扩大抗DPPX脑炎的临床谱:一个多中心的回顾性研究
Yining Gao1, Yizongheng Zhang1, Hangxing Chunyu2
1Department of Neurology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Frontiers in neuroscience
|May 17, 2024
概括
这项研究揭示了抗 dipeptidyl-peptidase-like protein-6 (DPPX) 脑炎的关键临床特征,指出男性占主导地位和常见的症状,如认知障碍和过度兴奋. 由于复发风险,建议对男性患者进行长期监测.
科学领域:
- 神经免疫学 神经免疫学
- 自免疫性脑炎是什么?
- 罕见的神经疾病 罕见的神经疾病
背景情况:
- 抗 dipeptidyl-peptidase-like protein-6 (DPPX) 脑炎是一种罕见的自身免疫神经系统疾病,临床和实验数据有限.
- 了解抗DPPX脑炎的各种临床表现和结果对于改善患者管理至关重要.
研究的目的:
- 综合描述一组患有抗DPPX脑炎的中国患者的临床特征,诊断结果和治疗反应.
- 确定与抗DPPX脑炎的发展和进展相关的潜在病因和危险因素.
主要方法:
- 一项涉及14名被诊断患有抗DPPX脑炎的患者的回顾性研究,该研究基于血清和/或脑脊液中的抗体检测.
- 数据收集包括患者人口统计,临床症状,自身抗体概况,神经成像 (包括PET/MRI),同时存在的疾病,治疗和随访结果.
主要成果:
- 该队列包括14名患者 (9名男性),平均年龄为45.93岁. 主要症状包括认知障碍 (50%),中枢神经系统过度兴奋 (42.9%),胃肠功能障碍 (35.7%) 和精神疾病 (35.7%).
- 在两名患者的18F-FDG PET/MRI上,注意到了特定的发现. 同时存在的自身抗体,副感染,自身免疫性疾病和瘤在一组患者中被确定. 大多数患者通过免疫疗法得到改善.
- 一个值得注意的发现是,三个男性患者在六个月内复发,而女性患者没有复发.
结论:
- 抗DPPX脑炎表现出不同的临床表现和结果,在这个队列中男性患者占主导地位.
- 胃肠道功能障碍,认知/精神障碍和中枢神经系统过度兴奋的经典三位一体是常见的. 感染,免疫失调和瘤是潜在的触发因素.
- 由于复发风险较高,男性患者需要长期监测. 这项研究突出了抗DPPX脑炎的新型临床特征.
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