May is officially designated as ALS Awareness Month, serving as a time to educate the public about this condition. Amyotrophic Lateral Sclerosis (ALS), often called Lou Gehrig’s disease, is a progressive neurodegenerative disease. It targets and destroys the nerve cells in the brain and spinal cord that control voluntary muscle movement. This loss of motor neurons leads to increasing weakness and paralysis throughout the body.
Understanding Amyotrophic Lateral Sclerosis
ALS is characterized by the degeneration and death of both upper motor neurons (originating in the brain) and lower motor neurons (extending from the spinal cord to the muscles). When these motor neurons die, they can no longer send signals to the muscles, causing them to weaken and waste away (atrophy). This results in the progressive loss of the ability to control muscle movement, while leaving the senses, such as sight, touch, and hearing, unaffected.
The disease’s progression rate varies significantly, but it is fatal, usually due to respiratory failure. Most cases (90 to 95 percent) are sporadic, occurring without a known family history. The remaining 5 to 10 percent are familial cases linked to a genetic cause. The average age of diagnosis is around 58 to 60 years old, with most people developing the disease between 40 and 70. ALS is more common in men than in women, though this difference tends to disappear after age 70.
Recognizing the Signs and Diagnosis
The initial signs of ALS can be subtle and easily overlooked, often resembling symptoms of other neurological conditions. Early manifestations involve painless, gradual muscle weakness, which may present in the limbs (limb-onset) or in the muscles controlling speech and swallowing (bulbar-onset). Patients may notice difficulty with fine motor tasks, such as buttoning a shirt or turning a key, or experience tripping due to foot weakness.
Other common early symptoms include involuntary muscle twitching (fasciculations), muscle cramps, and spasticity or stiffness. If the disease begins with bulbar involvement, the first signs may be slurred or nasal speech, or difficulty swallowing. Diagnosis relies on a careful neurological exam and observation of progressive symptoms over time.
To confirm the diagnosis and exclude other disorders, neurologists utilize tests like electromyography (EMG) and nerve conduction velocity (NCV) studies. The EMG measures the electrical activity in muscles in response to nerve stimulation, revealing signs of motor neuron damage. The combination of clinical presentation and supportive test results allows for confirmation of ALS.
Current Treatment and Management Strategies
While a cure remains the goal, several FDA-approved medications help slow disease progression and manage symptoms. Riluzole, the first approved drug, blocks the release of glutamate, a neurotransmitter that injures motor neurons when found in excess. Edaravone, an antioxidant, reduces oxidative stress on nerve cells, slowing functional decline in some patients.
A third drug, tofersen, is a genetically targeted therapy approved for people with ALS caused by a mutation in the SOD1 gene. Tofersen works by reducing the production of the toxic SOD1 protein, slowing disease progression in eligible individuals.
Beyond medication, comprehensive management relies on a specialized multidisciplinary care approach. Multidisciplinary clinics bring together a team of specialists, including neurologists, physical therapists, occupational therapists, respiratory therapists, and speech-language pathologists. This coordinated care improves the quality of life and may extend survival. Supportive interventions include non-invasive ventilation, nutritional support, and physical therapy to address mobility.
Actions for Awareness and Support
ALS Awareness Month provides an opportunity for the public to engage with the ALS community and support those affected.
Ways to Support ALS Awareness
- Become an advocate by contacting federal and local lawmakers to request funding for research and improved patient access to care.
- Share educational facts and personal stories about ALS on social media to broaden public understanding using hashtags like #ALSAwareness.
- Participate in events hosted by major ALS organizations, such as Walk to Defeat ALS events or other community fundraisers.
- Donate to organizations that fund research and provide patient services to accelerate the search for a cure.
- Support patient and caregiver networks, which offer practical and emotional resources for families navigating the disease.