Munhoz Renato P, Teive Helio A
Movement Disorders Centre, Toronto Western Hospital, University of Toronto, Toronto, ON, Canada.
Movement Disorders Unit, Neurology Service, Hospital de Clinicas, Federal University of Parana, Curitiba, Brazil.
Clin Neurol Neurosurg. 2014 Dec;127:71-4. doi: 10.1016/j.clineuro.2014.09.014. Epub 2014 Oct 5.
REM sleep behaviour disorder (RBD) is typically linked to synucleinopathies (SP). In this study we analyzed the utility and performance of RBD as a tool for the differential diagnosis of the most common forms of degenerative parkinsonism, including SPs and tauopathies.
Patients with a syndromic diagnosis of degenerative parkinsonism matched for gender, age, and disease stage were assessed using a structured protocol with demographic and clinical data, including the diagnosis of probable RBD (pRBD), ascertained clinically using established criteria.
One hundred cases of Parkinson's disease (PD), 87 with progressive supranuclear palsy (PSP), 72 with the parkinsonian form of multiple system atrophy (MSA), 50 with dementia with Lewy bodies (DLB), and 18 with corticobasal degeneration (CBD) were included. pRBD was found in 58 (58%) of the PD patients, 59 (81.9%) of those with MSA, 37 (74%) with DLB, 32 (36.7%) with PSP, and one (5.5%) with CBD. Among the SPs, pRBD was significantly more common in MSA when compared with PD patients. Differences were also significant individually for all SPs when compared to PSP. The positive predictive value (PPV) of pRBD for a SP was 82.3%, but sensitivity was 69.4% and specificity 68.6%.
In our sample, pRBD was more frequent in SPs than in PSP and CBD, however, its' frequency in PSP was significant. Although pRBD had a good PPV for a SP, all other measurements used for determine diagnostic performance were disappointing.
快速眼动睡眠行为障碍(RBD)通常与突触核蛋白病(SP)相关。在本研究中,我们分析了RBD作为鉴别诊断最常见形式的退行性帕金森病(包括SP和tau蛋白病)工具的效用和性能。
使用包含人口统计学和临床数据的结构化方案,对性别、年龄和疾病阶段相匹配的退行性帕金森病综合征诊断患者进行评估,包括使用既定标准临床确定的可能RBD(pRBD)诊断。
纳入了100例帕金森病(PD)患者、87例进行性核上性麻痹(PSP)患者、72例多系统萎缩帕金森型(MSA)患者、50例路易体痴呆(DLB)患者和18例皮质基底节变性(CBD)患者。在58例(58%)PD患者、59例(81.9%)MSA患者、37例(74%)DLB患者、32例(36.7%)PSP患者和1例(5.5%)CBD患者中发现了pRBD。在SP中,与PD患者相比,pRBD在MSA中更为常见。与PSP相比,所有SP个体之间的差异也很显著。pRBD对SP的阳性预测值(PPV)为82.3%,但敏感性为69.4%,特异性为68.6%。
在我们的样本中,pRBD在SP中比在PSP和CBD中更常见,然而,其在PSP中的发生率也很显著。尽管pRBD对SP有良好的PPV,但用于确定诊断性能的所有其他测量结果都令人失望。