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短暂性癫痫性遗忘综合征。

The syndrome of transient epileptic amnesia.

作者信息

Butler Christopher R, Graham Kim S, Hodges John R, Kapur Narinder, Wardlaw Joanna M, Zeman Adam Z J

机构信息

Division of Clinical Neurosciences, Western General Hospital, Edinburgh, United Kingdom.

出版信息

Ann Neurol. 2007 Jun;61(6):587-98. doi: 10.1002/ana.21111.

DOI:10.1002/ana.21111
PMID:17444534
Abstract

OBJECTIVE

Transient amnesia can be the principal manifestation of epilepsy. This diagnosis, however, is seldom suspected by clinicians and remains controversial. The amnestic attacks are often associated with persistent memory complaints. This study was designed to provide the first description of transient epileptic amnesia in a substantial series of patients.

METHODS

Fifty patients were recruited over 18 months using the following diagnostic criteria: (1) recurrent, witnessed episodes of amnesia; (2) other cognitive functions intact during attacks; and (3) compelling evidence of epilepsy. We assessed clinical features and performed neuropsychological evaluation in cases and 24 matched control subjects.

RESULTS

Transient epileptic amnesia develops in later life (mean onset, 62 years). Amnestic episodes are frequent (median, 12/year), brief (median duration, 30-60 minutes), and often occur on waking (37/50 cases). Epilepsy was the initial specialist diagnosis in only 12 of 50 cases. Attacks ceased on anticonvulsant medication in 44 of 47 treated patients. A total of 40 of 50 cases described persistent memory difficulties. Despite normal performance on standard memory tests, patients exhibited accelerated forgetting of verbal and visual material over 3 weeks by comparison with matched control subjects (p < 0.001). They also showed loss of autobiographical memories for events extending back over 40 years (p < 0.05).

INTERPRETATION

We propose that transient epileptic amnesia is a distinctive epilepsy syndrome, typically misdiagnosed at presentation and associated with accelerated long-term forgetting and autobiographical amnesia. The syndrome is of clinical and theoretic importance.

摘要

目的

短暂性失忆可能是癫痫的主要表现。然而,临床医生很少怀疑这种诊断,且该诊断仍存在争议。失忆发作常伴有持续性记忆障碍。本研究旨在对大量患者中的短暂性癫痫性失忆进行首次描述。

方法

在18个月内招募了50名患者,采用以下诊断标准:(1)反复出现、有目击者见证的失忆发作;(2)发作期间其他认知功能完好;(3)有确凿的癫痫证据。我们评估了临床特征,并对病例组和24名匹配的对照组受试者进行了神经心理学评估。

结果

短暂性癫痫性失忆在晚年发病(平均发病年龄62岁)。失忆发作频繁(中位数为每年12次),持续时间短(中位数持续时间为30 - 60分钟),且常发生在醒来时(50例中有37例)。在50例病例中,仅有12例最初由专科医生诊断为癫痫。47例接受治疗的患者中,44例在服用抗惊厥药物后发作停止。50例病例中有40例描述存在持续性记忆困难。尽管在标准记忆测试中表现正常,但与匹配的对照组相比,患者在3周内对言语和视觉材料的遗忘速度加快(p < 0.001)。他们还表现出对40多年前事件的自传体记忆丧失(p < 0.05)。

解读

我们认为短暂性癫痫性失忆是一种独特的癫痫综合征,通常在就诊时被误诊,且与长期遗忘加速和自传体失忆有关。该综合征具有临床和理论重要性。

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