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. 1. Background Motor neuron disease (MND) also referred to as amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative condition with an annual incidence of about 1.5 per 100,000 [1] and a United Kingdom (UK) prevalence of 4–6/100,000 [2]. There is a slight male preponderance with a male to female ratio of 3？:？2. It could occur at any age but the peak age of occurrence is between 50 and 75 years [3]. Multiple genetic and environmental factors interact resulting in loss of the upper motor neuron in the motor cortex and the lower motor neurons cell bodies in the brain stem and spinal cord [4, 5]. Pattern of onset could be spinal, truncal, or bulbar. The clinical features of MND include limb weakness, respiratory impairment, dysphagia, fatigue, sleep disorders, pain, psychosocial distress, communication deficits, cognitive impairment, and spasticity. Death occurs secondary to respiratory failure 2 to 4 years after disease onset on average; however survival of patients up to a decade has been reported [6]. There is currently no cure for MND; hence management is focused on symptomatic treatment, rehabilitative care, and palliative care. The disease exerts a huge psychological and economic burden on the patient and caregivers. 2. Review Strategy Evidence for this review was obtained from a search of the Cochrane data base, PUBMED, guidelines of National Institute for Clinical Excellence (NICE), American Academy of Neurology (AAN), and European Federation of Neurological Societies (EFNS); and peer-reviewed journal articles. MND diagnosis is based on the El Escorial diagnostic criteria [4, 5]. 3. Objectives This
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