Department of Neurology, Clinical Dementia Center, University Medical Center, Georg-August University, Göttingen, Germany.
J Alzheimers Dis. 2014;38(1):63-73. doi: 10.3233/JAD-130995.
Dementia with Lewy bodies (DLB) is difficult to differentiate from other neuro-degenerative diseases. Patients are often mistaken to suffer from Parkinson's disease (PD) or Alzheimer's disease (AD) because of the overlapping clinical appearances concerning cognition and movement.
We investigated the possibility for a valid differential diagnosis using cerebrospinal fluid (CSF) biomarkers.
In the context of a large retrospective study, we analyzed data of patients suffering from degenerative, ischemic, or inflammatory CNS (central nervous system) diseases and identified those with DLB (n = 34), PD (n = 37), and AD (n = 47) for further analyses.
We detected abnormalities in the CSF profiles of those patients with DLB while using a combination of decreased amyloid-β (Aβ)42 and increased tau levels. By stratification of data by disease severity, we observed a high sensitivity of this combination especially in the subgroup of patients with advanced stages, while the sensitivity in early forms was lower. In addition, with clinical deterioration, the abnormalities in the CSF profile became more pronounced.
We conclude that DLB can be distinguished from PD, in spite of both being synucleinopathies, by CSF profiles using neurodegenerative marker analysis. The pathophysiology of increased tau and decreased Aβ levels in those conditions has to be elucidated further, since both proteins are known to be involved in the pathogenesis of AD, but no clear explanation has been postulated for DLB yet.
路易体痴呆(DLB)与其他神经退行性疾病难以区分。由于认知和运动方面的临床表现重叠,患者经常被误诊为帕金森病(PD)或阿尔茨海默病(AD)。
我们研究了使用脑脊液(CSF)生物标志物进行有效鉴别诊断的可能性。
在一项大型回顾性研究中,我们分析了患有退行性、缺血性或炎症性中枢神经系统(CNS)疾病患者的数据,并确定了患有 DLB(n = 34)、PD(n = 37)和 AD(n = 47)的患者进行进一步分析。
我们发现,当使用 Aβ42 降低和 tau 水平升高的组合时,DLB 患者的 CSF 谱存在异常。通过按疾病严重程度对数据进行分层,我们观察到该组合在晚期患者亚组中的敏感性很高,而在早期形式中的敏感性较低。此外,随着临床恶化,CSF 谱中的异常变得更加明显。
我们得出结论,尽管 PD 和 DLB 都是突触核蛋白病,但通过使用神经退行性标志物分析的 CSF 谱可以将 DLB 与 PD 区分开来。这些情况下 tau 增加和 Aβ 水平降低的病理生理学需要进一步阐明,因为这两种蛋白质都已知与 AD 的发病机制有关,但尚未为 DLB 提出明确的解释。