Approximately 20,000 Americans have amyotrophic lateral sclerosis (ALS) with an additional 5,000 diagnosed each year, according to the ALS Association. Often referred to as Lou Gehrig's Disease after the famous baseball player who passed away from the disease, ALS is a progressive neurological degenerative disease affecting the motor neurons and muscles of the body for which there is no cure.
Identification
ALS is a neurological disease affecting the motor neurons of the nervous system. ALS is characterized by progressive muscle weakness and disability, leading to eventual death. It is caused by the destruction of neurons involved in motor control. There is no cure for ALS, but a combination of medications and therapy are used to slow the progression of the disease, treat symptoms, and assist the individual in adjusting to the challenges of living with ALS.
Symptoms
ALS is a progressive disease that gets worse with time. In the early stages, symptoms such as mild weakness in the legs and feet, muscle cramps and twitching, and speech slurring are often overlooked and ignored until they become worse. However, as the disease progresses, individuals continually become weaker and normal activities become more difficult. Eventually the disease leads to paralysis, as well as breathing and eating problems. Respiratory failure is the leading cause of death in individuals with ALS, according to The Mayo Clinic. There is no mental impairment associated with ALS, but it does increase the risk of developing dementia.
Causes
The symptoms of ALS are caused by the destruction of motor neurons that control movement. As these nerve cells die, muscles become weaker and eventually waste away due to lack of stimulation. Approximately 10 percent of ALS cases are caused by an inherited defective gene, with the majority appearing to be at random with no known cause, according to the National Institutes of Health. Possible causes of ALS include gene mutations in an antioxidant enzyme that targets free radicals in the body, an excess of the neurotransmitter glutamate, an autoimmune response, or environmental factors such as toxins or infectious agents.
Risk Factors
Most cases of ALS are diagnosed between the ages of 40 and 60. In individuals under the age of 65, men are more likely to be diagnosed with ALS. According to the Mayo Clinic, a child has a 50 percent chance of developing ALS if a parent also has ALS. There is also a higher incidence of ALS in Guam, West Guinea, and parts of Japan, according to the Mayo Clinic.
Diagnosis
There is no direct test for detecting the presence of ALS, but several tests are used to support a diagnosis, with the diagnosis coming most often from the observation of signs and symptoms by a physician. It is important to rule out other neurological diseases, especially in the early stages of the disease. Among the tests used to support an ALS diagnosis include an electromyogram, a nerve conduction velocity test (NCV), as well as an MRI, spinal tap, blood tests and muscle biopsy. An electromyogram measures the electrical activity of the muscles by the placement of electrodes into the muscles. An NCV test involves placing electrodes onto the skin, applying a small shock, and measuring the strength and speed of the responding nerve signals.
Treatment
The FDA has approved only one medication to slow the progression of ALS, as of 2010. The drug works by decreasing the levels of glutamate, a neurotransmitter implicated in the destruction of motor nerve cells. Other medications are aimed at relieving symptoms and improving the quality of life of individuals. Common medications prescribed help by relieving muscle cramps, fatigue, constipation, pain and depression. Physical, occupational, and speech therapy are beneficial for patients living with ALS to aid in adjusting to the difficulties associated with the disease. Physical therapy can help maintain muscle strength and range of motion while speech therapists teach techniques to limit speech impairment and introduce speech-assisting devices at later stages of the disease.
