崩反应媒介蛋白5-关联视神经病变:临床特征,放射学线索和结果
Rong Yan1, Yu Mao2, Huiyang Zeng3
1Department of Neurology, Beijing Tongren Hospital, Capital Medical University, Beijing, China.
Frontiers in neurology
|July 14, 2023
概括
崩反应媒介蛋白5-关联视觉神经病变 (CRMP5-ON) 经常伴有视觉盘,并可能与癌症有关. 早期癌症查和干预对于改善CRMP5-ON患者的治疗结果至关重要.
科学领域:
- 神经科学是一个神经科学.
- 眼科医生 眼科 眼科
- 免疫学 免疫学 免疫学
背景情况:
- 崩反应媒介蛋白5-关联视神经病变 (CRMP5-ON) 是一种罕见的自身免疫视神经病变.
- 了解其临床表现,诊断线索和治疗结果对于患者管理至关重要.
研究的目的:
- 审查CRMP5-ON患者的神经眼科发现和结果.
- 描述CRMP5-ON的临床表型,放射学发现和预后.
主要方法:
- 追溯病例系列和医疗病历审查.
- 参与的患者在2020年12月至2023年3月期间被诊断患有CRMP5血清阳性视觉神经病变 (ON).
- 主要结局包括神经眼科表现,放射性特征,临床结局,相关的自身抗体,副瘤综合征和治疗影响.
主要成果:
- 五名患者 (80%为女性,平均年龄为59.4岁) 被确定为CRMP5-ON.
- 所有患者都经历了无痛的双侧视盘;80%患有视炎,视网膜炎或脑膜炎.
- 核磁共振扫描显示80%的视神经或外增强.
- 60%的人有其他神经症状;80%的人有相关的癌症 (肺,甲状腺,胸腺瘤).
- 平均治疗后 (IVIG,IVMP,手术,化疗) 视力敏度改善到20/50.
结论:
- CRMP5-ON通常表现为双边光盘,通常伴有眼内炎症.
- 放射学发现可能包括视神经或增强.
- CRMP5-ON与神经瘤综合征密切相关,需要进行癌症查和及时干预以获得更好的预后.
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