Department of Ophthalmology, Shin-Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan.
J Formos Med Assoc. 2013 Mar;112(3):165-70. doi: 10.1016/j.jfma.2012.10.010. Epub 2012 Dec 23.
The deficiency of thiamine manifesting as Wernicke's encephalopathy (WE) and concurrent optic neuropathy is rare. Herein, we report the case of a 29-year-old patient who suffered from bilateral sudden blindness and a disturbance of consciousness after 2 months of chronic diarrhea and minimal food intake. In addition, bilateral abducens nerve palsy with multidirectional nystagmus and no light perception in both eyes were noted. An ophthalmoscopic examination revealed bilateral disc edema with peripapillary flame-shaped hemorrhages. Although the results of analyzing the composition of cerebrospinal fluid showed that they are within normal limits, magnetic resonance imaging (MRI) revealed bilateral hyperintensity over the mammillary body, dorsal medial thalamus, and periaqueductal gray matter. As we suspected thiamine deficiency-induced WE, a high dose of intravenous thiamine was prescribed. After the administration of thiamine, both visual acuity and visual field rapidly improved with the simultaneous recovery of consciousness. This case indicates that, although rare, thiamine deficiency with WE may still occur in patients with chronic diarrhea in Taiwan. Thiamine deficiency should be considered in the differential diagnosis for patients who encounter sudden visual loss after prolonged periods of poor food intake and poor vitamin supplementation.
维生素 B1 缺乏表现为韦尼克脑病（WE）和并发的视神经病变较为罕见。本文报告了 1 例 29 岁患者，该患者在慢性腹泻和低食物摄入 2 个月后出现双侧突发性失明和意识障碍。此外，还观察到双侧展神经麻痹伴多向性眼球震颤和双眼无光感。眼底检查显示双侧视盘水肿伴视盘周围火焰状出血。尽管分析脑脊液成分的结果显示在正常范围内，但磁共振成像（MRI）显示双侧乳头部、背内侧丘脑和导水管周围灰质高信号。由于我们怀疑是维生素 B1 缺乏引起的 WE，因此给予大剂量静脉注射维生素 B1。给予维生素 B1 后，视力和视野迅速改善，同时意识恢复。本病例表明，尽管罕见，但台湾慢性腹泻患者仍可能发生维生素 B1 缺乏伴 WE。对于长期摄入不佳的食物和维生素补充不足后出现突发性视力丧失的患者，应考虑维生素 B1 缺乏症的鉴别诊断。
