Amyotrophic Lateral Sclerosis (ALS), commonly known as Lou Gehrig’s disease, is a progressive neurodegenerative disease. It ravages the motor neurons that communicate between the brain and muscles. ALS usually manifests between the ages of 40 and 70, with an average age of 55 at the time of diagnosis. It progresses differently in every individual, making it a challenge to predict the course it will take.
Early symptoms include muscle twitching, stiffness, and weakness, which gradually escalate to more severe muscle deterioration. Diagnosing ALS is a meticulous process, involving numerous tests to rule out other conditions, and could include electromyography (EMG), nerve conduction studies, and MRI scans.
The only known and widely accepted treatments for ALS are riluzole and Edaravone. Studies and trials have found the riluzole drug extends the longevity of ALS victims by about 3 months, while edaravone assists those who suffer from the disease in basic bodily functions, such as movement. When affected by ALS, the neurons of the brain are believed to go through a process related to an overproduction of excitatory amino acids, mostly glutamate. This creates excitotoxicity in the neurons, and leads to more overproduction of mainly calcium and sodium. This ultimately leads to more overproduction of free radicals, chemicals overproduced by the brain that can damage the brain, and then finally neuron death.
Riluzole works by altering certain behaviors of natural body substances that affect neurons and brain cells. While this is one of the only two medications recognized by the Food and Drug Administration (FDA), it has only been proven to extend the patients survival by a few months. This is due to the cumulative damage that ALS has on the motor neurons of the human body. Riluzole is usually taken in the form of an oral pill. It uses glutamine antagonist activity in order to suppress the atrophy of the brain, motor neurons and spinal cord. This anti-glutamine property of the drug allows for suppression of excitotoxicity in the neurons of the brain, which is believed to be Riluzole’s mechanism of action. Riluzole is generally strongly recommended to anyone as soon as they are diagnosed with amyotrophic lateral sclerosis.
Edaravone is the second of the known treatments for ALS. Edavaron is an antioxidant, meaning it helps protect against free radicals. This drug prevents damage caused by these free radicals, unstable molecules and substances produced by the body to react to environmental and other pressures. Edavarone’s specific mechanism of action still remains a mystery, partly because of our incomplete knowledge on the pathophysiology of ALS. However, edaravone is generally accepted as a current treatment for ALS due to its most likely antioxidative properties. This drug is usually given to the patient as a sterile, clear, colorless liquid by intravenous (IV) injection from a suspended polypropylene bag.
Although both of these treatments are prevalent, they are palliative, meaning that rather than treating the pathogenesis of ALS, they treat the symptoms. Therefore, further research is necessary to find a treatment for ALS that can help patients survive.
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