The disorder affects men more often than women. Symptoms usually do not develop until adulthood, often not until after age 50. The gait may be stiff or clumsy . Reflexes may be abnormal, including loss of the gag reflex. An EMG indicates loss of muscle function caused by lack of nervous stimulation.
Treatment is aimed at control of symptoms.
Baclofen or diazepam may be used to control the spasticity the interferes with activities of daily living. Trihexyphenidyl or amitriptyline may be prescribed for people with impaired ability to swallow saliva. A referal to an otolaryngologist may be advised. Emotional support is vital in coping with the disorder, because mental functioning is not affected. Groups such as the ALS society may be available to assist in coping with the disorder.