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牙关紧闭作为抗Hu副肿瘤性神经系统综合征的首发表现

Trismus as the Initial Presentation of Anti-Hu Paraneoplastic Neurological Syndrome.

作者信息

Mitarnun Witoon, Apiwattanakul Metha, Chindavech Narin, Sombat Benyapha, Jantapatsakun Suttanon, Sornnuwat Pheeracha

机构信息

Neurology Unit, Department of Internal Medicine, Buriram Hospital, Buriram, Thailand.

Neuroimmunology Unit, Department of Neurology, Neurological Institute of Thailand, Bangkok, Thailand.

出版信息

Neurohospitalist. 2024 Jul;14(3):336-338. doi: 10.1177/19418744241237593. Epub 2024 Mar 4.

DOI:10.1177/19418744241237593
PMID:38895005
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11181965/
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-cm 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岁男性出现亚急性牙关紧闭和全小脑功能障碍。脑部影像学检查和常规血液检查结果均无异常。胸部计算机断层扫描显示右上肺有一个4.7×2.5×1.8厘米的肺部肿块,强化不明显,右侧气管旁和肺门淋巴结肿大。右锁骨上淋巴结活检证实为转移性癌,鉴别诊断为小细胞癌和低分化癌,提示肺癌为原发灶。副肿瘤免疫组化筛查显示血清中抗Hu抗体滴度为1:7680(正常范围<1:240),脑脊液(CSF)中滴度为1:256(正常范围<1:2)。线印迹法检测血清中抗Zic4抗体呈阳性,滴度>1:10(正常范围<1:10),而脑脊液抗Zic4为阴性(正常范围<1:2)。该患者发生了难治性医院获得性肺炎和呼吸衰竭,随后自行出院。这个罕见病例表明牙关紧闭可能是抗Hu副肿瘤性神经综合征的初始表现,并强调了临床认识的重要性。

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本文引用的文献

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Anti-Hu--associated paraneoplastic encephalomyelitis/sensory neuronopathy. A clinical study of 71 patients.抗Hu相关副肿瘤性脑脊髓炎/感觉神经元病。71例患者的临床研究。
Medicine (Baltimore). 1992 Mar;71(2):59-72. doi: 10.1097/00005792-199203000-00001.