Abstract

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. 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. 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. 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.

Highlights

  • Motor neuron diseases (MND), such as amyotrophic lateral sclerosis, are neurodegenerative diseases with a progressive degeneration of motor neurons and their axons

  • It is well known that the pathology is not limited to the initially affected cell populations

  • The aim of this study was to investigate the appearance of non-motor symptoms (NMS) in MND by comparing their prevalence to age- and sex-matched healthy individuals using the NMS questionnaire (NMSQuest)

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Summary

Introduction

Motor neuron diseases (MND), such as amyotrophic lateral sclerosis, are neurodegenerative diseases with a progressive degeneration of motor neurons and their axons. Disease spreading and involvement of other non-motor regions in the brain seem to occur [1]. Corresponding non-motor symptoms (NMS) like gastrointestinal-, autonomic-, neuropsychiatric-, and sleep disorders are well known in these diseases. Treatments of such NMS are fundamental elements in a modern and comprehensive health care for patients suffering from neurodegenerative diseases [2, 3]. 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. Recent evidence suggests disease spreading into non-motor brain regions in MND. The aim of this study was to comprehensively detect NMS in patients suffering from MND

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