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威尔逊氏病患者的嗅觉功能障碍

Olfactory dysfunction in patients with Wilson's Disease.

作者信息

Piechal Agnieszka, Bembenek Jan, Baranowska Anna, Litwin Tomasz, Mirowska-Guzel Dagmara, Członkowska Anna

机构信息

Second Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland.

Department of Experimental and Clinical Pharmacology, Centre for Preclinical Research and Technology, Medical University of Warsaw, Warsaw, Poland.

出版信息

Neurol Neurochir Pol. 2023;57(2):212-218. doi: 10.5603/PJNNS.a2023.0020. Epub 2023 Mar 20.

DOI:10.5603/PJNNS.a2023.0020
PMID:36939310
Abstract

INTRODUCTION

Many neurodegenerative disorders are associated with olfactory dysfunction (OD), but little is known about OD in Wilson's Disease (WD). We evaluated olfactory function in patients with WD.

MATERIAL AND METHODS

OD was examined in 68 patients with WD and 70 sex- and age-matched healthy controls using subjective testing with 'Sniffin Sticks'. Threshold discrimination identification (TDI) score and its three components (odour detection threshold, discrimination, and identification) were assessed.

RESULTS

Compared to controls, patients with WD had a significantly weaker sense of smell in terms of TDI (p < 0.01), odour discrimination (p < 0.01), and identification (p < 0.01), but not in terms of odour detection threshold (p = 0.27). Patients with predominantly neurological symptoms were characterised by greater OD by TDI (p < 0.01), odour detection threshold (p = 0.01), and discrimination (p = 0.03). The presence of pathological lesions (p = 0.04) in brain magnetic resonance imaging and generalised brain atrophy (p = 0.02) predisposed to worse TDI. In the WD group, weak inverse correlations between age and TDI score (r = -0.27), odour detection threshold (r = -0.3), and discrimination (r = -0.3) were found. Male gender was a risk factor for abnormal TDI in both WD and controls (both p = 0.02).

CONCLUSIONS

Patients with WD, particularly older individuals, more frequently had OD than healthy volunteers. Predominantly neurological symptoms, and the presence of typical brain MRI changes, predisposed patients with WD to smell disorders.

摘要

引言

许多神经退行性疾病都与嗅觉功能障碍(OD)有关,但对于威尔逊病(WD)中的OD却知之甚少。我们评估了WD患者的嗅觉功能。

材料与方法

使用“嗅觉棒”主观测试法,对68例WD患者和70例年龄及性别匹配的健康对照者进行OD检测。评估了阈值辨别识别(TDI)得分及其三个组成部分(气味检测阈值、辨别力和识别力)。

结果

与对照组相比,WD患者在TDI(p < 0.01)、气味辨别力(p < 0.01)和识别力(p < 0.01)方面嗅觉明显较弱,但在气味检测阈值方面无差异(p = 0.27)。以神经症状为主的患者,其TDI(p < 0.01)、气味检测阈值(p = 0.01)和辨别力(p = 0.03)方面的OD更明显。脑磁共振成像中病理性病变的存在(p = 0.04)和广泛性脑萎缩(p = 0.02)易导致TDI更差。在WD组中,年龄与TDI得分(r = -0.27)、气味检测阈值(r = -0.3)和辨别力(r = -0.3)之间存在弱负相关。男性性别是WD患者和对照组TDI异常的危险因素(均为p = 0.02)。

结论

WD患者,尤其是老年患者,比健康志愿者更易出现OD。以神经症状为主以及典型脑MRI改变的存在,使WD患者易患嗅觉障碍。

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引用本文的文献

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Olfactory function in Wilson's disease-systematic literature review.威尔逊氏病的嗅觉功能——系统文献综述
Acta Neurol Belg. 2025 Feb 10. doi: 10.1007/s13760-025-02741-y.
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Olfactory Dysfunction-A Potential Biomarker of Neurological Involvement in Wilson's Disease.嗅觉功能障碍——威尔逊病神经受累的潜在生物标志物
Mov Disord Clin Pract. 2025 Mar;12(3):398-399. doi: 10.1002/mdc3.14303. Epub 2024 Dec 17.
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Reply to: Olfactory Dysfunction: A Potential Biomarker of Neurological Involvement in Wilson's Disease.回复:嗅觉功能障碍:威尔逊病神经受累的潜在生物标志物。
Mov Disord Clin Pract. 2025 Mar;12(3):397. doi: 10.1002/mdc3.14301. Epub 2024 Dec 17.