Lou Gehrig's disease is often referred to as Amyotrophic lateral sclerosis (ALS), this is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. Motor neurons come from the brain to the spinal cord and from the spinal cord to the muscles throughout the entire body. The progressive degeneration of the motor neurons in ALS would eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is also lost. With voluntary muscle action progressively affected, for this reason patients in the later stages of the disease may become totally paralyzed (Choi, 1988).
ALS is led to mean no muscle nourishment. When a muscle has no nourishment, it atrophies or wastes away hence the name. In addition to this, lateral shows the areas in a person's spinal cord where part of the nerve cells that signal and control the muscles are located. As this area degenerates, it leads to scarring or hardening (sclerosis) in this particular region.
As motor neurons degenerate, this obviously means they can no longer send impulses to the muscle fibers that otherwise normally result in muscle movement. Early symptoms of ALS often include increasing muscle weakness, especially involving the arms and legs, speech, swallowing or breathing. When muscles no longer receive the messages from the motor neurons that they require to function, the muscles begin to atrophy (become smaller). Limbs begin to look thinner as muscle tissue atrophies (Choi, 1988).
Neurodegeneration is used mainly for diseases that are characterised by progressive loss of structure and function of neurons. There are many neurodegenerative diseases including amyotrophic lateral sclerosis that occurs as a result of neurodegenerative processes in selective areas. Several molecular studies have been designed both in animal models and in humans to determine the physiopathology of the disease in order to develop new approaches for neurodegeneration. ALS is a neurological disease of unknown origin which is characterised by a selective degeneration and death of upper and lower motor neurons this progresses to paralysis and death over a period of time.
ALS diagnosis is based on the El Escorial criteria carried out on mainly clinical and electrophysiological findings in four body regions. Also around 95% of ALS patients are sporadic whereas 5% are familial. In this particular group approximately 15% are caused by mutations in the SOD one gene that codes for the CuZn superoxide dismutase-1 (Bensimon, 1994). This is an enzyme that catalyzes the dismutation of superoxide to molecular oxygen and hydrogen peroxide. The symptoms and pathology of familial ALS patients with SOD1 mutations resemble those of patients with sporadic ALS. This suggests there are common mechanisms of neuron degeneration in both forms of the diseases. Several potential mechanisms of motor neuron degeneration in ALS have been projected. These...