Guagliardo Emily, Singh Divya, Thakkar Jasmine, Rodriguez Wilson, Pastrana Francesca, Qaqish Hanan, Chand Pratap
Neurology, Saint Louis University School of Medicine, St. Louis, USA.
Cureus. 2024 Jul 15;16(7):e64613. doi: 10.7759/cureus.64613. eCollection 2024 Jul.
Neuro-ophthalmic manifestations of Wernicke encephalopathy (WE) are uncommon and vary from nystagmus, oculomotor palsies, anisocoria, and optic disc edema to vision loss. We describe a case of a 53-year-old woman presenting with subacute bilateral painless vision decline, lower-extremities weakness with impaired ambulation, headache, and abdominal pain. Neurological examination was pertinent for confabulation, bilateral decreased visual acuity with an absent blink to threat, absent afferent pupillary defect and fundus abnormalities, and significant allodynia in bilateral lower extremities. Besides elevated inflammatory marker with an erythrocyte sedimentation rate (ESR) of 130 mm/hr, her infectious, autoimmune, paraneoplastic, and neuromyelitis optica work-up was overall unremarkable. Brain MRI showed abnormal fluid-attenuated inversion recovery (FLAIR) signaling in bilateral mammillary bodies and around periaqueductal gray matter concerning WE. Due to concerns of Wernicke-Korsakoff syndrome (WKS), parenteral high-dose thiamine was initiated with significant clinical improvement. The patient was also later found to have a positive anti-myelin oligodendrocyte glycoprotein (MOG) antibody, which was deemed false positive given the atypical phenotype and symptomatic improvement with thiamine supplementation. This case encourages the consideration of vision loss as a manifestation of WKS, especially in patients who have risk factors. Testing serum levels of thiamine is strongly encouraged; however, initiating empiric treatment is advocated for high clinical suspicion due to its reversible nature and minimal risk for side effects.
韦尼克脑病(WE)的神经眼科表现并不常见,从眼球震颤、动眼神经麻痹、瞳孔不等大、视盘水肿到视力丧失等症状各异。我们描述了一例53岁女性患者,表现为亚急性双侧无痛性视力下降、下肢无力伴行走障碍、头痛和腹痛。神经系统检查发现有虚构症、双侧视力下降、对威胁无眨眼反应、无传入性瞳孔障碍和眼底异常,以及双侧下肢明显的痛觉过敏。除了炎症标志物升高,红细胞沉降率(ESR)为130mm/小时外,她的感染、自身免疫、副肿瘤和视神经脊髓炎相关检查总体无异常。脑部磁共振成像(MRI)显示双侧乳头体和导水管周围灰质周围的液体衰减反转恢复(FLAIR)信号异常,提示韦尼克脑病。由于担心韦尼克-科尔萨科夫综合征(WKS),开始静脉注射高剂量硫胺素,临床症状有显著改善。该患者后来还被发现抗髓鞘少突胶质细胞糖蛋白(MOG)抗体呈阳性,但鉴于其非典型表型和补充硫胺素后症状改善,该抗体被认为是假阳性。这个病例促使我们考虑视力丧失可能是WKS的一种表现,尤其是在有危险因素的患者中。强烈建议检测血清硫胺素水平;然而,由于其可逆性和副作用风险极小,对于高度怀疑的临床情况,主张进行经验性治疗。