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ALS – Overview

November 17, 2021


ALS – amyotrophic lateral sclerosis – is a disease of the parts of the nervous system that control voluntary muscle movement. In ALS, motor neurons or nerve cells that control muscles are gradually lost. As these motor neurons are lost, the muscles they control become weak and then non-functional, which leads to muscle weakness, disability, and eventually death. The word amyotrophic comes from the Greek root that means without nourishment to muscles and refers to loss of signals nerve cells normally send to muscles. Lateral refers to the location of the damage in the spinal cord. Sclerosis means hardened or scarred and refers to the hardened nature of the spinal cord in advanced ALS. While there’s no cure for ALS, managing symptoms and using every technology at our disposal can go a long way to improving the quality of life of people with ALS. Rilutek – also known as Riluzole – approved in 1995 was the first treatment approved by the FDA to treat ALS. It works by blocking glutamate, since too much glutamate injures nerve cells. Therefore, it slows the progression of disease. Different formulations of Riluzole have been developed for people who have difficulty swallowing, such as Exservan, which dissolves on the tongue. Nuedexta – another formulation – was approved in 2010 for the treatment of pseudobulbar affect. It can help those people with uncontrollable laughing or crying. This usually impacts about 15 to 45% of people with ALS. Radicava is an infusion therapy for treatment of ALS that slows down the loss of physical function. These are the only four medications approved by the FDA to treat ALS. However, managing the symptoms is just as important – if not more important – than treating the underlying disease. People with ALS suffer from pain, muscle cramps, spasticity, depression, anxiety, difficulty breathing, and weight loss. We have plenty of therapies at our disposal to help them be more comfortable. Using every medication and tool in our toolbox to help ameliorate their symptoms is vital to improving their quality of life.

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