Huang N, Marie S K, Livramento J A, Chammas R, Nitrini R
Laboratory of Neurological Investigation, Cognitive and Behavioral Neurology Unit, Department of Neurology, University of São Paulo School of Medicine, Brazil.
Neurology. 2003 Aug 12;61(3):354-7. doi: 10.1212/01.wnl.0000078890.89473.ed.
The presence of 14-3-3 protein in the CSF has been described to have high sensitivity and specificity for Creutzfeldt-Jakob disease (CJD).
To relate 14-3-3 protein in the CSF with the clinical diagnoses of diseases causing rapidly progressive dementia.
The authors studied 46 patients with rapidly progressive dementia that was classified into three diagnostic groups: definitive or probable CJD, possible CJD, and other diagnoses. The definitive or probable CJD group comprised 17 patients (3 definitive sporadic, 1 probable iatrogenic, 3 familial, and 10 probable sporadic CJD cases), the possible CJD group was composed of 7 patients, and the group with other diagnoses had 22 patients. Detection of the 14-3-3 protein was done by the immunoblotting method.
In the definitive or probable CJD group, the test for 14-3-3 protein in CSF was positive in 14 (82%) cases, whereas 3 patients (1 probable sporadic and 2 familial cases) had negative results. CSF was positive for 14-3-3 protein in three of seven cases with possible CJD (42%). In the group with other diagnoses, three individuals had false-positive results (13%). Their diagnoses were definitive Alzheimer's disease, hypercalcemia, and multiple intracerebral hemorrhages.
The detection of 14-3-3 protein in CSF is a useful in vivo diagnostic test for CJD and, when used in the appropriate clinical context, shows a good correlation to CJD. The presence of the 14-3-3 protein in the CSF reinforces the CJD clinical diagnosis but may not be able to differentiate CJD from other causes of rapidly progressive dementia in everyday clinical practice.
脑脊液中14-3-3蛋白的存在对克雅氏病（CJD）具有高敏感性和特异性。
将脑脊液中的14-3-3蛋白与导致快速进展性痴呆的疾病临床诊断相关联。
作者研究了46例快速进展性痴呆患者，这些患者被分为三个诊断组：确诊或疑似CJD、可能的CJD以及其他诊断。确诊或疑似CJD组包括17例患者（3例确诊散发性、1例疑似医源性、3例家族性和10例疑似散发性CJD病例），可能的CJD组由7例患者组成，其他诊断组有22例患者。通过免疫印迹法检测14-3-3蛋白。
在确诊或疑似CJD组中，脑脊液14-3-3蛋白检测14例（82%）呈阳性，而3例患者（1例疑似散发性和2例家族性病例）结果为阴性。7例可能的CJD病例中有3例（42%）脑脊液14-3-3蛋白呈阳性。在其他诊断组中，3人出现假阳性结果（13%）。他们的诊断分别为确诊的阿尔茨海默病、高钙血症和多发性脑出血。
脑脊液中14-3-3蛋白的检测是CJD的一种有用的体内诊断试验，在适当的临床背景下使用时，与CJD有良好的相关性。脑脊液中14-3-3蛋白的存在强化了CJD的临床诊断，但在日常临床实践中可能无法将CJD与其他快速进展性痴呆病因区分开来。
