Nonfluent/agrammatic PPA with in-vivo cortical amyloidosis and Pick's disease pathology.
Nonfluent/agrammatic PPA with in-vivo cortical amyloidosis and Pick's disease pathology.
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DOI：
10.3233/ben-2012-120255
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发表时间：
2013
影响因子：
2.8
通讯作者：
Gorno-Tempini ML
中科院分区：
文献类型：
--
作者：
Caso F;Gesierich B;Henry M;Sidhu M;LaMarre A;Babiak M;Miller BL;Rabinovici GD;Huang EJ;Magnani G;Filippi M;Comi G;Seeley WW;Gorno-Tempini ML
生物标志物在预测额颞叶痴呆（FTD）临床谱系障碍的病理表现方面的作用仍在探索中。我们提供了一名66岁右利手女性的全面、前瞻性纵向数据，该患者符合原发性进行性失语的非流利/语法缺失变异型（nfvPPA）的现行标准。她最初有3年进行性言语和语言障碍病史，主要表现为严重的言语失用。在整个临床病程中，神经心理学和一般运动功能相对保留。基于体素的形态测量学（VBM）显示左侧额下回后部（IFG）和其下方的脑岛选择性皮质萎缩，且随时间推移加重，沿左侧运动前区延伸。在首次评估5年后，她出现轻度记忆障碍，并接受了正电子发射断层扫描 - 氟脱氧葡萄糖（PET - FDG）和匹兹堡复合物B（PiB）扫描，结果显示左侧额叶代谢减低和皮质淀粉样变。3年后（从首次出现症状起11年），尸检组织病理学评估显示为皮克病，伴有左侧额下回、脑岛中部和中央前回严重变性。还检测到阿尔茨海默病（AD）（CERAD常见/布拉克分期V期）。该患者表明，提示脑淀粉样变的生物标志物不应被视为AD病理可解释典型FTD临床/解剖综合征的确凿证据。
The role of biomarkers in predicting pathological findings in the frontotemporal dementia (FTD) clinical spectrum disorders is still being explored. We present comprehensive, prospective longitudinal data for a 66 year old, right-handed female who met current criteria for the nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA). She first presented with a 3-year history of progressive speech and language impairment mainly characterized by severe apraxia of speech. Neuropsychological and general motor functions remained relatively spared throughout the clinical course. Voxel-based morphometry (VBM) showed selective cortical atrophy of the left posterior inferior frontal gyrus (IFG) and underlying insula that worsened over time, extending along the left premotor strip. Five years after her first evaluation, she developed mild memory impairment and underwent PET-FDG and PiB scans that showed left frontal hypometabolism and cortical amyloidosis. Three years later (11 years from first symptom), post-mortem histopathological evaluation revealed Pick's disease, with severe degeneration of left IFG, mid-insula, and precentral gyrus. Alzheimer’s disease (AD) (CERAD frequent/Braak Stage V) was also detected. This patient demonstrates that biomarkers indicating brain amyloidosis should not be considered conclusive evidence that AD pathology accounts for a typical FTD clinical/anatomical syndrome.