Prakash Sanjay, Joshi Hemant, Patel Jay
Department of Neurology, Smt BK Shah Medical Institute and Research Centre, Vadodara, Gujarat, India.
BMJ Case Rep. 2018 Aug 20;2018:bcr-2018-226170. doi: 10.1136/bcr-2018-226170.
Progressive supranuclear palsy (PSP) may be a risk factor for thiamine deficiency. The classic symptoms of PSP (postural instability, supranuclear vertical gaze palsy and dementia) overlap with the clinical triad of Wernicke's encephalopathy (cognitive impairment, gait problems and ocular abnormality). Therefore, superimposed thiamine deficiency in patients with PSP may aggravate the pre-existing symptoms of PSP. Here, we are reporting a 64-year-old woman having supranuclear ocular palsy, gait instability and dementia for the past 2-3 years. The patient fulfilled the diagnostic criteria of PSP. In parallel, she fulfilled the Caine's criteria of Wernicke's encephalopathy. Her serum thiamine level was low. Supplementation of thiamine led to marked improvement in the symptoms which had been present for many years. These symptoms were originally presumed to be due to PSP. This case highlights the needs to identify superimposed thiamine deficiency in patients with PSP.
进行性核上性麻痹（PSP）可能是硫胺素缺乏的一个危险因素。PSP的典型症状（姿势不稳、核上性垂直凝视麻痹和痴呆）与韦尼克脑病的临床三联征（认知障碍、步态问题和眼部异常）重叠。因此，PSP患者叠加硫胺素缺乏可能会加重PSP已有的症状。在此，我们报告一名64岁女性，在过去2至3年中患有核上性眼肌麻痹、步态不稳和痴呆。该患者符合PSP的诊断标准。同时，她也符合韦尼克脑病的凯恩标准。她的血清硫胺素水平较低。补充硫胺素后，多年来一直存在的症状有显著改善。这些症状最初被认为是由PSP引起的。该病例凸显了识别PSP患者中叠加硫胺素缺乏的必要性。
