Coexistence of IgLON5-IgG and SOX1-IgG in a Patient with Progressive Brainstem Dysfunction.

Q3 Medicine Acta neurologica Taiwanica Pub Date : 2023-09-30

Chutithep Teekaput, Kanokkarn Teekaput, Surat Tanprawate, Adisak Kittisares, Metha Apiwattanakul

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引用次数: 0

Abstract

Purpose: The coexistence of IgLON5-IgG and SOX1-IgG is rare. Previous reports have shown that patients with IgLON5-IgG spectrum disease present with sleep disorders, bulbar involvement, and autonomic abnormality, while SOX1-IgG positive patients present with peripheral nervous system symptoms such as the Lambert-Eaton Myasthenic Syndrome (LEMS).

Case report: We report a patient who presented with progressive ophthalmoplegia, ptosis, oropharyngeal dysphagia, gait instability, and sleep disorders. The paraneoplastic antibody screening tested doublepositive for IgLON5-IgG and SOX1-IgG. However, there was no clinical sign of LEMS in this patient. After extensive cancer screening, only lung nodules with hilar adenopathy were noted.

Conclusion: The coexistence of IgLON5-IgG with onconeuronal SOX1-IgG would suggest an underlying immune-mediated paraneoplastic process rather than secondary autoimmunity because of neurodegeneration. This is the first IgLON5-IgG case reported in Thailand, with a case of doublepositive IgLON5-IgG and SOX1-IgG as well. Keyword: IgLON5-IgG, SOX1-IgG, Paraneoplastic process, case report.

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进展性脑干功能障碍患者IgLON5 IgG和SOX1-IgG共存。

目的：IgLON5-IgG和SOX1-IgG共存的情况很少见。先前的报告显示，IgLON5-IgG谱系疾病患者表现为睡眠障碍、延髓受累和自主神经异常，而SOX1-IgG阳性患者表现为外周神经系统症状，如Lambert-Eaton肌无力综合征（LEMS）。病例报告：我们报告了一名患者，其表现为进行性眼肌麻痹、上睑下垂、口咽吞咽困难，步态不稳定和睡眠障碍。副肿瘤抗体筛查检测IgLON5 IgG和SOX1 IgG双阳性。然而，该患者没有LEMS的临床症状。经过广泛的癌症筛查，仅发现肺结节伴肺门腺病变。结论：IgLON5-IgG与肿瘤神经元SOX1-IgG的共存表明存在潜在的免疫介导的副肿瘤过程，而不是神经退行性变引起的继发性自身免疫。这是泰国报告的第一例IgLON5 IgG病例，还有一例IgLON8 IgG和SOX1 IgG双阳性病例。关键词：IgLON5 IgG，SOX1 IgG，副肿瘤过程，病例报告。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Acta neurologica Taiwanica Medicine-Neurology (clinical)

CiteScore

1.30

自引率

0.00%

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