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呼吸功能受损导致肌萎缩侧索硬化症患者嗅觉障碍。

Impairment in Respiratory Function Contributes to Olfactory Impairment in Amyotrophic Lateral Sclerosis.

作者信息

Günther René, Schrempf Wiebke, Hähner Antje, Hummel Thomas, Wolz Martin, Storch Alexander, Hermann Andreas

机构信息

Division for Neurodegenerative Diseases, Department of Neurology, Technische Universität Dresden, Dresden, Germany.

German Center for Neurodegenerative Diseases (DZNE) Dresden, Dresden, Germany.

出版信息

Front Neurol. 2018 Feb 26;9:79. doi: 10.3389/fneur.2018.00079. eCollection 2018.

Abstract

BACKGROUND

Nonmotor symptoms are very common in neurodegenerative diseases. In patients suffering from amyotrophic lateral sclerosis (ALS), olfactory dysfunction was first reported more than 20 years ago; however, its pathophysiological correlates and further implications remain elusive.

METHODS

In this so far largest case-control study, we analyzed olfactory performance with the "Sniffin' Sticks," a validated olfactory testing kit used in clinical routine. This test kit was designed to investigate different qualities of olfaction including odor threshold, odor discrimination, and odor identification.

RESULTS

ALS patients were mildly but significantly impaired in TDI score, the composite of the three subtests (ALS 27.7 ± 7.9, Controls 32.3 ± 5.8). In contrast to Parkinson's disease, ALS patients did not show impaired performance in the suprathreshold tests identification and discrimination. However, the odor threshold was markedly decreased (ALS 6.0 ± 3.4, Controls 8.77 ± 3.6). This pattern of olfactory loss resembles sinonasal diseases, where olfactory dysfunction results from impeded odorant transmission to the olfactory cleft. The evaluation of medical history and clinical data of ALS patients showed that patients with perception of dyspnea (TDI 25.7 ± 8.0) performed significantly worse in olfactory testing compared to those who did not (TDI 30.0 ± 7.4). In line with that, we found that in patients with preserved respiratory function (vital capacity >70% of index value), olfactory performance did not differ from healthy controls.

CONCLUSION

These findings suggest that the mild impairment of olfaction in patients suffering from ALS should at least partly be considered as a consequence of impaired respiratory function, and odor threshold might be a marker of respiratory dysfunction in ALS.

摘要

背景

非运动症状在神经退行性疾病中非常常见。在患有肌萎缩侧索硬化症(ALS)的患者中,嗅觉功能障碍早在20多年前就有报道;然而,其病理生理相关性及进一步影响仍不明确。

方法

在这项迄今为止最大规模的病例对照研究中,我们使用“嗅觉棒”分析嗅觉表现,“嗅觉棒”是临床常规使用的经过验证的嗅觉测试工具。该测试工具旨在研究嗅觉的不同特性,包括气味阈值、气味辨别和气味识别。

结果

ALS患者的TDI评分轻度但显著受损,TDI评分是三个子测试的综合得分(ALS患者为27.7±7.9,对照组为32.3±5.8)。与帕金森病不同,ALS患者在阈上测试(识别和辨别)中表现未受损。然而,气味阈值明显降低(ALS患者为6.0±3.4,对照组为8.77±3.6)。这种嗅觉丧失模式类似于鼻鼻窦疾病,在鼻鼻窦疾病中,嗅觉功能障碍是由于气味剂向嗅裂的传输受阻所致。对ALS患者的病史和临床数据评估显示,有呼吸困难感的患者(TDI为25.7±8.0)在嗅觉测试中的表现明显比没有呼吸困难感的患者(TDI为30.0±7.4)差。与此一致,我们发现呼吸功能保留(肺活量>指标值的70%)的患者,其嗅觉表现与健康对照组无差异。

结论

这些发现表明,ALS患者嗅觉的轻度受损至少部分应被视为呼吸功能受损所致,气味阈值可能是ALS患者呼吸功能障碍的一个标志物。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1912/5834512/a99fa780acd6/fneur-09-00079-g001.jpg

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