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伴瘤性小脑变性和 Lambert-Eaton 肌无力综合征伴 SOX-1 抗体。

Paraneoplastic Cerebellar Degeneration and Lambert-Eaton Myasthenic Syndrome with SOX-1 Antibodies.

机构信息

Department of Neurology, Kansai Electric Power Hospital, Japan.

Division of Clinical Neurology, Kansai Electric Power Medical Research Institute, Japan.

出版信息

Intern Med. 2021 May 15;60(10):1607-1610. doi: 10.2169/internalmedicine.5934-20. Epub 2020 Dec 15.

DOI:10.2169/internalmedicine.5934-20
PMID:33328403
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8188018/
Abstract

A 69-year-old man was admitted to our hospital for progressive muscle weakness in both lower limbs and limb ataxia (day 0). Nerve conduction studies showed low compound muscle action potential amplitudes at rest and increased amplitudes after maximum voluntary contraction. Blood testing revealed SOX-1 antibodies. He was diagnosed with paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome (PCD-LEMS). He died from aspiration pneumonia on day 9. Small-cell lung carcinoma (SCLC), which had not been obvious on computed tomography, was found during the autopsy. Patients with PCD-LEMS who test positive for SOX-1 antibodies should be carefully evaluated for SCLC.

摘要

一位 69 岁男性因双下肢进行性肌无力和肢体共济失调(入院第 0 天)而被收入我院。神经传导研究显示,在静息状态下复合肌肉动作电位幅度较低,在最大随意收缩后幅度增加。血液检测显示 SOX-1 抗体阳性。他被诊断为副肿瘤性小脑变性和 Lambert-Eaton 肌无力综合征(PCD-LEMS)。他于入院第 9 天因吸入性肺炎死亡。尸检时发现了小细胞肺癌(SCLC),但 CT 检查不明显。SOX-1 抗体阳性的 PCD-LEMS 患者应仔细评估 SCLC。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f5c/8188018/f070649a39d9/1349-7235-60-1607-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f5c/8188018/f6e9d1433298/1349-7235-60-1607-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f5c/8188018/f070649a39d9/1349-7235-60-1607-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f5c/8188018/f6e9d1433298/1349-7235-60-1607-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6f5c/8188018/f070649a39d9/1349-7235-60-1607-g002.jpg

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Lambert-Eaton Myasthenic Syndrome.兰伯特-伊顿肌无力综合征
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Prevalence of anti-SOX1 reactivity in various neurological disorders.各种神经系统疾病中抗SOX1反应性的患病率。
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