Anti-LGI1 encephalitis

脑炎 医学 高强度 边缘脑炎 儿科 失忆症 自身免疫性脑炎 神经心理学 内科学 认知 磁共振成像 放射科 精神科 免疫学 病毒
作者
Agnes van Sonderen,Roland D. Thijs,Elias C. Coenders,Lize C. Jiskoot,Esther Sánchez,Marienke A.A.M. de Bruijn,Marleen H. van Coevorden‐Hameete,Paul W. Wirtz,Marco W.J. Schreurs,Peter A.E. Sillevis Smitt,Maarten J. Titulaer
出处
期刊:Neurology [Ovid Technologies (Wolters Kluwer)]
卷期号:87 (14): 1449-1456 被引量:466
标识
DOI:10.1212/wnl.0000000000003173
摘要

This nationwide study gives a detailed description of the clinical features and long-term outcome of anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis.We collected patients prospectively from October 2013, and retrospectively from samples sent to our laboratory from January 2007. LGI1 antibodies were confirmed with both cell-based assay and immunohistochemistry. Clinical information was obtained in interviews with patients and their relatives and from medical records. Initial MRI and follow-up MRI were revised blindly. Neuropsychological assessment was performed in those patients with follow-up over 2 years.Annual incidence in the Netherlands was 0.83/million. A total of 34/38 patients had a limbic encephalitis. Subtle focal seizures (66%, autonomic or dyscognitive) and faciobrachial dystonic seizures (FBDS, 47%) mostly occurred before onset of memory disturbance. Later in the disease course, 63% had tonic-clonic seizures. Initial MRI showed hippocampal T2 hyperintensity in 74% of the patients. These lesions evolved regularly into mesial temporal sclerosis (44%). Substantial response to immunotherapy was seen in 80%, with early response of seizures and slow recovery of cognition. At follow-up ≥2 years, most surviving patients reported mild residual cognitive deficit with spatial disorientation. A total of 86% had persistent amnesia for the disease period. Relapses were common (35%) and presented up to 8 years after initial disease. Two-year case fatality rate was 19%.Anti-LGI1 encephalitis is a homogenous clinical syndrome, showing early FBDS and other focal seizures with subtle clinical manifestations, followed by memory disturbances. Better recognition will lead to earlier diagnosis, essential for prompt start of treatment. Long-term outcome of surviving patients is mostly favorable, but relapses are common.
最长约 10秒,即可获得该文献文件

科研通智能强力驱动
Strongly Powered by AbleSci AI
更新
大幅提高文件上传限制,最高150M (2024-4-1)

科研通是完全免费的文献互助平台,具备全网最快的应助速度,最高的求助完成率。 对每一个文献求助,科研通都将尽心尽力,给求助人一个满意的交代。
实时播报
dominate应助ljzzj123456采纳,获得10
3秒前
内向大碗完成签到 ,获得积分10
3秒前
asw发布了新的文献求助10
3秒前
3秒前
6秒前
别潜然完成签到,获得积分10
6秒前
7秒前
7秒前
lin发布了新的文献求助10
9秒前
大个应助苹果傲南采纳,获得10
10秒前
Hello应助Ann采纳,获得10
10秒前
10秒前
11秒前
11秒前
13秒前
young完成签到,获得积分10
13秒前
13秒前
百里酚蓝完成签到 ,获得积分10
14秒前
xiao发布了新的文献求助10
14秒前
hydrangea应助赤侠采纳,获得30
15秒前
bryceeluo应助e厘米采纳,获得10
15秒前
ff完成签到 ,获得积分10
16秒前
完美世界应助大胆迎梅采纳,获得10
16秒前
16秒前
16秒前
单薄碧灵完成签到 ,获得积分10
18秒前
天晴完成签到,获得积分20
18秒前
一一应助余杭村王小虎采纳,获得20
19秒前
xiao完成签到,获得积分10
20秒前
科研通AI2S应助onetec采纳,获得10
21秒前
21秒前
22秒前
leftarrow完成签到,获得积分10
22秒前
23秒前
涂楚捷发布了新的文献求助10
23秒前
yilin发布了新的文献求助10
23秒前
跳跃醉蝶完成签到,获得积分10
25秒前
单于思雁发布了新的文献求助30
26秒前
pp应助自然的箴采纳,获得10
26秒前
李健的小迷弟应助MUAL采纳,获得10
26秒前
高分求助中
The ACS Guide to Scholarly Communication 2500
Microlepidoptera Palaearctica, Volumes 1 and 3 - 13 (12-Volume Set) [German] 1122
PraxisRatgeber Mantiden., faszinierende Lauerjäger. – Buch gebraucht kaufen 700
Mantiden - Faszinierende Lauerjäger – Buch gebraucht kaufen 700
Ожившие листья и блуждающие цветы. Практическое руководство по содержанию богомолов [Alive leaves and wandering flowers. A practical guide for keeping praying mantises] 500
Development of a new synthetic process for the synthesis of (S)-methadone and (S)- and (R)-isomethadone as NMDA receptor antagonists for the treatment of depression 500
A Dissection Guide & Atlas to the Rabbit 500
热门求助领域 (近24小时)
化学 医学 生物 材料科学 工程类 有机化学 生物化学 物理 内科学 纳米技术 计算机科学 化学工程 复合材料 基因 遗传学 催化作用 物理化学 免疫学 量子力学 细胞生物学
热门帖子
关注 科研通微信公众号,转发送积分 3093589
求助须知:如何正确求助?哪些是违规求助? 2745564
关于积分的说明 7586157
捐赠科研通 2396871
什么是DOI,文献DOI怎么找? 1271459
科研通“疑难数据库(出版商)”最低求助积分说明 615172
版权声明 598844