Neurology:肝性脊髓病:肝性脑病的一种罕见神经系统并发症

2019-07-31 zyx整理 神经科病例撷英拾粹

57岁酒精性肝硬化男性,表现为肝性脑病(HE)和HE期间恶化的进行性步态障碍4年(视频)。查体提示下肢痉挛状态和无力。

57岁酒精性肝硬化男性,表现为肝性脑病(HE)和HE期间恶化的进行性步态障碍4年(视频)。查体提示下肢痉挛状态和无力。脑MRI可见基底节高信号,磁共振波谱提示谷氨酰胺/谷氨酸比值增加,肌醇减少(图)。脊柱MRI检查正常,并排除其他原因。诊断为肝性脊髓病。

肝性脊髓病是一种罕见的肝病并发症,其特征是数年来进行性痉挛状态和下肢无力。HE发作期间症状通常会恶化。HE治疗和肝移植可部分改善步态。


(图:A:T1WI可见苍白球内高信号[肝性脑病引起的锰沉积];B:磁共振波谱提示显示谷氨酰胺/谷氨酸[Glx,2.15–2.45ppm]比值增加,伴肌醇[Ins,3.45ppm]和胆碱[Cho,3.2ppm]下降。N-乙酰天门冬氨酸[NAA,2.0ppm]指数正常;这些表现常见于HE)


(酒精性肝硬化患者表现为痉挛性步态和下肢无力)

原始出处:Ciarlariello VB1, Fujino MVT1, Almeida MD1, et al. Teaching Video NeuroImages: Hepatic myelopathy: An unusual neurologic complication of hepatic encephalopathy. Neurology. 2019 Jul 16;

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    2020-05-09 丁鹏鹏
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    2019-10-25 yinhl1978
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    2020-02-26 HNYYM
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    2020-07-09 showtest
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    2019-08-02 syscxl

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