多重自身免疫性:神经炎光学谱系障碍与兰伯特-伊顿肌肌综合征
Isobel Sarah Platt1,2, Albert Joseph2, Vasiliki Tsirka3
1Department of Clinical and Movement Neurosciences, University College London, London, UK.
Practical neurology
|February 18, 2025
概括
这一案例突出了一个患有神经omyelitis optica光谱障碍 (NMOSD) 的患者,他发展了兰伯特-伊顿肌肌综合征 (LEMS). 识别LEMS对于管理神经肌肉症状的NMOSD患者至关重要.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
- 在瘤学瘤学.
背景情况:
- 一名56岁的妇女患有水素-4抗体阳性神经脊髓炎光学谱障碍 (AQ4-NMOSD),呈现出逐渐下降的运动能力,体重减轻和声.
- 她的病史包括自身免疫性甲状腺毒毒症和高血压,显著的吸烟史引起了对恶性瘤的初步担忧.
研究的目的:
- 描述一例罕见的同时发生的兰伯特-伊顿肌综合征 (LEMS) 病例,该病例发生在患者中,该患者已被诊断为AQ4-NMOSD.
- 强调在已确诊的NMOSD患者中考虑和诊断其他自身免疫性疾病的重要性.
主要方法:
- 临床表现和神经生理学检查是关键的诊断工具.
- 电生理学揭示了前突触神经肌肉结位功能障碍.
- 抗体检测证实了高压通道抗体的升高,导致LEMS诊断.
主要成果:
- 该患者被诊断出患有兰伯特-伊顿骨髓综合征 (LEMS),同时她现有的AQ4-NMOSD.
- 在LEMS管理方面,开始使用3,4-diaminopyridine和rituximab治疗.
- 连续的FDG-PETCT扫描还没有确定潜在的恶性瘤.
结论:
- 兰伯特-伊顿肌综合征 (LEMS) 可以在患有神经髓炎光学谱系障碍 (NMOSD) 的患者中出现.
- 大约一半的LEMS病例与其他自身免疫性疾病有关.
- 及时识别和治疗LEMS等同时发生的自身免疫性疾病对于NMOSD患者的结果至关重要.
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