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运动神经元病患者的非运动症状

Non-Motor Symptoms in Patients Suffering from Motor Neuron Diseases.

作者信息

Günther René, Richter Nicole, Sauerbier Anna, Chaudhuri Kallol Ray, Martinez-Martin Pablo, Storch Alexander, Hermann Andreas

机构信息

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

Department of Basic and Clinical Neuroscience, The Maurice Wohl Clinical Neuroscience Institute, King's College London , London , UK.

出版信息

Front Neurol. 2016 Jul 25;7:117. doi: 10.3389/fneur.2016.00117. eCollection 2016.

Abstract

BACKGROUND

The recently postulated "disease spreading hypothesis" has gained much attention, especially for Parkinson's disease (PD). The various non-motor symptoms (NMS) in neurodegenerative diseases would be much better explained by this hypothesis than by the degeneration of disease-specific cell populations. Motor neuron disease (MND) is primarily known as a group of diseases with a selective loss of motor function. However, recent evidence suggests disease spreading into non-motor brain regions also in MND. The aim of this study was to comprehensively detect NMS in patients suffering from MND.

METHODS

We used a self-rating questionnaire including 30 different items of gastrointestinal, autonomic, neuropsychiatric, and sleep complaints [NMS questionnaire (NMSQuest)], which is an established tool in PD patients. 90 MND patients were included and compared to 96 controls.

RESULTS

In total, MND patients reported significantly higher NMS scores (median: 7 points) in comparison to controls (median: 4 points). Dribbling, impaired taste/smelling, impaired swallowing, weight loss, loss of interest, sad/blues, falling, and insomnia were significantly more prevalent in MND patients compared to controls. Interestingly, excessive sweating was more reported in the MND group. Correlation analysis revealed an increase of total NMS score with disease progression.

CONCLUSION

NMS in MND patients seemed to increase with disease progression, which would fit with the recently postulated "disease spreading hypothesis." The total NMS score in the MND group significantly exceeded the score for the control group, but only 8 of the 30 single complaints of the NMSQuest were significantly more often reported by MND patients. Dribbling, impaired swallowing, weight loss, and falling could primarily be connected to motor neuron degeneration and declared as motor symptoms in MND.

摘要

背景

最近提出的“疾病传播假说”备受关注,尤其是在帕金森病(PD)方面。该假说比疾病特异性细胞群的退化能更好地解释神经退行性疾病中的各种非运动症状(NMS)。运动神经元病(MND)主要被认为是一组具有选择性运动功能丧失的疾病。然而,最近的证据表明,MND中疾病也会扩散到非运动脑区。本研究的目的是全面检测MND患者的NMS。

方法

我们使用了一份自评问卷,其中包括30项不同的胃肠道、自主神经、神经精神和睡眠方面的主诉[NMS问卷(NMSQuest)],这是PD患者中常用的工具。纳入了90例MND患者,并与96例对照进行比较。

结果

总体而言,与对照组(中位数:4分)相比,MND患者报告的NMS得分显著更高(中位数:7分)。与对照组相比,流涎、味觉/嗅觉受损、吞咽受损、体重减轻、兴趣丧失、悲伤/忧郁、跌倒和失眠在MND患者中更为普遍。有趣的是,MND组报告多汗的情况更多。相关分析显示,总NMS得分随疾病进展而增加。

结论

MND患者的NMS似乎随疾病进展而增加,这与最近提出的“疾病传播假说”相符。MND组的总NMS得分显著超过对照组,但在NMSQuest的30项单项主诉中,只有8项在MND患者中报告得更频繁。流涎、吞咽受损、体重减轻和跌倒可能主要与运动神经元退化有关,并被视为MND中的运动症状。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eead/4958907/33a27b333ab7/fneur-07-00117-g001.jpg

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