Department of Neurology, Assaf Harofeh Medical Center, 70300, Zerifin, Israel.
Sackler Faculty of Medicine, Tel-Aviv University, Tel Aviv, Israel.
J Neural Transm (Vienna). 2019 May;126(5):607-611. doi: 10.1007/s00702-018-1958-1. Epub 2018 Nov 29.
Previous studies have suggested that disease duration in Creutzfeldt-Jakob disease (CJD) may be related to the radiological findings or cerebrospinal fluid (CSF) tau levels; however, it is not yet established whether clinical, radiological, and laboratory findings at diagnosis can predict survival or have a prognostic value. The aim of this study was to examine whether the disease duration is correlated with clinical, radiological, and laboratory variables. The study population consisted of consecutive familial CJD (fCJD) patients that were assessed within 1 week from the diagnosis including the CJD neurological scale (CJD-NS), Minimental Status Examination, Frontal Assessment Battery, NIH Stroke Scale, and the expanded disability status scale. In addition, a single MRI study was done and measurements of the extent of the cortical and subcortical involvement were performed. CSF was examined as part of the workout, and tau levels were determined. Sixty-nine fCJD patients were included in the study (43 males, mean age 59.3 ± 8.4, range 44-79 years). The mean disease duration was 7.3 ± 6.9 months (median 5.6 months, range 2-20 months). A significant correlation was found between the disease duration and the CJD-NS, the disease burden as reflected by the degree of cortical involvement by DWI, and the CSF tau levels. The findings of the current study reveal that several findings at disease onset including the disease severity, the cortical changes, and the tau levels are each individually correlated with disease duration and can be used by the clinician as a tool to predict the disease course and prognosis.
先前的研究表明，克雅氏病（CJD）的病程可能与影像学表现或脑脊液（CSF）tau 水平有关；然而，目前尚不清楚诊断时的临床、影像学和实验室检查结果是否可以预测生存或具有预后价值。本研究旨在探讨病程是否与临床、影像学和实验室变量相关。研究人群包括连续的家族性 CJD（fCJD）患者，这些患者在诊断后 1 周内接受评估，包括 CJD 神经量表（CJD-NS）、简易精神状态检查、额叶评估量表、NIH 卒中量表和扩展残疾状况量表。此外，还进行了单次 MRI 研究，并对皮质和皮质下受累的程度进行了测量。作为常规检查的一部分，还检测了 CSF，并测定了 tau 水平。本研究共纳入 69 例 fCJD 患者（43 例男性，平均年龄 59.3±8.4 岁，范围 44-79 岁）。平均病程为 7.3±6.9 个月（中位数 5.6 个月，范围 2-20 个月）。病程与 CJD-NS、DWI 反映的皮质受累程度以及 CSF tau 水平之间存在显著相关性。本研究结果表明，发病时的一些发现，包括疾病严重程度、皮质变化和 tau 水平，与病程均存在个体相关性，临床医生可将其作为预测疾病过程和预后的工具。
