There is often a pivotal event, such as an unexplained fall, alerting the patient that something is wrong. Paralysis usually occurs as the disease progresses. The disease does not usually affect mental abilities. Swallowing may become difficult. The person may tend to inhale food or saliva.
Death usually results from lung infections.
During the early stages, treatment focuses on quality of life. An enjoyable life is quite possible with the emotional support of family and friends. Depending on the patient's condition, braces, walkers or a wheelchair may help. The home may need changes to accommodate lost mobility. There are drugs that help control drooling and spastic movement.