Department of Neurology, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117 Berlin, Germany.
J Neurol Neurosurg Psychiatry. 2012 Feb;83(2):195-8. doi: 10.1136/jnnp-2011-300411. Epub 2011 Sep 20.
Anti-NMDA receptor (NMDAR) encephalitis is a recently characterised autoimmune disorder mainly affecting young women. Although the clinical features of the acute disease are well characterised, cognitive long-term outcome has not been examined in detail.
The authors investigated cognitive performance in nine patients with proven anti-NMDAR encephalitis after recovery from the acute disease period (median 43 months after disease onset, range 23 to 69). Patients underwent a comprehensive neuropsychological assessment, including memory tasks that have previously been shown to be sensitive for hippocampal dysfunction.
Substantial persistent cognitive impairments were observed in eight out of nine patients that mainly consisted of deficits in executive functions and memory. The severity of these deficits varied inter-individually. Patients with early immunotherapy performed significantly better. The most severe deficits were observed with inefficient or delayed initial treatment.
Our results suggest that cognitive deficits constitute a major long-term morbidity of anti-NMDAR encephalitis. These deficits relate to the distribution of NMDARs in the human brain and their functional role in normal cognition. Good cognitive long-term outcome may depend on early and aggressive treatment.
抗 N- 甲基-D- 天冬氨酸受体（NMDAR）脑炎是一种新近被描述的自身免疫性疾病，主要影响年轻女性。尽管急性疾病的临床特征已得到很好的描述，但认知的长期预后尚未得到详细研究。
作者研究了 9 名确诊的抗 NMDAR 脑炎患者在急性疾病期恢复后的认知表现（中位数为发病后 43 个月，范围为 23 至 69 个月）。患者接受了全面的神经心理学评估，包括以前显示对海马功能障碍敏感的记忆任务。
9 名患者中有 8 名存在明显的持续性认知障碍，主要表现为执行功能和记忆缺陷。这些缺陷的严重程度因人而异。接受早期免疫治疗的患者表现明显更好。初始治疗效率低下或延迟会导致最严重的缺陷。
我们的研究结果表明，认知缺陷是抗 NMDAR 脑炎的主要长期致残因素。这些缺陷与 NMDAR 在人类大脑中的分布及其在正常认知中的功能作用有关。良好的认知长期预后可能取决于早期和积极的治疗。
