TY - JOUR T1 - CSF P-Tau181和其他生物标志物在神经元核内包涵体病患者中的应用JF -神经学JO -神经学SP - e1009 LP - e1019 DO - 10.1212/WNL.0000000首页000201647六世- 100 - 10盟栗原市Masanori盟——小松Hiroki盟,仙谷由人Renpei盟——城市涩川市举行的Mari盟森本晃司,Satoru AU -松原科技界盟,荒川晶盟——Orita Makoto AU - Ishibashi吴克群盟——Mitsutake Akihiko盟——柴田Shota盟——Ishiura Hiroyuki AU -足立,薰盟——Ohse Kensuke AU -波多野,Keiko AU - Ihara,恭子非盟- Higashihara魔法盟,仁科靖盟——Tokumaru阿雅美岛绿盟——Ishii吴克群盟——斋藤裕盟——Murayama Shigeo AU - Kanemaru,背景和目的在苏氨酸181位点磷酸化的脑脊液tau蛋白(p-tau181)是阿尔茨海默病(AD)广泛使用的生物标志物，最首页近被认为可以反映AD大脑中的β-淀粉样蛋白和/或p-tau蛋白沉积。神经元核内包体病(NIID)是一种以神经元、胶质细胞和其他体细胞核内包体为特征的神经退行性疾病。症状包括痴呆、神经病等。CSF生物标志物未见报道。本研究的目的是调查包括p-tau181在内的CSF生物标志物是否在NIID患者中发生改变。方法回顾性观察研究。比较了12例NIID患者、120例基于CSF生物标志物谱生物学证实的阿尔茨海默临床综合征患者和临床诊断为其他神经认知障碍的患者(路易体痴呆[DLB]， 24例;额颞叶痴呆[FTD] 13;进行性核上性麻痹[PSP]， 21; and corticobasal syndrome [CBS], 13). Amyloid PET using Pittsburgh compound B (PiB) was performed in 6 patients with NIID.Results The mean age of patients with NIID, AD, DLB, FTD, PSP, and CBS was 71.3, 74.6, 76.8, 70.2, 75.5, and 71.9 years, respectively. CSF p-tau181 was significantly higher in NIID (72.7 ± 24.8 pg/mL) compared with DLB, PSP, and CBS and was comparable between NIID and AD. CSF p-tau181 was above the cutoff value (50.0 pg/mL) in 11 of 12 patients with NIID (91.7%). Within these patients, only 2 patients showed decreased CSF Aβ42, and these patients showed negative or mild local accumulation in PiB PET, respectively. PiB PET scans were negative in the remaining 4 patients tested. The proportion of patients with increased CSF p-tau181 and normal Aβ42 (A−T+) was significantly higher in NIID (75%) compared with DLB, PSP, and CBS (4.2%, 4.8%, and 7.7%, respectively). CSF HVA and 5-HIAA concentrations were significantly higher in patients with NIID compared with disease controls.Discussion CSF p-tau181 was increased in patients with NIID without amyloid accumulation. Although the deposition of p-tau has not been reported in NIID brains, the molecular mechanism of tau phosphorylation or secretion of p-tau may be altered in NIID.5-HIAA=5-hydroxyindole acetic acid; AD=Alzheimer disease; Aβ42=amyloid-beta 1–42; ANCOVA=analysis of covariance; CBS=corticobasal syndrome; CL=centiloid; DLB=dementia with Lewy bodies; FAB=Frontal Assessment Battery; FXTAS=fragile X-associated tremor/ataxia syndrome; FTD=frontotemporal dementia; HSV-1=herpes simplex virus type 1; HVA=homovanillic acid; MAO=monoaminoxidase; MMSE=Mini-Mental State Examination; NIID=neuronal intranuclear inclusion disease; p-tau181=tau phosphorylated at threonine 181; PiB=Pittsburgh compound B; PSP=progressive supranuclear palsy; t-tau=total tau ER -