Motor Neuron Disease

This chapter is an overview of the diagnosis and treatment of motor neuron disorders using amyotrophic lateral sclerosis (ALS) as the prototypical disorder. ALS is a rapidly progressive neurodegenerative disease caused by the combined loss of upper and lower motor neurons. The diagnosis of ALS can be made using the Awaji-shima criteria. Diagnostic strategies to assess ALS mimickers include imaging and laboratory studies. Current practice parameters including those of the American Academy of Neurology provide the basis for recommendations for treatment of ALS. The FDA has approved disease-modifying medications and several commonly used therapeutics are available for symptomatic treatment of dysphagia, sialorrhea, spasticity, depression, and anxiety. Rehabilitation strategies include adaptive mobility equipment, treatment of impaired communication, exercise, and restrictive lung disease management. ALS is one of a group of heterogeneous disorders within the category of motor neuron diseases. While there is no cure for ALS, symptomatic treatment provides improvement in quality of life and prolongs patient survival.