关键词: anti-Hu anti-Zic4 lung cancer pancerebellar dysfunction paraneoplastic neurological syndrome trismus

来  源:   DOI:10.1177/19418744241237593   PDF(Pubmed)

Abstract:
A 73-year-old man presented with subacute trismus and pancerebellar dysfunction. Brain imaging and routine blood test results were unremarkable. Chest computed tomography revealed an indistinctly enhancing 4.7 × 2.5 × 1.8-cm3 pulmonary mass in the right upper lung, with enlarged right paratracheal and hilar lymph nodes. Biopsy of the right supraclavicular lymph node confirmed metastatic carcinoma, with differential diagnoses of small cell carcinoma and poorly differentiated carcinoma, indicating lung cancer as the primary source. Paraneoplastic immunohistochemistry screening revealed anti-Hu antibodies in the serum at a titer of 1:7680 (normal range <1:240) and in the cerebrospinal fluid (CSF) at a titer of 1:256 (normal range <1:2). The line blot method yielded positive results for anti-Zic4 antibodies in serum, with a titer of >1:10 (normal range <1:10), whereas CSF anti-Zic4 was negative (normal range <1:2). The patient developed non-responsive hospital-acquired pneumonia and respiratory failure, and discharged himself against medical advice. This rare case indicates that trismus can be an initial manifestation of anti-Hu paraneoplastic neurological syndrome, and emphasizes the importance of clinical awareness.
摘要:
一名73岁的男子表现为亚急性三端和小脑功能障碍。脑影像学和血常规检查结果无明显变化。胸部计算机断层扫描显示右上肺的肺肿块不明显增强4.7×2.5×1.8-cm3,右气管旁和肺门淋巴结肿大。右锁骨上淋巴结活检证实转移癌,小细胞癌和低分化癌的鉴别诊断,表明肺癌是主要来源。副肿瘤免疫组织化学筛选显示血清中抗Hu抗体的滴度为1:7680(正常范围<1:240),脑脊液(CSF)中抗Hu抗体的滴度为1:256(正常范围<1:2)。线印迹方法对血清中的抗Zic4抗体产生阳性结果,效价>1:10(正常范围<1:10),而CSF抗Zic4为阴性(正常范围<1:2)。患者出现无反应性医院获得性肺炎和呼吸衰竭,并不顾医嘱自行出院.这种罕见的病例表明,三聚体可能是抗Hu副肿瘤神经综合征的初始表现,强调临床意识的重要性。
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