Departments of Neurology, University of California, San Francisco, CA 94143-1207, USA.
Alzheimer Dis Assoc Disord. 2013 Jan-Mar;27(1):74-83. doi: 10.1097/WAD.0b013e31824a7df4.
Behavioral variant frontotemporal dementia and semantic dementia have been associated with striatal degeneration, but few studies have delineated striatal subregion volumes in vivo or related them to the clinical phenotype. We traced caudate, putamen, and nucleus accumbens on magnetic resonance images to quantify volumes of these structures in behavioral variant frontotemporal dementia, semantic dementia, Alzheimer disease, and healthy controls (n=12 per group). We further related these striatal volumes to clinical deficits and neuropathologic findings in a subset of patients. Behavioral variant frontotemporal dementia and semantic dementia showed significant overall striatal atrophy compared with controls. Moreover, behavioral variant frontotemporal dementia showed panstriatal degeneration, whereas semantic dementia featured a more focal pattern involving putamen and accumbens. Right-sided striatal atrophy, especially in the putamen, correlated with the overall behavioral symptom severity and with specific behavioral domains. At autopsy, patients with behavioral variant frontotemporal dementia and semantic dementia showed striking and severe tau or TAR DNA-binding protein of 43 kDa pathology, especially in ventral parts of the striatum. These results demonstrate that ventral striatum degeneration is a prominent shared feature in behavioral variant frontotemporal dementia and semantic dementia and may contribute to the social-emotional deficits common to both disorders.
行为变异型额颞叶痴呆和语义性痴呆与纹状体变性有关，但很少有研究在体内描绘纹状体亚区体积，并将其与临床表型相关联。我们在磁共振图像上追踪尾状核、壳核和伏隔核，以量化这些结构在行为变异型额颞叶痴呆、语义性痴呆、阿尔茨海默病和健康对照组（每组 12 名）中的体积。我们进一步将这些纹状体体积与一组患者的临床缺陷和神经病理学发现相关联。行为变异型额颞叶痴呆和语义性痴呆与对照组相比，表现出明显的总体纹状体萎缩。此外，行为变异型额颞叶痴呆表现为全纹状体变性，而语义性痴呆则表现为更局灶性的病变，涉及壳核和伏隔核。右侧纹状体萎缩，特别是壳核，与整体行为症状严重程度以及特定行为领域相关。在尸检中，行为变异型额颞叶痴呆和语义性痴呆患者表现出明显且严重的 tau 或 TAR DNA 结合蛋白 43 kDa 病理学，特别是在纹状体的腹侧部分。这些结果表明，腹侧纹状体变性是行为变异型额颞叶痴呆和语义性痴呆的一个突出共同特征，可能导致这两种疾病共有的社交情感缺陷。
