Williams U E, Philip-Ephraim E E, Oparah S K
Internal Medicine Department, University of Calabar, Calabar, Cross River State 540242, Nigeria.
J Neurodegener Dis. 2014;2014:435164. doi: 10.1155/2014/435164. Epub 2014 Nov 18.
Motor neuron disease is a neurodegenerative disease characterized by loss of upper motor neuron in the motor cortex and lower motor neurons in the brain stem and spinal cord. Death occurs 2-4 years after the onset of the disease. A complex interplay of cellular processes such as mitochondrial dysfunction, oxidative stress, excitotoxicity, and impaired axonal transport are proposed pathogenetic processes underlying neuronal cell loss. Currently evidence exists for the use of riluzole as a disease modifying drug; multidisciplinary team care approach to patient management; noninvasive ventilation for respiratory management; botulinum toxin B for sialorrhoea treatment; palliative care throughout the course of the disease; and Modafinil use for fatigue treatment. Further research is needed in management of dysphagia, bronchial secretion, pseudobulbar affect, spasticity, cramps, insomnia, cognitive impairment, and communication in motor neuron disease.
运动神经元病是一种神经退行性疾病,其特征是运动皮层中的上运动神经元以及脑干和脊髓中的下运动神经元丧失。发病后2至4年患者会死亡。线粒体功能障碍、氧化应激、兴奋性毒性和轴突运输受损等细胞过程之间复杂的相互作用被认为是神经元细胞丢失的潜在发病机制。目前有证据支持使用利鲁唑作为疾病修饰药物;采用多学科团队护理方法进行患者管理;使用无创通气进行呼吸管理;使用肉毒杆菌毒素B治疗流涎;在疾病全过程中提供姑息治疗;以及使用莫达非尼治疗疲劳。在运动神经元病的吞咽困难、支气管分泌物、假性延髓情绪、痉挛、抽筋、失眠、认知障碍和沟通管理方面,还需要进一步研究。