期刊文献+

重度遗忘性额颞痴呆的病理学证据

Pathologically proven frontotemporal dementia presenting with severe amnesia
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摘要 Early and severe memory impairment is generally held to be an exclusion criter ion for the clinical diagnosis of frontotemporal dementia (FTD). However, clinic al experience suggests that some patients with otherwise typical FTD can be amne sic from presentation, or even present solely with amnesia. A review of severe a mnesia at presentation in patients with pathologically proven FTD is therefore w arranted. The present study examined the records of all patients in the joint Ca mbridge-Sydney neuropathological series of patients with dementia and a patholo gical diagnosis of FTD to identify those for whom memory complaints were dominan t at presentation. Eight of 71 patients met these criteria. For two patients, me mory loss was the only complaint; for one patient, memory loss was accompanied b y personality change; for two patients, memory loss was accompanied by prominent dysexecutive symptoms; and for three patients, memory loss was accompanied by a pathy but no other behavioural changes. In seven patients local specialist teams initially diagnosed Alzheimer’s disease; four patients entered anticholinester ase drug trials. All eight later developed behavioural features: in four, the di agnosis was revised to FTD, while in four the diagnosis of FTD was made only on europathological examination after death. In conclusion, severe amnesia at prese ntation in FTD is commoner than previously thought and the clinical consensus cr iteria 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. Early and severe memory impairment is generally held to be an exclusion criter ion for the clinical diagnosis of frontotemporal dementia (FTD). However, clinic al experience suggests that some patients with otherwise typical FTD can be amne sic from presentation, or even present solely with amnesia. A review of severe a mnesia at presentation in patients with pathologically proven FTD is therefore w arranted. The present study examined the records of all patients in the joint Ca mbridge-Sydney neuropathological series of patients with dementia and a patholo gical diagnosis of FTD to identify those for whom memory complaints were dominan t at presentation. Eight of 71 patients met these criteria. For two patients, me mory loss was the only complaint; for one patient, memory loss was accompanied b y personality change; for two patients, memory loss was accompanied by prominent dysexecutive symptoms; and for three patients, memory loss was accompanied by a pathy but no other behavioural changes. In seven patients local specialist teams initially diagnosed Alzheimer's disease; four patients entered anticholinester ase drug trials. All eight later developed behavioural features: in four, the di agnosis was revised to FTD, while in four the diagnosis of FTD was made only on europathological examination after death. In conclusion, severe amnesia at prese ntation in FTD is commoner than previously thought and the clinical consensus cr iteria 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.
出处 《世界核心医学期刊文摘(神经病学分册)》 2005年第8期13-13,共1页 Digest of the World Core Medical Journals:Clinical Neurology
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