文摘
摘要目的:确定额患者认知行为表型的患病率anti-leucine-rich glioma-inactivated蛋白1 (LGI1)脑炎。
背景:认知障碍是一种常见的表现anti-LGI1脑炎和记忆缺陷通常定义为突出。我们经常遇到额认知行为赤字在评估这些患者,但这在文献中尚未被很好的描述。
设计/方法:我们回顾性病人从三个三级中心在多伦多,安大略省anti-LGI1确诊脑炎2013年10月至2022年9月。病人电子病历进行评估认知功能和额叶功能分类基于行为变异额颞叶痴呆诊断标准(bvFTD)。
结果:15个患者识别(平均年龄65年(18 - 84);8(53.3%)的男性)。14例(93.3%)患者认知症状,局部大脑额叶。两个发达在类固醇治疗这些症状,因此排除在进一步分析。剩下的12名患者出现行为抑制解除(n = 11),冷漠或惯性(n = 5),持续的,刻板或强迫性仪式行为(n = 4), hyperorality和饮食改变(n = 4),神经心理档案主要赤字在执行任务(n = 4),和损失的同情或移情(n = 1)。7(46.7%)为可能的bvFTD符合诊断标准。其中,3有重要功能下降和没有神经成像结果与bvFTD一致。顺行性记忆障碍是常见的(n = 11),和患者提出语言赤字(n = 3)和困难与空间导航(n = 3)。的12个患者额叶症状,只有5面部与臂的矛盾的发作。
结论:anti-LGI1脑炎患者可以表现出正面的认知行为症状除了记忆障碍,临床评估和筛查这些特性可能有助于确定诊断。医生还应该考虑anti-LGI1 bvFTD脑炎的鉴别诊断。
披露:李医生没有披露。Climans博士没有披露。天已收到博士个人薪酬在5000 - 9999美元的范围为Parabon Nanolabs担任顾问。天已收到博士个人薪酬制度在5000 - 9999美元的范围为礼来公司担任顾问。天已收到博士个人薪酬在500 - 4999美元的范围作为一个编辑,副主编,或编辑顾问委员会成员力学(EBSCO健康)。天已收到博士个人薪酬在10000 - 49999美元的范围为巴罗法作为专家证人。天博士ANI制药公司的股票。天已收到博士研究机构国立卫生研究院/ NIA的支持。天已收到博士研究的机构支持陈扎克伯格倡议。天已收到博士研究的机构阿尔茨海默氏症协会的支持。 The institution of Dr. Day has received research support from National Institutes of Health / NINDS. The institution of Dr. Day has received research support from Horizon Therapeutics. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Presenter at Annual Meeting (CME) with American Academy of Neurology. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Content Development (CME) with PeerView, Inc. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Content Development (CME) with Continuing Education, Inc. Dr. Day has a non-compensated relationship as a Clinical Director with AntiNMDA Receptor Encephalitis Foundation that is relevant to AAN interests or activities. Dr. Hebert has nothing to disclose. Dr. Lapointe has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Ramos has nothing to disclose. The institution of Dr. Steriade has received research support from NIH. The institution of Dr. Steriade has received research support from Dorris Duke Fund to Retain Clinician Scientists. Dr. Wennberg has nothing to disclose. Dr. Muccilli has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Muccilli has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Muccilli has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Tang-Wai has nothing to disclose.
