Amyotrophic Lateral Sclerosis (ALS) is a type of motor neuron disease, often called Lou Gehrig's disease within the US. The term Motor Neuron Disease is used synonymously for ALS in some countries, however, ALS is only one type of motor neuron disease defined by motor neuron degeneration in both the brain and the spinal cord. When motor neurons die as a result of ALS, the ability of the brain to control muscle movement is lost. When motor neurons degenerate in the spinal cord, muscles weaken and often atrophy. The consequence of both brain and spinal cord cell loss is poorly coordinated, weak movements resulting in progressive disability.
All voluntary muscles can be affected by ALS, including respiration. Common symptoms include: muscular weakness, abnormal muscle tone, fatigue/exercise intolerance, shortness of breath, depression, and speech and swallowing difficulties. There is often an under recognized cognitive component and/or a pseudobulbar (emotional lability) component seen as well. Sight, touch, hearing, taste, smell, digestion and bowel/bladder are generally not affected. ALS affects all races, both sexes, and ages from 18-95, although the percentages increase with age, and men are affected slightly more often than women. There are childhood forms of ALS and in a minority of patients, the disease can be inherited. At present there is no standardized lab test to diagnose patients with ALS. An accurate diagnosis depends on the clinical and EMG examination. Finding new methods to make an early diagnosis of ALS and the related motor neuron disease is an active area of research at the Carolinas Neuromuscular/ALS Center. The earlier that a patient can be identified, the more likely new neuroprotective drugs may be helpful.
Although there is no cure at this time, there are multiple modes of supportive and adjunctive therapies that can change the course of the disease. In addition, one drug is currently approved for ALS patients. Rilutek has a modest effect on respiratory function and may increase life expectancy. Proactive, aggressive medical care is extremely important, and results in increased quality of life for people with ALS and the related motor neuron diseases. Available treatments in respiratory therapy, nutrition, physical therapy, occupational therapy and speech therapy can be synergistic.