Department of Pathology.
Fishberg Department of Neuroscience, Friedman Brain Institute, Ronald M. Loeb Center for Alzheimer's Disease, Icahn School of Medicine at Mount Sinai, New York, New York.
J Neuropathol Exp Neurol. 2019 Jan 1;78(1):31-37. doi: 10.1093/jnen/nly104.
Essential tremor (ET) patients develop more cognitive impairment and dementia than controls, although there are surprisingly few data on the neuropathological basis for cognitive changes in ET. In this postmortem study, we assessed tau and other pathologies in 26 ET cases and 73 controls (non-ET) (1:3 matching). The mean age = 88.6 years; 55% were cognitively normal, 24% had mild cognitive impairment (MCI), and 20% had dementia. We found similar burdens of pathology using Braak, β-amyloid and Lewy body assessments in ET and controls. In contrast, among cognitively normal subjects, ET cases had a higher number of NFT-positive neurons in the neocortex than controls (p < 0.001); the number of NFT-positive neurons in the medial temporal lobe was similar in these 2 groups (p = 0.22). Among subjects with MCI, ET cases also had higher numbers of NFT-positive neurons in the neocortex than controls (p < 0.001) but again, not in the medial temporal lobe (p = 0.55). Among subjects with dementia, the number of NFT-positive neurons was similar in ET cases and controls. Cognitive function correlated with quantitative neurofibrillary tangle counts in ET cases and controls. In the context of ET, pre-dementia tau burden is higher than in the absence of ET, suggesting a predisposition to tau pathology.
特发性震颤（ET）患者比对照组更容易出现认知障碍和痴呆，尽管关于 ET 认知变化的神经病理学基础的数据却少之又少。在这项尸检研究中，我们评估了 26 例 ET 病例和 73 例对照组（非 ET）（1:3 匹配）的 tau 和其他病理。平均年龄为 88.6 岁；55%认知正常，24%有轻度认知障碍（MCI），20%有痴呆。我们发现 ET 和对照组的 Braak、β-淀粉样蛋白和路易体评估具有相似的病理负担。相比之下，在认知正常的受试者中，ET 病例的新皮质中 NFT 阳性神经元数量多于对照组（p<0.001）；这两组内侧颞叶 NFT 阳性神经元数量相似（p=0.22）。在 MCI 受试者中，ET 病例的新皮质中 NFT 阳性神经元数量也多于对照组（p<0.001），但内侧颞叶中则不然（p=0.55）。在痴呆受试者中，ET 病例和对照组的 NFT 阳性神经元数量相似。认知功能与 ET 病例和对照组的神经纤维缠结定量计数相关。在 ET 的背景下，痴呆前 tau 负担高于没有 ET 的情况，这表明 tau 病理存在倾向性。
