What is Lou Gehrig's disease (Amyotrophic Lateral Sclerosis, ALS)?

From the Guidelines

Lou Gehrig's disease, also known as Amyotrophic Lateral Sclerosis (ALS), is a progressive neurodegenerative disorder that affects nerve cells in the brain and spinal cord, and the most recent and highest quality study recommends a multidisciplinary approach to care, including physical therapy, occupational therapy, speech therapy, respiratory support, and nutritional management, as outlined in the 2024 National Academies of Sciences, Engineering, and Medicine report 1.

Key Recommendations for ALS Care

• Multidisciplinary care: A team-based approach to care, including physical therapy, occupational therapy, speech therapy, respiratory support, and nutritional management, is essential for improving quality of life and slowing disease progression 1.
• Respiratory support: The use of respiratory assist devices (RAD) can improve quantity and quality of life, and Medicare qualification for RAD reimbursement should be updated to align with current best practices 1.
• Nutritional management: Patients with ALS should receive nutritional counseling to manage weight loss and maintain adequate nutrition, and gastrostomy feeding may be recommended for patients with severe dysphagia 1.
• Access to care: Individuals with ALS should have equitable access to affordable multidisciplinary care, services, and caregiver support, and delays in approval and delivery of equipment and services should be minimized 1.

Treatment Options for ALS

• Riluzole (Rilutek): May extend survival by 2-3 months by reducing glutamate toxicity, with typical dosing of 50 mg twice daily 1.
• Edaravone (Radicava): Can slow the decline in daily functioning, administered as an intravenous infusion in 28-day cycles 1.

Quality of Life Considerations

• Palliative care: Patients with ALS should have access to palliative care services, including pain management, emotional support, and end-of-life care 1.
• Caregiver support: Caregivers of individuals with ALS should receive support and resources to manage the physical and emotional demands of caregiving 1.

From the Research

Definition and Overview of Lou Gehrig's Disease

• Lou Gehrig's disease, also known as Amyotrophic Lateral Sclerosis (ALS), is a progressive neurodegenerative disease that impairs upper and lower motor neurons, leading to debilitating motor dysfunction and eventually respiratory failure 2, 3, 4.
• ALS is characterized by progressive degeneration of motor neurons, a paralytic disorder caused by motor neuron degeneration, and is diverse in its presentation, course, and progression 2.

Prevalence and Incidence

• The prevalence of ALS varies globally, with incidence rates ranging from 1.5 to 3.8 per 100,000 individuals, significantly affecting populations aged 45-80 4.
• In the USA, there are approximately 25,000 patients with ALS, with an average age of onset of 55 years, and an incidence and prevalence of 1-2 and 4-6 per 100,000 each year, respectively 2.
• Approximately 30,000 Americans currently have the disease, with an annual incidence rate of one to two cases per 100,000 3.

Symptoms and Diagnosis

• ALS presents with diverse symptomatology, including dysarthria, dysphagia, muscle atrophy, and hyperreflexia 4.
• The disease usually presents with problems in dexterity or gait resulting from muscle weakness, and difficulty in speaking or swallowing is the initial symptom in the bulbar form of the disease 3.
• Diagnosis is determined by excluding other conditions and utilizing clinical examinations, laboratory tests, and nerve conduction/electromyography studies 5.

Treatment and Management

• Current management involves aggressive, individualized alleviation of symptoms and complications 3.
• Therapeutic strategies for ALS, still in nascent stages, involve symptomatic management and experimental approaches targeting molecular pathways implicated in ALS pathology 4.
• Gene therapy, focusing on specific ALS mutations, and stem cell therapy emerge as promising avenues 4.
• The best practices for the management of ALS include an interdisciplinary approach aimed at addressing the physical and psychological needs and desires of patients and their families and caregivers 5.