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自身免疫性脑炎致慢性颞叶癫痫的发病机制。

Factors underlying the development of chronic temporal lobe epilepsy in autoimmune encephalitis.

机构信息

IRCCS "NEUROMED", Pozzilli (IS), Italy.

Epilepsy Unit, Department of Neurosciences/Mental Health, "Sapienza" University, Rome, Italy.

出版信息

J Neurol Sci. 2019 Jan 15;396:102-107. doi: 10.1016/j.jns.2018.10.026. Epub 2018 Oct 30.

DOI:10.1016/j.jns.2018.10.026
PMID:30447604
Abstract

PURPOSE

Limbic encephalitis (LE) is an autoimmune condition characterized by amnestic syndrome, psychiatric features and seizures. Early diagnosis and prompt treatment are crucial to avoid long-term sequelae, including psycho-cognitive deficits and persisting seizures. The aim of our study was to analyze the characteristics of 33 LE patients in order to identify possible prognostic factors associated with the development of chronic epilepsy.

METHODS

This is a retrospective cohort study including adult patients diagnosed with LE in the period 2010-2017 and followed up for ≥12 months. Demographics, seizure semiology, EEG pattern, MRI features, CSF/serum findings were reviewed.

RESULTS

All 33 LE patients (19 M/14F, mean age 61.2 years) presented seizures. Thirty subjects had memory deficits; 22 presented behavioural/mood disorders. Serum and/or CSF auto-antibodies were detected in 12 patients. In 31 subjects brain MRI at onset showed typical alterations involving temporal lobes. All patients received immunotherapy. At follow-up, 13/33 had developed chronic epilepsy; predisposing factors included delay in diagnosis (p = .009), low seizure frequency at onset (p = .02), absence of amnestic syndrome (p = .02) and absence/rarity of inter-ictal epileptic discharges on EEG (p = .06).

CONCLUSIONS

LE with paucisymptomatic electro-clinical presentation seemed to be associated to chronic epilepsy more than LE presenting with definite and severe "limbic syndrome".

摘要

目的

边缘性脑炎(LE)是一种自身免疫性疾病,其特征为健忘综合征、精神特征和癫痫发作。早期诊断和及时治疗对于避免长期后遗症至关重要,包括心理认知缺陷和持续癫痫发作。我们的研究目的是分析 33 例 LE 患者的特征,以确定与慢性癫痫发生相关的可能预后因素。

方法

这是一项回顾性队列研究,纳入了 2010 年至 2017 年间诊断为 LE 并随访时间≥12 个月的成年患者。回顾了患者的人口统计学、癫痫发作半侧特征、脑电图模式、MRI 特征、CSF/血清发现。

结果

33 例 LE 患者(19 例男性/14 例女性,平均年龄 61.2 岁)均出现癫痫发作。30 例患者存在记忆障碍;22 例患者存在行为/情绪障碍。12 例患者血清和/或 CSF 自身抗体检测呈阳性。31 例患者在发病时脑 MRI 显示出涉及颞叶的典型改变。所有患者均接受免疫治疗。随访时,13/33 例患者发展为慢性癫痫;易患因素包括诊断延迟(p=0.009)、发病时癫痫发作频率低(p=0.02)、无健忘综合征(p=0.02)和 EEG 上无/罕见间发性癫痫放电(p=0.06)。

结论

与表现明确且严重的“边缘综合征”相比,电临床表现较少的 LE 似乎与慢性癫痫的相关性更大。

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