Multidisciplinary Interventions in Motor Neuron Disease.

Journal of Neurodegenerative Diseases Pub Date : 2014-01-01 Epub Date: 2014-11-18 DOI:10.1155/2014/435164
U E Williams, E E Philip-Ephraim, S K Oparah
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Abstract

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.

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运动神经元疾病的多学科干预。
运动神经元病是一种神经退行性疾病,其特征是运动皮层的上运动神经元和脑干和脊髓的下运动神经元丧失。患者在发病 2-4 年后死亡。线粒体功能障碍、氧化应激、兴奋毒性和轴突运输受损等复杂的细胞过程相互作用,被认为是神经细胞丧失的致病过程。目前有证据表明,利鲁唑可作为一种疾病调节药物;多学科团队护理方法可用于患者管理;无创通气可用于呼吸管理;肉毒杆菌毒素 B 可用于治疗鼻出血;姑息治疗可贯穿整个病程;莫达非尼可用于疲劳治疗。在运动神经元病的吞咽困难、支气管分泌、假性横纹肌痉挛、痉挛、失眠、认知障碍和交流等方面的治疗还需要进一步研究。
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