Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig's disease, is a rapidly progressive, invariably fatal neurological disease that attacks the nerve cells (neurons) responsible for controlling voluntary muscles (muscle action we are able to control, such as those in the arms, legs, and face). The disease belongs to a group of disorders known as motor neuron diseases, which are characterized by the gradual degeneration and death of motor neurons.
Pathophysiology: The characterizing highlight of ALS is the demise of both upper and lower engine neurons in the engine cortex of the cerebrum, the mind stem, and the spinal string. Before their pulverization, engine neurons create protein-rich incorporations in their cell bodies and axons. This may be somewhat because of imperfections in protein degradationThese considerations regularly contain ubiquitin, and by and large consolidate one of the ALS-related proteins: SOD1, TAR DNA tying protein.
About 5 percent of cases, a clear genetic history exists. The disease is classed as autosomal dominant in these patients; that is, that almost half of all family members show a clear history of ALS. Studies in the early 1990s on the genetic form of the disease, including work by one of our scientific advisors, Dr. Robert Brown, revealed that a single gene defect could account for a portion of these familial cases.