Understanding ALS
Knowledge is Power in the Fight Against ALS
Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig’s Disease, is a disease that slowly destroys the nerve cells in the brain and spinal cord that control muscle movement. As these nerve cells, called motor neurons, break down, they stop sending signals to the muscles, causing the muscles to weaken and waste away. Over time, the loss of motor neurons leads to the inability to move, speak, eat, and breathe. While there is no cure for ALS, there are treatments that can help manage symptoms and improve the quality of life for those affected.
Key Facts and Insights
Incidence and Prevalence
ALS affects about 5 out of every 100,000 people worldwide. Each year, about 5,000 people in the United States are diagnosed with ALS. Veterans are twice as likely to develop ALS, compared to those who have not served in the military.
Symptoms
Early symptoms of ALS can include muscle weakness or stiffness. Gradually, all muscles under voluntary control are affected, and individuals lose their strength and the ability to speak, eat, move, and breathe.
Diagnosis
ALS is diagnosed through a series of tests including electromyography (EMG), nerve conduction velocity (NCV), and magnetic resonance imaging (MRI).
Life Expectancy
The average life expectancy of a person with ALS is about 3 to 5 years from the time of diagnosis. However, some people may live longer, and about 10% of people with ALS survive for 10 or more years.
Treatment
Although there is no cure for ALS, there are treatments that can help manage symptoms and improve quality of life. Medications can slow the progression of the disease and therapies can help maintain muscle function and mobility for as long as possible. Supportive care, including physical therapy and assistive devices, can also significantly enhance quality of life for those with ALS. Advances in research are continuously bringing new hope for better treatments and eventually a cure.
A Brief History
- 1869: French neurologist Jean-Martin Charcot first describes the disease. Because of his pioneering work, ALS is sometimes referred to as Charcot's disease in France.
- 1939: Lou Gehrig's diagnosis brings ALS into the public eye. His farewell speech at Yankee Stadium is one of the most famous moments in sports history.
- 1993: Researchers identify a genetic mutation associated with ALS, providing a significant breakthrough in understanding the disease. The gene, called SOD1, is the first gene discovered to be linked to familial ALS. Since then, more than 40 genes associated with ALS have been discovered.
- 1995: Riluzole is approved by the FDA as the first drug treatment for ALS, shown to slow the progression of the disease.
- 2014: The ALS Ice Bucket Challenge raises over $200 million for ALS research, greatly increasing public awareness and funding for the disease.
- 2017: Edaravone, marketed as Radicava, is approved by the FDA as a treatment for ALS, offering another option to slow disease progression.
- 2021: The ACT for ALS Act is passed by Congress, providing increased funding and resources for ALS research and patient support.
- 2022: The FDA approves Tofersen (marketed as Qalsody), a targeted therapy for a specific genetic mutation in the SOD1 gene, marking another milestone in personalized medicine for ALS.