Shible Ahmed A, Ramadurai Deepa, Gergen Daniel, Reynolds Paul M
Department of Clinical Pharmacy, University of Colorado Skaggs School of Pharmacy and Pharmaceutical Sciences, Aurora, CO, USA.
Department of Pharmacy, Hamad General Hospital, Hamad Medical Corporation, Doha, Qatar.
Am J Case Rep. 2019 Mar 13;20:330-334. doi: 10.12659/AJCR.914051.
BACKGROUND Beriberi due to thiamine (vitamin B1) deficiency has two clinical presentations. Patients with dry beriberi present with neuropathy, and patients with wet beriberi present with heart failure, with or without neuropathy. Dry beriberi can mimic the most common form of Guillain-Barre syndrome (GBS), an acute inflammatory demyelinating polyradiculoneuropathy (AIDP). Severe thiamine deficiency results in Wernicke's encephalopathy. This report of a case of dry beriberi and Wernicke's encephalopathy due to thiamine deficiency includes a review of the literature. CASE REPORT A 56-year old woman with a history of gallstone pancreatitis and protein-calorie malnutrition was treated six months previously with total parenteral nutrition (TPN). She initially presented at another hospital with paresthesia of the lower limbs, arms, and neck, and symptoms of encephalopathy. Initial diagnosis of GBS was made, based on magnetic resonance imaging (MRI) and cerebrospinal fluid (CSF) findings. Despite five days of intravenous immunoglobulin (IVIG) treatment, her encephalopathy worsened, requiring transfer to our hospital, where she required intubation and treatment with vasopressors. A repeat MRI of her brain showed changes consistent with Wernicke's encephalopathy. Following treatment with high-dose intravenous thiamine, her mental status improved within 48 hours, and by the third hospital day, she no longer required intubation. CONCLUSIONS Symptoms and signs of dry beriberi due to thiamine deficiency can mimic those of acute or chronic GBS. However, thiamine repletion leads to rapid clinical improvement and can prevent irreversible neurologic sequelae, including Korsakoff syndrome. Clinicians should consider thiamine deficiency in malnourished patients presenting with symptoms and signs of GBS.
背景 硫胺素（维生素B1）缺乏所致的脚气病有两种临床表现。干性脚气病患者表现为神经病变，湿性脚气病患者表现为心力衰竭，可伴有或不伴有神经病变。干性脚气病可能类似于格林-巴利综合征（GBS）最常见的形式，即急性炎症性脱髓鞘性多发性神经根神经病（AIDP）。严重的硫胺素缺乏会导致韦尼克脑病。本病例报告介绍了一例因硫胺素缺乏导致的干性脚气病和韦尼克脑病，并对相关文献进行了综述。病例报告 一名56岁女性，有胆石性胰腺炎和蛋白质-热量营养不良病史，6个月前接受了全胃肠外营养（TPN）治疗。她最初在另一家医院就诊时，出现下肢、手臂和颈部感觉异常以及脑病症状。根据磁共振成像（MRI）和脑脊液（CSF）检查结果，初步诊断为GBS。尽管接受了5天的静脉注射免疫球蛋白（IVIG）治疗，她的脑病仍恶化，需要转至我院，在我院她需要插管并使用血管升压药治疗。对她的脑部进行的重复MRI检查显示出与韦尼克脑病相符的变化。在接受大剂量静脉注射硫胺素治疗后，她的精神状态在48小时内有所改善，到住院第三天，她不再需要插管。结论 硫胺素缺乏所致干性脚气病的症状和体征可能类似于急性或慢性GBS的症状和体征。然而，补充硫胺素可使临床症状迅速改善，并可预防包括科萨科夫综合征在内的不可逆神经后遗症。临床医生对于出现GBS症状和体征的营养不良患者应考虑硫胺素缺乏的可能性。
