过去十年中评估的三名患者，他们患有急性AVS并伴有轻微的下跳性眼球震颤（DBN），随后出现构音障碍、进行性躯干和肢体共济失调以及DBN强度增加。
所有患者均接受了神经系统检查、视频眼震图检查、MRI、血清肿瘤标志物检查、脑脊液检查、副肿瘤综合征检测组检测以及肿瘤学检查。在确诊为癌症/副肿瘤综合征后，我们采用血浆置换（PLEX）、大剂量类固醇、手术及肿瘤学检查进行治疗。三名患者中有一名还接受了肿瘤治疗。
所有三名患者均有急性AVS、下跳性眼球震颤DBN以及无法进行串联步态。三名患者中有两名头部脉冲试验（HIT）正常。随着急性眩晕、恶心和呕吐症状消退，随之出现了以持续性DBN、水平和垂直跟踪受损、视动性眼震抑制受损、躯干和肢体共济失调以及构音障碍为特征的进行性小脑综合征。所有患者的脑部MRI检查均正常，排除了中风。脑脊液检查显示淋巴细胞增多和蛋白升高。一名患者确诊为卵巢癌，血清CA - 125水平高；另一名患者为原发性不明的未分化癌，CA - 125高，还有一名患者患有食管癌。所有患者PCA - 1抗体滴度均为阳性，也称为抗Yo抗体。一名接受快速免疫抑制治疗的患者，共济失调进展减缓了18个月，而另外两名治疗开始延迟的患者共济失调进展更快。
与PCA - 1抗体（抗Yo）相关的副肿瘤性脑炎靶向浦肯野细胞和小脑皮质颗粒层中的细胞。临床上，我们的患者表现为以DBN为特征的中枢性AVS，随后出现进行性共济失调且神经影像学检查无明显异常。快速开始治疗可能有更大机会预防进一步的神经功能衰退。任何患有AVS以及DBN且MRI正常的患者，在考虑用大剂量类固醇和血浆置换（PLEX）进行快速治疗以降低快速进展和不可逆神经功能衰退风险之前，都应迅速进行检查以排除代谢、中毒和感染性病因。
