关键词: 14-3-3 protein biomarker cerebrospinal fluid rapidly progressive dementia

Mesh : Humans Aged Neurodegenerative Diseases 14-3-3 Proteins / cerebrospinal fluid Myoclonus Creutzfeldt-Jakob Syndrome / diagnosis cerebrospinal fluid Biomarkers / cerebrospinal fluid Autoimmune Diseases of the Nervous System

来  源:   DOI:10.3233/JAD-220718

Abstract:
The 14-3-3 protein in cerebrospinal fluid (CSF) is a suitable biomarker for the diagnosis of Creutzfeldt-Jakob disease (CJD). However, it has also been detected in various non-prion-related rapidly progressive dementia (RPD), which affected its diagnostic performance and clinical utilization.
To investigate the general disease distribution with positive 14-3-3 result and to evaluate the association between CSF 14-3-3 protein and the clinical features in patients with non-prion RPD.
A total of 150 patients with non-prion RPD were enrolled. The clinical data were collected and CSF 14-3-3 test was performed for all patients. The distribution of various diseases with a positive 14-3-3 result was analyzed and the association of CSF 14-3-3 with clinical features was tested.
The CSF 14-3-3 protein was detected in 23.3% of non-prion RPD patients, and the most frequent diagnoses were autoimmune encephalitis (22.9%) and neurodegenerative disease (22.9%). CSF 14-3-3 protein was more common in older patients (p = 0.028) and those presenting myoclonus (p = 0.008). In subgroup analysis, the positive 14-3-3 test was more common in neurodegenerative disease with a long time from the symptom onset to CSF 14-3-3 test (p = 0.014).
CSF 14-3-3 protein could be detected in a broad spectrum of non-prion RPD. In particular, patients with autoimmune encephalitis and rapidly progressive neurodegenerative diseases and those with myoclonus have a greater likelihood of a positive 14-3-3 result. These results could help clinicians interpret the results of CSF 14-3-3 protein more reasonably.
摘要:
背景:脑脊液(CSF)中的14-3-3蛋白是诊断克雅氏病(CJD)的合适生物标志物。然而,它也已在各种非朊病毒相关的快速进展性痴呆(RPD)中检测到,这影响了其诊断性能和临床利用率。
目的:探讨非朊病毒RPD患者14-3-3阳性的一般疾病分布及其与临床特征的关系。
方法:共纳入150例非朊病毒RPD患者。收集临床资料,对所有患者进行CSF14-3-3检验。分析了14-3-3阳性结果的各种疾病的分布,并测试了CSF14-3-3与临床特征的关联。
结果:在23.3%的非朊病毒RPD患者中检测到CSF14-3-3蛋白,最常见的诊断是自身免疫性脑炎(22.9%)和神经退行性疾病(22.9%).CSF14-3-3蛋白在老年患者(p=0.028)和出现肌阵挛症的患者(p=0.008)中更为常见。在亚组分析中,14-3-3试验阳性在神经退行性疾病中更为常见,从症状发作到CSF14-3-3试验的时间较长(p=0.014).
结论:CSF14-3-3蛋白可以在广谱的非朊病毒RPD中检测到。特别是,自身免疫性脑炎和快速进展的神经退行性疾病患者以及肌阵挛者14-3-3阳性结果的可能性更大.这些结果可以帮助临床医生更合理地解释CSF14-3-3蛋白的结果。
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