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进行性双侧顶叶萎缩:阿尔茨海默病的一种非典型表现。

Progressive biparietal atrophy: an atypical presentation of Alzheimer's disease.

作者信息

Ross S J, Graham N, Stuart-Green L, Prins M, Xuereb J, Patterson K, Hodges J R

机构信息

University Neurology Unit, University of Cambridge, UK.

出版信息

J Neurol Neurosurg Psychiatry. 1996 Oct;61(4):388-95. doi: 10.1136/jnnp.61.4.388.

DOI:10.1136/jnnp.61.4.388
PMID:8890778
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC486580/
Abstract

OBJECTIVES

To define the clinical, neuropsychological, and radiological features of bilateral parietal lobe atrophy.

METHODS

Four patients underwent a comprehensive longitudinal neuropsychological assessment, as well as MRI and HMPAO-SPECT.

RESULTS

The consistent findings in the patients were early visuospatial problems, agraphia of a predominantly peripheral (or apraxic) type, and difficulty with bimanual tasks, all of which outweighted deficits in memory and language until later in the course of the illness. As the disease progressed, impairments in the phonological aspects of language and in auditory-verbal short term memory were often striking, perhaps reflecting spread from the parietal lobe to perisylvian language areas. Three patients went on to develop a global dementia and fulfilled the criteria for a clinical diagnosis of probable Alzheimer's disease; the fourth patient has only recently been identified. Neuroimaging disclosed bilateral parietal lobe atrophy (MRI) and hypoperfusion (SPECT), which was out of proportion to that seen elsewhere in the brain. One patient has died and had pathologically confirmed Alzheimer's disease with particular concentration in both superior parietal lobes.

CONCLUSIONS

Bilateral biparietal atrophy is a recognisable clinical syndrome which can be the presenting feature of Alzheimer's disease. Although the label "posterior cortical atrophy" has been applied to such cases, review of the medical literature suggests that this broad rubric actually consists of two main clinical syndromes with features reflecting involvement of the occipitotemporal (ventral) and biparietal (dorsal) cortical areas respectively.

摘要

目的

明确双侧顶叶萎缩的临床、神经心理学及影像学特征。

方法

对4例患者进行了全面的纵向神经心理学评估,以及磁共振成像(MRI)和锝-99m六甲基丙二胺肟单光子发射计算机断层扫描(HMPAO-SPECT)。

结果

患者一致的表现为早期视觉空间问题、主要为外周型(或失用型)的失写症以及双手任务困难,在疾病后期之前,所有这些表现均比记忆和语言缺陷更为突出。随着疾病进展,语言的语音方面及听觉言语短期记忆损害常常很明显,这可能反映了病变从顶叶扩散至颞周语言区。3例患者继而发展为全面性痴呆,并符合可能的阿尔茨海默病临床诊断标准;第4例患者最近才被确诊。神经影像学显示双侧顶叶萎缩(MRI)和灌注不足(SPECT),其程度超过大脑其他部位所见。1例患者已死亡,病理证实为阿尔茨海默病,病变尤其集中在双侧顶上叶。

结论

双侧顶叶萎缩是一种可识别的临床综合征,可为阿尔茨海默病的首发特征。尽管“后部皮质萎缩”这一术语已用于此类病例,但医学文献回顾表明,这一宽泛的类别实际上由两种主要临床综合征组成,其特征分别反映枕颞叶(腹侧)和双侧顶叶(背侧)皮质区域受累。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4284/486580/83015838bc82/jnnpsyc00010-0066-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4284/486580/83015838bc82/jnnpsyc00010-0066-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4284/486580/83015838bc82/jnnpsyc00010-0066-a.jpg

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