Emergencies in Motor Neuron Diseases

Motor neuron diseases cause loss of innervation of muscles, resulting in disabling progressive muscle weakness. The most common motor neuron disease in children is spinal muscular atrophy (SMA); in adults, the most common form is amyotrophic lateral sclerosis (ALS). Although both diseases are characterized by progressive motor neuron loss, the disease presentation, diagnosis, and management of both diseases are quite different. Emergencies encountered in patients with SMA are often related to early diagnosis and early initiation of therapy, whereas in the case of ALS they are mostly related to worsening of bulbar and respiratory muscle weakness. In this chapter, an overview of the most commonly encountered emergencies in SMA and ALS is given.