Clinical and neuropathological characteristics of hippocampal sclerosis - A community-based study

Clinical and neuropathological characteristics of hippocampal sclerosis - A community-based study
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DOI:
10.1001/archneur.59.7.1099
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发表时间:
2002-07-01
影响因子:
--
通讯作者:
Larson, EB
Larson, EB
中科院分区:
其他
文献类型:
--
作者:
Leverenz, JB;Agustin, CM;Larson, EB

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背景资料:海马硬化症(HS)是一种神经病理学发现,其特征在于海马CA-1和下托中的神经元丢失和神经胶质增生。以往的研究表明,这是一个常见的尸检发现,在老年人痴呆症。然而,这些研究是从选定的样本,因此不一定代表患者看到在一般的medical community.Objectives:检查HS的临床和病理特征,在社区为基础的病例系列痴呆症,并比较这些特点与观察对象阿尔茨海默病(AD)从同一研究样本。134例尸检病例来自社区痴呆症登记处。16例(12%)尸检诊断为HS。从相同的文件中选择了32例神经病理诊断为AD的对照病例。对每例HS的神经病理学特征进行了审查,包括严重程度、分布和其他病理过程。对HS和对照组的临床特征进行了盲法审查,以评估风险factors.Results:HS病变的严重程度和分布范围广泛,病例之间的病变严重程度和年龄在个别病例中存在很大差异。系列神经心理和行为评估显示HS和AD组之间的痴呆进展的临床特征和速率相似。在入组时进行的所有神经心理学测试中,只有路径A的增强表现区分了HS和AD组(64秒,0个错误vs 114秒,0.6个错误; P小于或等于0.05)。AD患者中至少携带1个载脂蛋白ε 4等位基因的比例显著高于HS患者(61%vs31%,χ 2 =3.81,P <0.05)。虽然病历审查表明,较高的频率,临床中风和神经放射学白色物质异常的HS组,血管疾病的危险因素和脑血管疾病的神经病理学证据之间并没有显着差异groups.Conclusions:我们的研究结果表明,HS是一个常见的病理发现在社区为基础的痴呆症。患有HS的个体与患有AD的个体具有相似的初始症状和痴呆进展率,因此经常被误分类为患有AD。我们的临床和病理结果表明,HS具有进行性疾病的特点,但根本原因仍然难以捉摸。
Background: Hippocampal sclerosis (HS) is a neuropathologic finding characterized by neuronal loss and gliosis in the CA-1 and subiculum of the hippocampus. Previous studies of HS have shown that this is a common postmortem finding in elderly subjects with dementia. However, these studies were from selected samples and therefore are not necessarily representative of patients seen in the general medical community.Objectives: To examine the clinical and pathologic characteristics of HS in a community-based case series of dementia and to compare these characteristics with those observed in subjects with Alzheimer disease (AD) from the same study sample.Methods: One hundred thirty-four autopsy cases were available from a community-based registry of dementia. Sixteen cases (12%) had a postmortem diagnosis of HS. Thirty-two comparison control cases with a neuropathologic diagnosis of AD were selected from the same files. Each case of HS was reviewed for HS neuropathologic features, including severity, distribution, and additional pathologic processes. Blinded review of clinical characteristics for the HS and control groups was performed to assess risk factors.Results: There was a wide range of severity and distribution of HS lesions between cases and substantial variability in lesion severity and age within individual cases. Serial neuropsychologic and behavioral assessments revealed similar clinical features and rates of dementia progression between HS and AD groups. Of all neuropsychologic tests performed at enrollment, only enhanced performance on Trails A differentiated the HS from the AD group (64 seconds, 0 errors vs 114 seconds, 0.6 errors; Pless than or equal to.05). The number of AD cases with at least 1 apolipoprotein epsilon4 allele was significantly greater than the HS cases (61% vs 31%; chi(2)=3.81, Pless than or equal to.05). Although medical record review indicated higher frequencies of clinical stroke and neuroradiologic white matter abnormalities in the HS group, risk factors for vascular disease and neuropathologic evidence of cerebrovascular disease did not differ between the groups.Conclusions: Our results suggest that HS is a frequent pathologic finding in community-based dementia. Individuals with HS have similar initial symptoms and rates of dementia progression to those with AD and therefore are frequently misclassified as having AD. Our clinical and pathologic findings suggest that HS has characteristics of a progressive disorder although the underlying cause remains elusive.