Amber Roguski , Michal Rolinski , Matt W. Jones , Alan Whone
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引用次数: 0
Abstract
Introduction
The earliest stages of alpha-synucleinopathies are accompanied by non-specific prodromal symptoms such as diminished sense of smell, constipation and depression, as well as more specific prodromal conditions including REM Sleep Behaviour Disorder (RBD). While the majority of RBD patients will develop an alpha-synucleinopathy, one of the greatest clinical challenges is determining whether and when individual patients will phenoconvert. Clinical evaluation of a patient presenting with RBD should therefore include robust and objective assessments of known alpha-synucleinopathy prodromes.
Methods
This study compared olfactory function self-report measures with psychophysical ‘Sniffin’ Stick 16-item Identification’ test scores in Control (n = 19), RBD (n = 16) and PD (n = 17) participants.
Results
We confirm that olfactory test scores are significantly diminished in RBD and PD groups compared to Controls (p < 0.001, One-Way ANOVA with Tukey-Kramer Post-Hoc, effect size = 0.401). However, RBD participants were only 56 % accurate when self-reporting olfactory dysfunction, hence markedly less likely to perceive or acknowledge their own hyposmia compared to Controls (p = 0.045, Fisher’s Exact Test, effect-size = 0.35).
Conclusion
When isolated RBD presents with hyposmia, there is an increased likelihood of phenoconversion to Parkinson’s Disease (PD) or Dementia with Lewy Bodies (DLB); unawareness of olfactory dysfunction in an individual with isolated RBD may therefore confound differential diagnosis and prognosis. Our results evidence the fallibility of olfactory function self-report in the context of RBD prognosis, indicating that clinical assessments of RBD patients should include more reliable measures of olfactory status.
引言α-突触核蛋白病的早期阶段伴有非特异性前驱症状,如嗅觉减退、便秘和抑郁,以及更具体的前驱症状,包括快速眼动睡眠行为障碍(RBD)。虽然大多数RBD患者会发展为α-突触核蛋白病,但最大的临床挑战之一是确定个别患者是否以及何时会进行表型转换。因此,对RBD患者的临床评估应包括对已知的α-突触核蛋白病前驱物进行有力和客观的评估。方法本研究将对照组(n=19)、RBD组(n=16)和PD组(n=17)的嗅觉功能自我报告量表与心理物理“Sniffin”Stick 16项目识别测试得分进行了比较。结果我们证实,与对照组相比,RBD和PD组的嗅觉测试得分显著降低(p<0.001,Tukey Kramer Post Hoc的单向方差分析,效应大小=0.401)。然而,RBD参与者在自我报告嗅觉功能障碍时的准确率仅为56%,因此,与对照组相比,他们明显不太可能感知或承认自己的尿道下裂(p=0.045,Fisher精确检验,效应大小=0.35)。结论当孤立的RBD表现为尿道下裂时,表型转化为帕金森病(PD)或路易体痴呆(DLB)的可能性增加;因此,对孤立性RBD患者嗅觉功能障碍的不了解可能会混淆鉴别诊断和预后。我们的研究结果证明,在RBD预后的背景下,嗅觉功能自我报告是错误的,这表明对RBD患者的临床评估应该包括更可靠的嗅觉状态测量。