Ye Lisha, Xu Zhouwei, Deng Jiangshan, Yang Jiajun
Department of Neurology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital, Shanghai, China.
School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
Front Neurol. 2020 Jun 10;11:451. doi: 10.3389/fneur.2020.00451. eCollection 2020.
The classical triad-ophthalmoplegia, cerebellar dysfunction, and altered mental state-in addition to bilateral symmetrical periventricular lesions are actually common to see, and clinicians tend to associate that with Wernicke's encephalopathy (WE). The diagnosis is strengthened with a likely deficiency of thiamine. We herein describe a malnourished patient with clinical triad and hyperintensities in the circumventricular regions, and she turned out to have neuromyelitis optica spectrum disorder (NMOSD) after many twists and turns. Despite totally different pathogenic mechanisms, NMOSD can mimic WE, sometimes even exhibiting radiological features similar to that of WE, thereby complicating the diagnosis. Our case highlights how similar these two diseases could be and the importance of differential diagnosis in clinical practice, which are so far rarely reported. Some clinical and radiological differences of these two diseases are summarized to help establish a prompt diagnosis.
经典三联征——眼肌麻痹、小脑功能障碍和精神状态改变——以及双侧对称的脑室周围病变实际上很常见,临床医生往往将其与韦尼克脑病(WE)联系起来。硫胺素缺乏可能会强化诊断。我们在此描述了一名营养不良的患者,有临床三联征且脑室周围区域有高信号,经过多次波折,她最终被诊断为视神经脊髓炎谱系障碍(NMOSD)。尽管致病机制完全不同,但NMOSD可模仿WE,有时甚至表现出与WE相似的放射学特征,从而使诊断变得复杂。我们的病例突出了这两种疾病可能有多相似以及临床实践中鉴别诊断的重要性,而这一点迄今为止鲜有报道。总结了这两种疾病的一些临床和放射学差异,以帮助快速做出诊断。