Department of Medicine, Pamela Youde Nethersole Eastern Hospital, 3 Lok Man Road, Chai Wan, Hong Kong.
Hong Kong Med J. 2012 Feb;18(1):48-55.
To review evidences on the management of patients with motor neuron disease.
PubMed literature searches from January 1982 up to January 2011.
Key words for literature search were "motor neuron disease review (MND)". Only the articles which concentrated on the ventilation, nutrition, cognitive or multidisciplinary approaches for motor neuron disease were included. Case reports were not included in the review. In addition, publications were identified using the World Wide Web from references in these papers. Only articles in English were considered.
A total of 782 articles were retrieved using the key word search, of which 72 concentrated on ventilation, nutrition, cognitive or multidisciplinary approaches. From these, 43 articles were eventually included and formed the basis of this review.
Motor neuron disease is an adult-onset neurodegenerative disease that leads to weakness of limb, bulbar, and respiratory muscles. It displays an ethnic variation in incidence; 90% of cases are sporadic and 10% are familial. New diagnostic criteria have been proposed to increase diagnostic sensitivity. Proper clinical studies, electrophysiology, and neuroimaging are necessary before reaching a diagnosis of motor neuron disease. Riluzole remains the only disease-modifying drug approved for this disease; it prolongs life by 3 to 4 months. Multidisciplinary care units are important in the management of motor neuron disease patients. Non-invasive positive pressure ventilation prolongs life in motor neuron disease patients with respiratory failure. Enteral feeding is usually recommended for affected patients with malnutrition. Cognitive impairment is common in these patients, for whom a formal neuropsychiatric assessment is recommended. Appropriate palliative care is needed for these patients in order to improve their quality of dying.
Motor neuron disease is an incurable disease, for which a highly effective treatment is still pending. Symptomatic treatment remains the mainstay of management. A multidisciplinary approach embracing advances in non-invasive ventilation and gastrostomy can improve quality of life and extend the survival of motor neuron disease patients.
综述运动神经元病的治疗进展。
PubMed 数据库 1982 年 1 月至 2011 年 1 月文献检索。
检索关键词为“运动神经元病综述（MND）”。仅纳入专注于运动神经元病通气、营养、认知或多学科治疗的文章。病例报告不纳入综述。此外，通过引用文献在互联网上查找相关资料。仅纳入英文文献。
通过关键词检索共获得 782 篇文献，其中 72 篇集中于通气、营养、认知或多学科治疗。最终纳入 43 篇文章作为本综述的依据。
运动神经元病是一种成年起病的神经退行性疾病，导致肢体、延髓和呼吸肌无力。其发病率存在种族差异，90%为散发性，10%为家族性。新的诊断标准提高了诊断敏感性。诊断运动神经元病前必须进行适当的临床研究、电生理和神经影像学检查。利鲁唑是唯一批准用于治疗该病的疾病修饰药物，可将患者的生存期延长 3~4 个月。多学科护理单元对运动神经元病患者的管理非常重要。对于有呼吸衰竭的运动神经元病患者，无创正压通气可延长其生存期。对于有营养不良的患者，通常推荐肠内营养。这些患者常存在认知障碍，建议进行正式的神经心理学评估。为了提高患者临终生活质量，需要给予适当的姑息治疗。
运动神经元病是一种不可治愈的疾病，目前仍急需有效的治疗方法。对症治疗仍是治疗的主要手段。多学科方法包括无创通气和胃造口术的进步可以改善生活质量，延长运动神经元病患者的生存期。
