Clinical, neuroimaging, and neuropathological characterization of a patient with Alzheimer's disease syndrome due to Pick's pathology.
Clinical, neuroimaging, and neuropathological characterization of a patient with Alzheimer's disease syndrome due to Pick's pathology.
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DOI：
10.1080/13554794.2021.1936072
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发表时间：
2022-03
期刊：
影响因子：
0.8
通讯作者：
Grinberg, Lea T.
中科院分区：
文献类型：
--
作者：
Toller, Gianina;Zitser, Jennifer;Sukhanov, Paul;Grant, Harli;Miller, Bruce L.;Kramer, Joel H.;Rosen, Howard J.;Rankin, Katherine P.;Grinberg, Lea T.
匹克病（PiD）是一种额颞叶变性，与之相关的最常见的神经退行性综合征是行为变异型额颞叶痴呆（bvFTD），其特征是社会行为发生显著变化。然而，在极少数情况下，匹克病可表现为以情景记忆早期受损为特征的阿尔茨海默病型痴呆。我们描述了一名患有匹克病所致晚发性阿尔茨海默病型痴呆患者的纵向认知和社会情感变化，以及结构影像学和神经病理学发现。该患者表现出缓慢进展的情景记忆障碍，随后出现执行功能障碍和社会行为改变，但视觉空间、语言和运动功能保留。与这种临床特征相一致，灰质萎缩和潜在的匹克病主要见于突显网络和语义评估网络的颞叶前部和额叶区域，而与语言相关的颞叶结构以及参与视觉空间功能的顶叶区域相对保留。我们从这名有记忆问题的患者身上得到的发现表明，早期类似行为变异型额颞叶痴呆的社会症状，如同理心缺失和去抑制，以及疾病进展缓慢和运动症状少，是提示像匹克病这样的额颞叶变性病理的关键临床特征。本病例报告可能有助于提高临床医生和临床研究人员预测非典型阿尔茨海默病患者病理的能力，这对于将患者纳入针对潜在蛋白质病的疾病修饰临床试验最终至关重要。
The most common neurodegenerative syndrome associated with Pick’s disease pathology (PiD), a type of frontotemporal lobar degeneration, is behavioral variant frontotemporal dementia (bvFTD), which is characterized by profound changes in social behavior. However, in rare cases, PiD can manifest as an Alzheimer’s disease-type dementia featuring early impairment of episodic memory. We describe the longitudinal cognitive and socioemotional changes as well as the structural imaging and neuropathological findings of a patient who presented with late-onset AD-type dementia with PiD. The patient showed slowly progressive episodic memory impairment, followed by executive dysfunctions and changes in social behavior, but preserved visuospatial, language, and motor functions. Consistent with this clinical profile, gray matter atrophy and underlying PiD were most predominantly found in anterior temporal and frontal lobe regions of the salience and semantic-appraisal networks, with relative sparing of language-related temporal lobe structures and parietal areas involved in visuospatial functioning. Our findings from this patient presenting with memory complaints suggest that early bvFTD-like social symptoms such as loss of empathy and disinhibition as well as slow disease progression and paucity of motor symptoms are key clinical features suggestive of a frontotemporal lobar degeneration pathology like PiD. This case report may help improve the ability of clinicians and clinical researchers to predict the pathology of patients with atypical presentations of AD, which will ultimately be critical for enrollment of patients into disease-modifying clinical trials targeting the underlying proteinopathy.
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影响因子：
14.5
作者：
Graham, A;Davies, R;Hodges, J
通讯作者：
Hodges, J
影响因子：
16.2
作者：
Andrews-Hanna, Jessica R.;Reidler, Jay S.;Sepulcre, Jorge;Poulin, Renee;Buckner, Randy L.
通讯作者：
Buckner, Randy L.
影响因子：
11.2
作者：
Hodges, JR;Davies, RR;Halliday, GM
通讯作者：
Halliday, GM
影响因子：
9.9
作者：
ESLINGER, PJ;DAMASIO, AR
通讯作者：
DAMASIO, AR
影响因子：
6
作者：
Choudhury, Parichita;Scharf, Eugene L.;Boeve, Bradley F.
通讯作者：
Boeve, Bradley F.