Zeman A Z, Boniface S J, Hodges J R
University of Cambridge Neurology Unit, Addenbrooke's Hospital, UK.
J Neurol Neurosurg Psychiatry. 1998 Apr;64(4):435-43. doi: 10.1136/jnnp.64.4.435.
To clarify the clinical and neuropsychological aspects of transient epileptic amnesia (TEA) based on 10 personally studied cases as well as review of 21 previously published cases; and to propose tentative diagnostic criteria for the diagnosis of TEA.
All 10 patients and informants underwent a standardised clinical interview. The radiological and neurophysiological (EEG) data were also reviewed in all cases. The diagnosis of transient epileptic amnesia was made on the basis of the following criteria: (1) there was a history of recurrent witnessed episodes of transient amnesia; (2) cognitive functions other than memory were judged to be intact during typical episodes by a reliable witness; (3) there was evidence for a diagnosis of epilepsy. This evidence was provided by either (a) wake or sleep EEG, or (b) the co-occurrence of other seizure types (if their roughly concurrent onset or close association with episodes of transient amnesia suggested a connection), or (c) a clear cut response to anticonvulsant therapy, or by a combination of these three factors. In addition all patients were administered a comprehensive neuropsychological test battery designed to assess verbal and non-verbal anterograde memory and retrograde memory for famous personalities and personal events. Their results were compared with those of 25 age and IQ matched normal controls.
TEA usually begins in later life, with a mean age of 65 years in this series. Episodes are typically brief, lasting less than one hour, and recurrent, with a mean frequency of three a year. Attacks on waking are characteristic. Repetitive questioning occurs commonly during attacks. The anterograde amnesia during episodes is, however, often incomplete so that patients may later be able to "remember not being able to remember". The extent of the retrograde amnesia during attacks varies from days to years. Most patients experience other seizure types compatible with an origin in the temporal lobes, but transient amnesia is the only manifestation of epilepsy in about one third of patients. Epileptiform abnormalities arising from the temporal lobes are most often detected on interictal sleep EEG. Despite normal performance on tests of anterograde memory, many patients complain of persistent interictal disturbance of autobiographical memory, involving a significant but variable loss of recall for salient personal episodes. The epochs affected may predate the onset of epilepsy by many years.
TEA is an identifiable syndrome and comprises episodic transient amnesia with an epileptic basis, without impairment of other aspects of cognitive function. Future studies should consider the question of whether TEA reflects ictal activity or a postictal state, and the mechanism of the persistent autobiographical amnesia. It is hypothesised that the latter may result in part from impairment of very long term memory consolidation as a result of epileptic activity in mesial temporal structures.
基于10例个人研究病例以及对21例既往发表病例的回顾,阐明短暂性癫痫性遗忘(TEA)的临床和神经心理学特征;并提出TEA诊断的初步标准。
所有10例患者及 informant 均接受了标准化临床访谈。所有病例均回顾了放射学和神经生理学(脑电图)数据。短暂性癫痫性遗忘的诊断基于以下标准:(1)有反复发作的短暂性遗忘发作史;(2)可靠证人判断在典型发作期间除记忆外的认知功能完好;(3)有癫痫诊断的证据。该证据由以下之一提供:(a)清醒或睡眠脑电图,或(b)其他发作类型的同时出现(如果它们大致同时发作或与短暂性遗忘发作密切相关提示有联系),或(c)对抗惊厥治疗的明确反应,或这三个因素的组合。此外,所有患者均接受了一套全面的神经心理学测试,旨在评估对名人及个人事件的言语和非言语顺行性记忆及逆行性记忆。将他们的结果与25名年龄和智商匹配的正常对照者的结果进行比较。
TEA通常在晚年发病,本系列病例的平均年龄为65岁。发作通常短暂,持续时间少于1小时,且反复发作,平均每年发作3次。醒时发作具有特征性。发作期间经常出现反复提问。然而,发作期间的顺行性遗忘往往不完全,因此患者后来可能能够“记得自己记不住”。发作期间逆行性遗忘的程度从数天到数年不等。大多数患者经历与颞叶起源相符的其他发作类型,但约三分之一的患者中短暂性遗忘是癫痫的唯一表现。颞叶起源的癫痫样异常最常在发作间期睡眠脑电图中检测到。尽管顺行性记忆测试表现正常,但许多患者抱怨发作间期自传体记忆持续受到干扰,包括对突出个人事件的回忆明显但程度不一的丧失。受影响的时期可能在癫痫发作开始前许多年就已存在。
TEA是一种可识别的综合征,包括具有癫痫基础的发作性短暂性遗忘,且认知功能的其他方面无损害。未来的研究应考虑TEA反映发作期活动还是发作后期状态的问题,以及持续性自传体遗忘的机制。据推测,后者可能部分是由于内侧颞叶结构中的癫痫活动导致极长期记忆巩固受损所致。