单侧皮层自身免疫脑炎:一个病例系列和与晚发拉斯穆森脑炎的比较
Sophia Damman1, Persen Sukpornchairak2, Amit Ahituv3
1Case Western Reserve University School of Medicine, 10900 Euclid Avenue, Cleveland, OH 44106, USA.
Journal of neuroimmunology
|May 10, 2024
概括
一种单边自身免疫脑炎 (AE) 的新型模式在老年人中更为常见,并且往往是血清阴性. 这种情况类似于拉斯穆森脑炎,但对免疫疗法反应更好.
科学领域:
- 神经学 神经学
- 免疫学 免疫学 免疫学
- 神经科学是一个神经科学.
背景情况:
- 自身免疫性脑炎 (AE) 涵盖了一系列炎症性脑疾病.
- 拉斯穆森脑炎是一种罕见的慢性炎症神经疾病,影响大脑的一半球.
- 晚期发病的神经疾病带来了独特的诊断和治疗挑战.
研究的目的:
- 描述一种新发现的自身免疫性脑炎 (AE) 的解剖学模式.
- 描述AE中严格单边皮质炎症的临床表现,病因和结果.
- 为了将这种模式与拉斯穆森晚期发作的脑炎进行比较.
主要方法:
- 来自两个高等推学术中心的回顾性数据收集.
- 纳入标准用于识别相关患者病例.
- 对临床数据,神经成像 (MRI),脑电图 (EEG) 和血清学发现的分析.
主要成果:
- 确定了12例单边皮层AE (平均年龄65岁,58%为女性).
- 常见的表现包括焦点发作,认知能力下降,半皮质缺陷和单方面MRI/EEG异常.
- 病因包括异常病 (6),瘤病 (2),阴性病 (2) 和后COVID-19 (2);大多数是血清阴性.
结论:
- 单侧皮质AE在老年人中更为普遍,通常是异常和血清阴性.
- 这种AE变种与晚发拉斯穆森脑炎具有共同特征,但对免疫治疗有更好的反应.
- 考虑针对耐火拉斯穆森脑炎的干预措施,如半球切除术,可能与耐火单边AE病例有关.
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