Illinois Neurologic Institute, Saint Francis Medical Center, University of Illinois College of Medicine, Peoria, Illinois..
J Neuroophthalmol. 2021 Dec 1;41(4):e655-e660. doi: 10.1097/WNO.0000000000001057.
Wernicke encephalopathy (WE) is classically described by a clinical triad consisting of confusion, ataxia, and ophthalmoplegia, but recent reports emphasize a history of malnutrition along with 2 elements of the WE triad (Caine's criteria) to enhance diagnostic sensitivity. The ophthalmoplegia, vestibular, and auditory expeditious improvement with intravenous thiamine usually confirms the diagnosis; serum levels generally provide additional diagnostic certainty.
Here, we discuss the case of a woman with a distant history of gastric sleeve, poor nutrition and protracted vomiting, who developed acute confusion, imbalance, near-total external ophthalmoplegia (EO), and hearing loss. The baseline thiamine level was 28 πmol/L (Normal: 70-180 πmol/L). We performed serial neurological, vestibular, and audiological examination to document over 5 days, the effect of intravenous (IV) thiamine, and again at 3 months with continued oral supplementation. We provide serial documentation with photographs and video recording of oculomotor abnormalities, audiometric testing, and a video of horizontal head impulse testing, and imaging findings.
Over the course of 5 days of IV thiamine supplementation, we demonstrate our patient's resolution of near complete EO. We assessed vestibular paresis with horizontal head impulse testing, after complete resolution of the EO. The initially positive bilateral h-HIT showed decreased gain and overt corrective saccades, it clinically resolved by day 5, but video h-HIT testing demonstrated persistent decreased horizontal vestibulo-ocular reflex (VOR) gain and covert horizontal saccades, which persisted at the 3-month examination. By contrast, the vertical VOR gain was normal without corrective saccades. Bedside audiometry completed during the acute phase demonstrated severely restricted auditory speech comprehension, which normalized 3 months later. Severe truncal ataxia improved as well.
This case is an example of how awareness of the variations in the clinical presentation of WE can be crucial in achieving an early diagnosis and obtaining better outcomes. A history of the poor nutritional status can be an important clue to aid in this early diagnosis.
Wernicke 脑病（WE）经典表现为三联征，包括意识混乱、共济失调和眼肌瘫痪，但近期的报告强调，病史中有营养不良以及 WE 三联征的 2 个要素（凯恩标准）可提高诊断敏感性。静脉内给予硫胺素后眼肌瘫痪、前庭和听觉可迅速改善，通常可确诊；血清水平通常可提供额外的诊断确定性。
在这里，我们讨论了一位有胃袖状切除术、营养不良和长期呕吐病史的女性，她出现了急性意识混乱、平衡障碍、近乎完全外展性眼肌瘫痪（EO）和听力损失。基线硫胺素水平为 28πmol/L（正常值：70-180πmol/L）。我们进行了连续的神经学、前庭和听力检查，以记录 5 天内静脉内（IV）硫胺素的效果，然后在 3 个月时继续口服补充硫胺素，进行检查。我们提供了连续的记录，包括眼动异常的照片和视频记录、听力测试和水平头脉冲测试的视频以及影像学发现。
在 5 天的 IV 硫胺素补充过程中，我们展示了我们患者近乎完全 EO 的缓解。我们通过水平头脉冲测试评估了前庭功能障碍，此时 EO 已完全缓解。最初双侧 h-HIT 阳性显示增益降低和明显的矫正性扫视，在第 5 天临床缓解，但视频 h-HIT 测试显示水平前庭眼反射（VOR）增益持续降低和隐蔽性水平扫视，在 3 个月的检查中仍然存在。相比之下，垂直 VOR 增益正常，没有矫正扫视。急性阶段完成的床边听力测试显示严重受限的听觉言语理解能力，3 个月后恢复正常。严重的躯干共济失调也有所改善。
这个病例是一个例子，说明了对 WE 临床表现的变化的认识如何在早期诊断和获得更好的结果中至关重要。营养不良的病史可能是帮助早期诊断的重要线索。
