Sialorrhea in patients with ALS: current treatment options.

Degenerative Neurological and Neuromuscular Disease Pub Date : 2019-03-20 eCollection Date: 2019-01-01 DOI:10.2147/DNND.S168353
Giancarlo Garuti, Fabrizio Rao, Viviana Ribuffo, Valeria A Sansone
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引用次数: 28

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder of the motor neuron, which selectively affects it both at central (first motor-neuron) and peripheral level (second motor-neuron). The disease shows up at a mean age of 56 years and the most affected are males. Although ALS may start as a bulbar or spinal disease, with the progression of the disease typically both become evident. Pharmacological approved treatments for ALS are still limited and include riluzole and edaravone which improve survival over time. Despite this, ALS leads to progressive muscle involvement and requires a complex multidisciplinary approach to manage increasing disability which goes beyond motor neurons. Sialorrhea is, amongst others, one of the most disabling symptoms in ALS. The complexity in managing saliva is due to a muscular spasticity and to a scarce palatino-lingual muscles control, rather than to an overproduction of saliva. These features could increase the risk of aspiration pneumonia and limit the use of noninvasive mechanical ventilation. We reviewed the treatment for sialorrhea in ALS patients that are available at this time, emphasizing pros and cons for each approach. Our purpose is to create a practical tool for the diagnosis, in order to facilitate the quantification and management of sialorrhea in everyday practice.

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ALS患者的唾液:目前的治疗方案。
肌萎缩性侧索硬化症(ALS)是一种运动神经元的进行性神经退行性疾病,可选择性地影响中枢(第一运动神经元)和外周(第二运动神经元)。这种疾病出现的平均年龄为56岁,受影响最大的是男性。虽然ALS可能以球茎或脊柱疾病开始,但随着疾病的进展,这两种疾病通常都会变得明显。经药理学批准的ALS治疗方法仍然有限,包括利鲁唑和依达拉奉,它们可以提高长期生存率。尽管如此,肌萎缩侧索硬化症导致进行性肌肉受累,需要复杂的多学科方法来管理日益增加的残疾,而不仅仅是运动神经元。唾液是ALS最严重的致残症状之一。控制唾液的复杂性是由于肌肉痉挛和缺乏腭舌肌控制,而不是由于唾液的过量产生。这些特征可能增加吸入性肺炎的风险并限制无创机械通气的使用。我们回顾了目前可用的治疗ALS患者唾液的方法,强调了每种方法的优缺点。我们的目的是创造一种实用的诊断工具,以便在日常实践中促进唾液的量化和管理。
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