Yuksel Deniz, Yilmaz Deniz, Uyar Neval Y, Senbil Nesrin, Gurer Yavuz, Anlar Banu
Department of Pediatric Neurology, Dr Sami Ulus Children's Hospital, 06930 Ankara, Turkey.
Brain Dev. 2010 Jun;32(6):467-71. doi: 10.1016/j.braindev.2009.11.009. Epub 2009 Dec 23.
Neurodegenerative diseases characterized by cytoskeletal deformation and neurofibrillary tangles are associated with altered levels of tau and related proteins in cerebrospinal fluid (CSF). Neuronal or glial fibrillary tangles have been shown in 20% of subacute sclerosing panencephalitis (SSPE) patients. We therefore investigated CSF samples from 60 newly diagnosed SSPE and 31 neurological control patients for total tau (t-tau), phosphorylated tau (p-tau), and S100-B levels by ELISA. There was no difference between patient and control groups in t-tau and S100-B levels. p-Tau was lower in the SSPE group (p=0.009). Past history of measles infection, measles immunization status, latent period between measles and onset of SSPE, duration of symptoms, frequency of myoclonia, neurological deficit index, stage and progression rate of the disease, CSF glucose levels and cell counts, CSF and serum measles IgG titer, distribution of lesions on brain magnetic resonance imaging were not related to t-tau, p-tau and S100-B levels. Mental status and age were negatively correlated with t-tau, and male gender and EEG abnormalities were associated with higher t-tau levels. The levels of tau proteins in our patients suggest there is no, or only scarce and immature, neurofibrillary tangle formation in SSPE. Autopsy studies showing neurofibrillary tangles might have examined older patients with longer disease and more parenchymal involvement.
以细胞骨架变形和神经原纤维缠结为特征的神经退行性疾病与脑脊液(CSF)中tau蛋白及相关蛋白水平的改变有关。在20%的亚急性硬化性全脑炎(SSPE)患者中已发现神经元或胶质原纤维缠结。因此,我们通过酶联免疫吸附测定法(ELISA)检测了60例新诊断的SSPE患者和31例神经科对照患者的脑脊液样本中的总tau蛋白(t-tau)、磷酸化tau蛋白(p-tau)和S100-B水平。患者组和对照组的t-tau和S100-B水平没有差异。SSPE组的p-Tau较低(p = 0.009)。麻疹感染病史、麻疹免疫状况、麻疹与SSPE发病之间的潜伏期、症状持续时间、肌阵挛频率、神经功能缺损指数、疾病分期和进展率、脑脊液葡萄糖水平和细胞计数、脑脊液和血清麻疹IgG滴度、脑磁共振成像上病变的分布与t-tau、p-tau和S100-B水平无关。精神状态和年龄与t-tau呈负相关,男性和脑电图异常与较高的t-tau水平相关。我们患者的tau蛋白水平表明,SSPE中不存在神经原纤维缠结,或仅存在稀少且不成熟的神经原纤维缠结。尸检研究显示神经原纤维缠结,可能检查的是病程较长、脑实质受累较多的老年患者。