Pathologically proven frontotemporal dementia presenting with severe amnesia
Pathologically proven frontotemporal dementia presenting with severe amnesia
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DOI：
10.1093/brain/awh348
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发表时间：
2005-03-01
期刊：
影响因子：
14.5
通讯作者：
Hodges, J
中科院分区：
文献类型：
--
作者：
Graham, A;Davies, R;Hodges, J
早期和严重的记忆障碍通常被认为是额颞叶痴呆（FTD）临床诊断的排除标准。然而，临床经验表明，一些典型FTD患者可能从就诊时就出现遗忘症，甚至仅表现为遗忘症。因此，有必要对经病理证实的FTD患者的重度遗忘症进行审查。本研究检查了剑桥-悉尼联合神经病理学系列痴呆患者和病理学诊断为FTD的所有患者的记录，以确定那些在就诊时记忆主诉占主导地位的患者。71例患者中有8例符合这些标准。对于两名患者，记忆丧失是唯一的投诉;对于一名患者，记忆丧失伴随着人格改变;对于两名患者，记忆丧失伴随着突出的执行障碍症状;对于三名患者，记忆丧失伴随着冷漠，但没有其他行为变化。在7名患者中，当地专家小组最初诊断为阿尔茨海默病; 4名患者进入抗胆碱酯酶药物试验。所有8例患者后来都出现了行为特征：4例患者的诊断修改为FTD，而4例患者的FTD诊断仅在死亡后的神经病理学检查中做出。总之，FTD患者出现重度遗忘症的情况比以前认为的更常见，可能需要修订FTD诊断的临床共识标准。FTD患者记忆障碍的潜在基础可能是异质性的，不同亚组的解释不同。
Early and severe memory impairment is generally held to be an exclusion criterion for the clinical diagnosis of frontotemporal dementia (FTD). However, clinical experience suggests that some patients with otherwise typical FTD can be amnesic from presentation, or even present solely with amnesia. A review of severe amnesia at presentation in patients with pathologically proven FTD is therefore warranted. The present study examined the records of all patients in the joint Cambridge-Sydney neuropathological series of patients with dementia and a pathological diagnosis of FTD to identify those for whom memory complaints were dominant at presentation. Eight of 71 patients met these criteria. For two patients, memory loss was the only complaint; for one patient, memory loss was accompanied by personality change; for two patients, memory loss was accompanied by prominent dysexecutive symptoms; and for three patients, memory loss was accompanied by apathy but no other behavioural changes. In seven patients local specialist teams initially diagnosed Alzheimer's disease; four patients entered anticholinesterase drug trials. All eight later developed behavioural features: in four, the diagnosis was revised to FTD, while in four the diagnosis of FTD was made only on neuropathological examination after death. In conclusion, severe amnesia at presentation in FTD is commoner than previously thought and the clinical consensus criteria for the diagnosis of FTD may need to be revised. The underlying basis of the memory impairments in patients with FTD may be heterogeneous, with different explanations in different subgroups.