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Neurology病例:抗Hu抗体相关副肿瘤综合征听力丧失一例

 闫振文 2020-12-04

一位67岁的妇女被评估为亚急性4肢轻瘫、平衡恶化和双侧感音神经性听力损失(SNHL)。她被诊断为一个隐匿性小细胞肺癌的多发性淋巴结转移。神经生理学检查显示感觉神经病。

CSF分析发现轻微的蛋白质或糖原稍高,无细胞,抗Hu抗体阳性。脑MRI显示内耳道声神经对比增强(图1和图2)。亚急性SNHL是抗Hu相关的副肿瘤神经综合征(PNS)中最常见的颅神经病变。该病例强调了MRI在PNS诊断中的重要性,扩大了PNS中放射影像学发现的MRI频谱。

图1脑MRI:利用稳态采集图像进行的轴向快速成像显示了内部声通道中听神经(箭头)的高信号。

图2脑MRI:轴位成像增强脂肪抑制T1加权图像显示声神经对比度增强(箭头)。

 英文如下:

A case of hearing loss in a paraneoplasticsyndrome associated with anti-Hu antibody

 

A 67-year-old woman was evaluatedfor subacute 4-limb paresthesias, deteriorating balance, and bilateralsensorineural hearing loss (SNHL). She was diagnosed with multiple lymph node metastasisfrom an occult small cell lung carcinoma. Neurophysiologic testing revealedsensory neuronopathy.

CSFanalysis found mild proteinorrachia, no cells, and positivity for anti-Huantibodies. Brain MRI showed contrast enhancement of acoustic nerves in theinner acoustic channels (figures1 and 2).Subacute SNHL is the most common cranial neuropathy in anti-Hu-relatedparaneoplastic neurologic syndromes (PNS). This case underlines the importanceof MRI in PNS diagnosis, extending MRI spectrum of radiologic findings in PNS.

FIGURE 1 Brain MRI

Axial fast imaging employing steadystate acquisition image shows hyperintensity of acoustic nerves (arrows) in theinner acoustic channels.

FIGURE 2 Brain MRI

Axial enhanced fat-suppressedT1-weighted image shows contrast enhancement of acoustic nerves (arrows).

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