Green A J E
The National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK.
Neuropathol Appl Neurobiol. 2002 Dec;28(6):427-40. doi: 10.1046/j.1365-2990.2002.t01-2-00427.x.
The differential diagnosis of dementia can be difficult in the early stages of disease, and with the emergence of new therapeutic agents for Alzheimer's disease (AD) there is an increasing need for reliable and accurate diagnostic tests. The concept of brain-specific proteins was first proposed in the 1960s and, since that time, methods have developed to measure these proteins in the cerebrospinal fluid (CSF). The concentration of individual brain-specific proteins can be altered in disease, and these changes are thought to reflect the underlying pathology. CSF tau protein and amyloid peptide A beta 42 concentrations are altered in AD and have been proposed as early diagnostic tests for this disease. The data from a number of studies suggest that these proteins may be of value, but are less specific than previously thought and further studies with neuropathological confirmation are required before these tests can be introduced into clinical practice. The detection of 14-3-3 in the CSF is an accurate test for sporadic Creutzfeldt-Jakob disease (CJD) and this accuracy has lead the World Health Organization to revise the clinical criteria for probable sporadic CJD to include a positive CSF 14-3-3. However, CSF 14-3-3 is less useful in the diagnosis of variant CJD, where studies are underway investigating the value of other CSF proteins.
在疾病的早期阶段，痴呆症的鉴别诊断可能会很困难。随着阿尔茨海默病（AD）新治疗药物的出现，对可靠且准确的诊断测试的需求日益增加。脑特异性蛋白的概念最早在20世纪60年代被提出，从那时起，测量脑脊液（CSF）中这些蛋白的方法得到了发展。个体脑特异性蛋白的浓度在疾病中会发生改变，这些变化被认为反映了潜在的病理状况。脑脊液中的tau蛋白和淀粉样肽Aβ42浓度在AD中会发生改变，并已被提议作为该疾病的早期诊断测试。多项研究的数据表明，这些蛋白可能具有价值，但特异性低于先前的认知，在将这些测试引入临床实践之前，还需要进行更多有神经病理学确认的研究。脑脊液中14-3-3的检测是散发性克雅氏病（CJD）的一项准确测试，这种准确性促使世界卫生组织修订了可能散发性CJD的临床标准，将脑脊液14-3-3阳性纳入其中。然而，脑脊液14-3-3在变异型CJD的诊断中作用较小，目前正在进行研究以探讨其他脑脊液蛋白的价值。
