USC Roski Eye Institute, Keck School of Medicine, University of Southern California, Los Angeles, United States.
USC Roski Eye Institute, Keck School of Medicine, University of Southern California, Los Angeles, United States.
J Clin Neurosci. 2020 Aug;78:422-425. doi: 10.1016/j.jocn.2020.04.101. Epub 2020 Apr 29.
A 22-year old male with a history of B-cell acute lymphoblastic leukemia with recent bone marrow transplantation and on immunosuppressive therapy presented with painless, subacute vision loss of two weeks duration. He exhibited a horizontal gaze palsy, nystagmus, and mildly swollen and hyperemic optic discs with peripapillary flame hemorrhage on retinal exam. He had bilateral cecocentral scotomas on visual field exam, and MRI of his brain/orbits demonstrated hyperintensities in the hypothalamus, periaqueductal gray, and dorsal rostral medullary regions. After continued progression of symptoms despite discontinuation of the patient's tacrolimus, an empiric trial of IV thiamine treatment was started before the patient's lab vitamin levels were available, given strong clinical suspicion for a nutritional etiology. The patient's clinical presentation improved dramatically, and he achieved a final visual acuity of 20/20, full visual fields bilaterally, and resolution of nystagmus. A final diagnosis of Wernicke's encephalopathy was supported by his clinical course, imaging findings, and further confirmation with blood thiamine levels. This case presents unique ocular manifestations of Wernicke's encephalopathy and highlights the importance of early diagnosis in this potentially reversible condition.
一位 22 岁男性，既往患有 B 细胞急性淋巴细胞白血病，近期行骨髓移植并接受免疫抑制治疗，因无痛性、亚急性视力丧失两周就诊。他表现出水平性眼球运动障碍、眼球震颤，眼底检查可见视神经盘肿胀、充血，伴有视盘周围火焰状出血。视野检查显示双侧旁中心暗点，头颅/眼眶 MRI 显示下丘脑、导水管周围灰质和背侧颅颈髓区域呈高信号。尽管停用了他克莫司，患者的症状仍继续进展，在等待患者的实验室维生素水平结果期间，由于强烈的临床怀疑存在营养性病因，开始进行经验性 IV 硫胺素治疗试验。患者的临床症状显著改善，最终视力达到 20/20，双侧视野完全，眼球震颤消失。他的临床病程、影像学发现以及血液硫胺素水平的进一步证实，均支持诊断为韦尼克脑病。该病例表现出韦尼克脑病独特的眼部表现，强调了在这种潜在可逆转的情况下早期诊断的重要性。
