Frontotemporal lobar degeneration without lobar atrophy
Frontotemporal lobar degeneration without lobar atrophy
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DOI：
10.1001/archneur.63.11.1632
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发表时间：
2006-11-01
影响因子：
--
通讯作者：
Dickson, Dennis W.
中科院分区：
文献类型：
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作者：
Josephs, Keith A.;Whitwell, Jennifer L.;Dickson, Dennis W.
背景资料：额颞叶变性伴仅泛素免疫反应性神经元包涵体（FTLD-U）是额颞叶痴呆最常见的形式。海马硬化（HpScl）的特征是海马第1角和海马下托的神经元丢失和胶质增生，这在许多FTLD-U病例中发生。目的：确定FTLD-U中是否存在HpScl的任何临床或磁共振成像相关性。设计：我们回顾了24例FTLD-U患者的人口统计学和临床特征，并主观评估了神经元丢失的严重程度和泛素-在额叶和颞叶皮质和海马齿状回的阳性神经元病变。设置：马约诊所，罗切斯特，明尼苏达州。患者：26例病例被确定从医疗记录链接系统查询，符合临床标准，并有尸检材料可用于进一步的研究。在病理学研究显示同时存在阿尔茨海默病后，两例病例被排除在进一步分析之外，剩下24例病例纳入研究。根据是否存在HpScl.Main Outcome Measures的情况下进行细分的情况下：灰质萎缩的模式进行了评估，在FTLD-U的情况下，没有HpScl使用基于体素的morphometry.Results：6的24例FTLD-U没有HpScl。在有和没有HpScl的病例之间，在人口统计学或临床特征（包括疾病持续时间）方面没有发现差异;然而，基于体素的形态学分析显示，在没有HpScl的病例中，皮质萎缩最小，这与FTLD-U伴HpScl的中度至重度额叶和颞叶萎缩模式显著不同。这一发现是符合组织病理学observation.Conclusions：尽管相似的临床特征，FTLD-U与HpScl的情况下，从没有HpScl的病理结果和结构成像不同。具体而言，不含HpScl的FTLD-U显示平均最小或无皮质萎缩，即使在终末期疾病中。因此，没有HpScl的FTLD-U不符合所提出的FTLD分期方案，被低估，并且可能具有不同的遗传和环境基础。
Background: Frontotemporal lobar degeneration with ubiquitin-only-immunoreactive neuronal inclusions (FTLD-U) is the most common form of frontotemporal dementia. Neuronal loss and gliosis in cornu ammonis 1 and the subiculum of the hippocampus are features of hippocampal sclerosis (HpScl), which occurs in many cases of FTLD-U.Objective: To determine if there were any clinical or magnetic resonance imaging correlates of HpScl in FTLD-U.Design: We reviewed demographics and clinical features of 24 cases of FTLD-U and subjectively assessed the severity of neuronal loss and frequency of ubiquitin-positive neuronal lesions in the frontal and temporal cortices and the dentate gyrus of the hippocampus.Setting: Mayo Clinic, Rochester, Minn.Patients: Twenty-six cases were identified from the medical records linkage system query that met clinical criteria and had autopsy material available for additional studies. Two cases were excluded from further analysis after pathologic studies revealed coexisting Alzheimer disease, leaving 24 cases included in the study. Cases were subdivided based on the presence or absence of HpScl.Main Outcome Measures: Patterns of gray matter atrophy were assessed in cases of FTLD-U with and without HpScl using voxel-based morphometry.Results: Six of the 24 cases of FTLD-U did not have HpScl. No differences were found in demographic or clinical features, including disease duration, between cases with and without HpScl; however, voxel-based morphometry analysis revealed minimal cortical atrophy in cases without HpScl, which was significantly different from the pattern of moderate to severe frontal and temporal lobe atrophy in FTLD-U with HpScl. This finding was in keeping with histopathologic observations.Conclusions: Despite similar clinical features, cases of FTLD-U with HpScl differ from those without HpScl with respect to pathologic findings and structural imaging. Specifically, FTLD-U without HpScl showed on average minimal or no cortical atrophy, even at end-stage disease. Consequently, FTLD-U without HpScl does not conform to the proposed FTLD staging scheme, is underrecognized, and may have different genetic and environmental underpinnings.