Amyotrophic Lateral Sclerosis, ALS, Lou Gehrigs Disease
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrigs Disease, is a rapidly progressive neuromuscular disease caused by the destruction of nerve cells in the brain and spinal cord. This causes loss of nervous control of the voluntary muscles, resulting in the degeneration and atrophy of the muscles. Eventually the respiratory muscles are affected which leads to death from an inability to breath.
There are three types of ALS: sporadic, familial, and Guamian. The most common form is sporadic. A small number of cases are inherited genetic disorders (familial). A large number of cases, however, occur in Guam and other Pacific territories. The familial type of ALS is caused by a genetic defect in superoxide dismutase, an antioxidant enzyme that continuously removes the highly toxic free radical, superoxide.
The causes of sporadic and Guamian ALS are unknown. Several hypothesis have been proposed including: Glutamate toxicity; Oxidative stress; Mitochondrial dysfunction; Autoimmune disease; Infectious diseases (Lyme disease, poliomyelitis, HIV, and Tertiary syphilis); Toxic chemical exposure (pesticides); Heavy metals toxicity (lead, mercury, aluminum, and manganese); Calcium and magnesium deficiency; Carbohydrate metabolism; and Growth factor deficiency.
There are no specific lab tests for ALS, although there may be a deficiency in hexosaminidase.
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