Kattah Jorge C, Dhanani Sara S, Pula John H, Mantokoudis Georgios, Tehrani Ali S Saber, Toker David E Newman
Department of Neurology (JCK, SSD, JHP), Illinois Neurologic Institute and the University of Illinois College of Medicine at Peoria; and Department of Neurology (GM, ASST, DENT), Johns Hopkins University School of Medicine, Baltimore, MD.
Neurol Clin Pract. 2013 Dec;3(6):460-468. doi: 10.1212/01.CPJ.0000435749.32868.91.
Non-encephalopathic presentations of CNS thiamine deficiency may be difficult to diagnose. We describe neuro-otologic findings of Wernicke syndrome in 5 patients with vestibular manifestations. Diagnosis was confirmed by low serum levels, response to replacement, and brain MRI to exclude other causes. All had bilaterally abnormal horizontal head impulse vestibulo-ocular reflex (VOR) responses and pathologic gaze-evoked nystagmus, without encephalopathy. After thiamine replacement, 4 had total resolution of vestibular and oculomotor findings. Novel findings included 2 patients whose VOR function improved within minutes of IV repletion and 1 whose recovery was documented by serial quantitative recordings. Early diagnosis of Wernicke by examining vestibular reflexes and prompt IV treatment might prevent encephalopathy and other neurologic or systemic complications of thiamine depletion.
中枢神经系统硫胺素缺乏的非脑病表现可能难以诊断。我们描述了5例有前庭表现的韦尼克综合征的神经耳科检查结果。通过低血清水平、补充治疗反应以及脑部MRI排除其他病因来确诊。所有患者双侧水平头脉冲前庭眼反射（VOR）反应异常且存在病理性视动性眼球震颤，无脑病表现。硫胺素补充后，4例患者的前庭和动眼神经检查结果完全恢复。新发现包括2例患者在静脉补充后数分钟内VOR功能改善，以及1例患者通过系列定量记录记录了恢复情况。通过检查前庭反射早期诊断韦尼克综合征并及时进行静脉治疗可能预防硫胺素缺乏引起的脑病及其他神经或全身并发症。
