Roguski Amber, Rolinski Michal, Jones Matt W, Whone Alan
School of Physiology, Pharmacology and Neuroscience, University of Bristol, Bristol, United Kingdom.
Department of Neurology, Torbay Hospital, Torquay, United Kingdom.
Clin Park Relat Disord. 2022 Dec 17;8:100176. doi: 10.1016/j.prdoa.2022.100176. eCollection 2023.
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.
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.
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).
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.
α-突触核蛋白病的最早阶段伴有非特异性前驱症状,如嗅觉减退、便秘和抑郁,以及更具特异性的前驱病症,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体患者是否以及何时会发生表型转换。因此,对出现RBD的患者进行临床评估应包括对已知的α-突触核蛋白病前驱症状进行有力且客观的评估。
本研究比较了对照组(n = 19)、RBD组(n = 16)和帕金森病(PD)组(n = 17)参与者的嗅觉功能自我报告测量结果与心理物理学“嗅棒16项识别”测试分数。
我们证实,与对照组相比,RBD组和PD组的嗅觉测试分数显著降低(p < 0.001,采用Tukey-Kramer事后检验的单因素方差分析,效应大小 = 0.401)。然而,RBD参与者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,他们明显不太可能察觉到或承认自己存在嗅觉减退(p = 0.045,Fisher精确检验,效应大小 = 0.35)。
当孤立性RBD伴有嗅觉减退时,发生表型转换为帕金森病(PD)或路易体痴呆(DLB)的可能性增加;因此,孤立性RBD患者未意识到嗅觉功能障碍可能会混淆鉴别诊断和预后。我们的结果证明了在RBD预后背景下嗅觉功能自我报告的不可靠性,表明对RBD患者的临床评估应包括更可靠的嗅觉状态测量方法。