Clinicopathological correlates in frontotemporal dementia
Clinicopathological correlates in frontotemporal dementia
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DOI：
10.1002/ana.20203
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发表时间：
2004-09-01
影响因子：
11.2
通讯作者：
Halliday, GM
中科院分区：
文献类型：
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作者：
Hodges, JR;Davies, RR;Halliday, GM
额颞叶痴呆 (FTD) 一词涵盖了一系列临床综合征，据信这些综合征不能可靠地映射到公认的病理学谱上。本研究通过两个中心的大型系列研究（n = 61）重新审视了 FTD 临床和病理亚型之间的关系。定义的临床亚型包括行为变异 FTD (n = 26)、语言变异（语义痴呆，n = 9；进行性非流利性失语，n = 8）和运动变异（皮质基底节变性，n = 9；和运动神经元疾病，n = 9），尽管大多数病例同时出现行为和语言问题。出乎意料的是，一些行为病例（n = 5）在就诊时表现出明显的健忘症。病理亚型包括具有 tau 免疫阳性包涵体的亚型（有 Pick 小体，n = 20；或没有，n = 11）、具有泛素免疫阳性包涵体的亚型（n = 16）和缺乏独特组织学的亚型（n = 14）。行为症状和语义痴呆与一系列病理相关。相比之下，其他临床表型具有相对一致的潜在病理学：运动神经元疾病预示着泛素化包涵体，帕金森病和失用症预示着皮质基底节病理学，非流利性失语症预示着皮克体。因此，可以预测很大一部分 FTD 患者的病理基础，这对于针对机械治疗的研究具有重要意义。
The term frontotemporal dementia (FTD) encompasses a range of clinical syndromes that are believed not to map reliably onto the spectrum of recognized pathologies. This study reexamines the relationships between clinical and pathological subtypes of FTD in a large series from two centers (n = 61). Clinical subtypes defined were behavioral variant FTD (n = 26), language variants (semantic dementia, n = 9; and progressive nonfluent aphasia, n = 8), and motor variants (corticobasal degeneration, n = 9; and motor neuron disease, n = 9), although most cases presented with a combination of behavioral and language problems. Unexpectedly, some behavioral cases (n = 5) had marked amnesia at presentation. The pathological subtypes were those with tau-immunopositive inclusions (with Pick bodies, n = 20; or without, n = 11), those with ubiquitin immunopositive inclusions (n = 16), and those lacking distinctive histology (n = 14). Behavioral symptoms and semantic dementia were associated with a range of pathologies. In contrast, other clinical phenotypes had relatively uniform underlying pathologies: motor neuron disease predicted ubiquitinated inclusions, parkinsonism and apraxia predicted corticobasal pathology, and nonfluent aphasia predicted Pick bodies. Therefore, the pathological substrate can be predicted in a significant proportion of FTD patients, which has important implications for studies targeting mechanistic treatments.