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[肌萎缩侧索硬化症的对症治疗]

[The symptomatic treatment of amyotrophic lateral sclerosis].

作者信息

van den Berg L H, van den Berg J P, Mathus-Vliegen E M, Kampelmacher M J, van Kesteren R G, Jennekens F G

机构信息

Afd. Neurologie, Universitair Medisch Centrum Utrecht, Utrecht.

出版信息

Ned Tijdschr Geneeskd. 2004 Mar 13;148(11):513-8.

Abstract

Patients with amyotrophic lateral sclerosis (ALS) have symptoms of progressive muscle weakness, of disturbed speech and swallowing, and in the terminal phase those of respiratory weakness. Treatment options, in particular those for excessive weight loss and respiratory weakness, should be introduced to the patients and their families when the patient is emotionally capable and before dysarthria severely hampers communication. Special equipment for keeping the patient as mobile as possible should be made available much earlier than in the case of other diseases of the muscles as in ALS progression is much faster. Cramps, pathological crying or laughter, spasms, and spasticity can all be treated by medication. When speech can no longer be understood, adaptive strategies such as sign language, mime, posture and communication apparatus varying from a note pad to advanced computer systems can be used. Sialorrhoea, caused by difficulty swallowing with its accompanying danger of aspiration can be halted by the use of medication, by radiotherapy and by the injection into the salivary glands of botulin A toxin. Weight loss, also a result of dysphagia, can be avoided by eating frequent small meals or if necessary performing a percutaneous endoscopic or radiological gastroscopy. Excess mucus in the respiratory tract can be treated with anticholinergics. Difficulty in coughing up thick and sticky mucus cannot always be adequately helped. Respiratory weakness is treatable by external respiratory supportive therapy using a nasal mask, as well as invasive respiratory support via a trachcostoma and by treating the symptoms of respiratory weakness. The latter form of treatment is palliative and forms part of terminal care. During the terminal phase restlessness, anxiety, pain, and dyspnoea require the most attention. Treatment requires careful multidisciplinary cooperation.

摘要

肌萎缩侧索硬化症(ALS)患者会出现进行性肌肉无力、言语和吞咽障碍症状,在疾病末期还会出现呼吸肌无力症状。当患者在情绪上能够接受且构音障碍尚未严重妨碍沟通之前,就应向患者及其家属介绍治疗方案,尤其是针对体重过度减轻和呼吸肌无力的治疗方案。与其他肌肉疾病相比,由于ALS的病情进展要快得多,所以应更早地为患者提供尽可能保持其活动能力的特殊设备。抽筋、病理性哭笑、痉挛和强直都可以通过药物治疗。当言语无法再被理解时,可以使用诸如手语、哑剧、姿势以及从便签本到先进计算机系统等各种沟通设备等适应性策略。吞咽困难导致的流涎及其伴随的误吸风险,可以通过药物治疗、放射治疗以及向唾液腺注射肉毒杆菌毒素A来控制。吞咽困难导致的体重减轻,可以通过少食多餐来避免,必要时可进行经皮内镜或放射学胃镜检查。呼吸道过多的黏液可以用抗胆碱能药物治疗。咳出浓稠黏痰困难的情况有时无法得到充分缓解。呼吸肌无力可以通过使用鼻罩进行外部呼吸支持治疗,以及通过气管造口进行有创呼吸支持并治疗呼吸肌无力症状来治疗。后一种治疗方式是姑息性的,是临终护理的一部分。在疾病末期,烦躁不安、焦虑、疼痛和呼吸困难需要给予最多关注。治疗需要仔细的多学科合作。

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