ALS stands for amyotrophic lateral sclerosis, which translates to "no muscle nourishment." It is an insidious neurodegenerative disease characterized by a progressive loss of motor neurons that provide voluntary movements and muscle power. Paralysis and death due to complications of respiratory muscle weakness occur within three to five years without intensive medical care. It is often referred to as Lou Gehrig's Disease because the great Yankee baseball player contracted this disease and died at the peak of his career.
Symptoms
A hallmark early symptom of ALS, occurring in 60 percent of patients, is muscle weakness in one or more of the following: hands, arms, legs, muscles of speech, swallowing or breathing. According to the Mayo Clinic, the hands and feet may be affected first, making it difficult to lift objects or walk. As the disease progresses, muscles atrophy. In the final stage, the patient may be paralyzed.
Significance
According to The ALS Association, about 30,000 Americans have the disease at any one time. There are 5,600 new cases of ALS diagnosed in the US each year. Sixty percent of ALS patients are men and 93 percent are Caucasian. The majority are between the ages of 40 to 60 years old when they develop ALS. People living in Guam, West New Guinea and parts of Japan have an increased risk of developing ALS; this may be due to dietary factors. In addition, soldiers are at higher risk. ALS occurs throughout the world and affects all races, ethnic groups and socioeconomic classes.
Genetics
According to Travis Dunckley, 90 percent of ALS cases have no family history of the disease, which means it may affect anyone, anywhere. Ten percent of cases have a close family member with ALS, which is caused by a mutation of many genes. The most common inheritance pattern for familial ALS is autosomal dominant, which means there is a 50 percent chance an offspring will inherit the mutated gene and develop ALS.
Diagnosis
ALS is difficult to diagnose. There is no single test to establish the diagnosis. Diagnosis is based on the presence of nerve dysfunction in multiple areas of the body in the absence of sensory symptoms or pain, since only motor neurons are involved. For most ALS patients, the muscles of the eyes and bladder are not affected.
Management
Rilutek--the brand name under which the drug riluzole is sold--is the only drug approved by FDA for treatment of ALS. It was shown to prolong the life of persons with ALS by a few months and may modestly slow disease progression.
