Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease with no current cure. However, a multidisciplinary treatment approach can alleviate symptoms, slow disease progression, and improve patients' quality of life. The treatment goals focus on maintaining muscle function, improving respiratory and swallowing capabilities, and providing comprehensive support through collaboration among a multidisciplinary medical team.
Current treatment strategies combine medication, physical therapy, assistive devices, and psychological support to form an integrated care model. The medical community continues to develop new therapies and actively explores potential breakthroughs such as gene therapy and cell therapy. Patients and their families should maintain close communication with their healthcare team to adjust treatment plans promptly according to changes in the disease condition.
ALS treatment must be tailored to individual patients. The treatment plan generally includes four core areas: medication to delay disease progression, symptom management, functional maintenance, and psychological support. While medications cannot cure ALS, they can slow nerve cell damage; non-pharmacological therapies assist with daily functional impairments, such as ventilators or nutritional support.
The composition of a multidisciplinary medical team is crucial for successful treatment, typically including neurologists, physical therapists, speech therapists, and dietitians. Treatment plans require regular evaluation to address the specific needs at different stages, such as when patients experience swallowing difficulties, which may necessitate immediate adjustments to nutritional support methods.
Currently, three drugs approved by global regulatory agencies for ALS are Riluzole (Rilutek), Edaravone (Radicava), and Relyvrio. These drugs slow nerve cell damage through different mechanisms. Riluzole inhibits the excitatory neurotransmitter glutamate, reducing neuronal overactivation-related damage; since its approval in 1995, its efficacy has been supported by long-term follow-up studies.
Edaravone acts as an antioxidant to reduce free radical damage to nerves. Relyvrio, approved in 2017, is a combination of Riluzole and Tiglutik, shown to extend survival by approximately 2-3 months. It is important to note that the effectiveness of these drugs varies among individuals, requiring regular assessment and dose adjustments. Additionally, physicians may prescribe adjunct medications to alleviate muscle tremors, pain, or depression.
Physical therapy is vital for delaying the deterioration of limb functions. Therapists design personalized exercise programs, including passive joint movements to prevent contractures, electrical stimulation to maintain muscle tone, and balance training to reduce fall risk. Respiratory training using specific breathing techniques helps slow the decline in lung capacity.
Speech therapists intervene in swallowing difficulties, recommending modified diets or conducting swallowing assessments. When swallowing risk becomes high, gastrostomy (G-tube) placement ensures adequate nutrition. During respiratory failure stages, non-invasive ventilators or biphasic positive airway pressure (BiPAP) devices may be required.
Assistive devices significantly improve quality of life. Speech input systems, specially designed keyboards, or eye-tracking devices help patients with communication impairments maintain interaction. Home modifications such as stairlifts and non-slip flooring reduce fall risk. Some patients may require wheelchairs or mechanical exoskeletons to prolong mobility.
Nutritional management plays a key role in ALS treatment. Patients often experience malnutrition due to swallowing difficulties. Dietitians develop high-calorie, high-protein dietary plans based on weight changes and muscle mass. When oral intake is insufficient, nasogastric or gastrostomy feeding may be necessary to maintain weight and muscle mass.
Psychological support systems are essential. Patients and families often experience anxiety or depression due to disease uncertainty. Counseling and support groups provide emotional resources. Family education programs also help caregivers understand disease progression and reduce caregiving burden.
Early planning for work and home environment adjustments is important. For example, patients with difficulty using computers can switch to voice input software, and those with writing challenges can train in one-handed typing. Home modifications such as installing grab bars in bathrooms and using large-button kitchen appliances help sustain independent living.
Gene therapy and cell therapy are current research hotspots. Targeted treatments for pathogenic genes such as C9ORF72 and SOD1 are in clinical trial phases, with antisense oligonucleotide technology showing initial promise. Cell therapy trials involving mesenchymal stem cell transplantation suggest potential to slow neurodegeneration, but long-term safety remains to be confirmed.
Research on neuroprotective factors and neural repair continues. For example, NMNAT gene therapy aims to repair damaged neuronal transport systems, while improved delivery methods for nerve growth factors (NGF) could expand therapeutic applications. Many of these emerging therapies are in phase II or III trials, and patients can check eligibility through clinical trial registries.
Immediate medical consultation is necessary when unexplained muscle weakness, atrophy, or increased muscle twitching occurs. Reassessment is required if respiratory function indices (such as FVC below 50%) or unexplained weight loss exceeding 5% are observed. Side effects like abnormal liver enzymes or increased fatigue should also prompt communication with a physician.
If caregivers notice worsening swallowing, decreased oxygen saturation during sleep, or early signs of respiratory failure (such as difficulty breathing when lying flat), urgent evaluation is advised. Treatment adjustments should be led by a neurologist and not made independently.
The effectiveness of treatment is mainly assessed through neurological rating scales (such as ALSFRS-R) tracking symptom progression, as well as observing muscle strength, maintenance of daily functions, and changes in respiratory and swallowing functions. Blood tests and imaging studies are also regularly performed to evaluate disease control.
What is the purpose of speech therapy for ALS patients? Which specific difficulties can it improve?Speech therapy aims to delay the deterioration of communication abilities by training oral muscle usage techniques to prolong speech capacity. Therapists guide patients in using assistive communication devices such as speech synthesizers or picture boards to help express needs effectively even as speech function declines.
How should ALS patients adjust their diet in daily life to prolong nutritional intake?High-calorie, high-protein diets are recommended, with modifications based on swallowing difficulty (e.g., pureed or liquid foods). When oral intake is insufficient, gastrostomy feeding is used to prevent malnutrition and aspiration pneumonia. Dietitians develop personalized dietary plans based on the patient's stage of swallowing function.
What potential benefits and risks do ALS clinical trials offer to patients?Clinical trials may provide access to new treatments not yet widely available, such as gene therapies or neuroprotective agents, but may also carry unknown side effects. Patients should discuss trial design, expected benefits, and risks in detail with their healthcare team and evaluate whether participation aligns with their disease stage and quality of life needs.
What are the key measures for respiratory management in end-stage ALS patients?End-stage patients often require non-invasive ventilation (NIV) or tracheostomy-assisted breathing. The medical team monitors lung capacity (e.g., negative pressure tests) and adjusts ventilation modes based on oxygen saturation and respiratory muscle strength to delay respiratory failure and improve quality of life.
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