Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease that currently has no complete cure. However, through a multifaceted treatment strategy, symptoms can be alleviated, disease progression can be delayed, and the quality of life for patients can be improved. The treatment goals are to maintain muscle function, improve respiratory and swallowing abilities, and provide comprehensive support to patients through collaboration within a multidisciplinary medical team.
Current treatment plans combine medications, physical therapy, assistive devices, and psychological support to form an integrated care model. The medical community continues to develop new therapies and actively explore potential breakthroughs such as gene therapy and cell therapy. Patients and their families should maintain close communication with the medical team to adjust treatment plans promptly according to changes in the patient's condition.
Treatment for ALS must be tailored to individual patient differences, typically encompassing four core areas: medication to slow disease progression, symptom management, functional maintenance, and psychological support. Although medication cannot cure the disease, it can delay neuronal damage; non-pharmacological therapies provide assistance for daily functional impairments, such as ventilators or nutritional support.
The composition of the multidisciplinary medical team is crucial for treatment success, usually including neurologists, physical therapists, speech therapists, and dietitians. Treatment plans need to be regularly evaluated to address the specific needs of patients at different stages, such as limb weakness or respiratory failure. For instance, when patients experience swallowing difficulties, nutritional support methods may need to be adjusted immediately.
Currently, there are three medications approved by global regulatory agencies for ALS: Riluzole (Rilutek), Edaravone (Radicava), and Relyvrio. These three drugs reduce neuronal damage through different mechanisms. Riluzole inhibits the excitatory neurotransmitter glutamate, reducing damage caused by excessive neuronal activation; its efficacy has been supported by long-term follow-up studies since its approval in 1995.
Edaravone reduces oxidative damage to neurons through its antioxidant effects, while Relyvrio, approved in 2017, is a combination of Riluzole and Tiglutik, which has been shown to extend patient survival by approximately 2-3 months. It is important to note that the efficacy of these medications varies among individuals, necessitating regular assessments to adjust dosages. Additionally, physicians may prescribe adjunct medications to alleviate muscle spasms, pain, or depressive symptoms.
Physical therapy is an important means to delay the deterioration of limb function. Therapists design individualized exercise programs, including passive joint movements to prevent contractures, electrical stimulation training to maintain muscle tone, and balance training to reduce the risk of falls. Respiratory function training helps patients slow the decline in lung capacity through specific breathing techniques.
Speech therapists intervene in the management of swallowing difficulties, possibly recommending a transition to a liquid diet or conducting swallowing function assessments. When the risk of swallowing is too high, a gastrostomy (G-tube) can ensure nutritional intake. In the stage of respiratory failure, non-invasive ventilators or BiPAP may be required as assistive devices.
The application of assistive devices can significantly improve quality of life. Voice input systems, specially designed keyboards, or eye-tracking devices help patients with communication function decline maintain their ability to communicate. Modifications to the home environment, such as installing stair lifts and non-slip flooring, can reduce the risk of accidental injuries. Some patients may need to use wheelchairs or mechanical exoskeletons to maintain mobility.
Nutritional management plays a critical role in ALS treatment. Patients often suffer from malnutrition due to swallowing difficulties, and dietitians design high-calorie, high-protein meal plans based on changes in weight and muscle mass. When oral intake is insufficient, feeding through a nasogastric tube or gastrostomy may be necessary to maintain weight and muscle mass.
The establishment of psychological support systems is essential. Patients and their families often experience anxiety or depression due to the uncertainty of the disease course. Psychological counseling and support groups can provide resources for emotional adjustment. Family education programs can also help caregivers understand the disease progression patterns, reducing the caregiving burden.
Adjustments to work and living environments should be planned early. For example, patients who have difficulty using computers can switch to voice input software, while those who find writing challenging can train in one-handed typing skills. In the home environment, adding grab bars in bathrooms and changing kitchen tools to large-button designs can help maintain independent living abilities.
Gene therapy and cell therapy are current research hotspots. Targeted therapies for pathogenic genes such as C9ORF72 and SOD1 are in clinical trial stages, with antisense oligonucleotide technology showing preliminary efficacy. In terms of cell therapy, results from mesenchymal stem cell transplantation trials suggest it may slow the rate of neurodegeneration, but long-term safety still needs verification.
Research on neuroprotective factors and nerve repair is also ongoing. For example, NMNAT gene therapy trials focus on repairing damaged neuronal transport systems, while improvements in the administration of nerve growth factor (NGF) may expand treatment options. These emerging therapies are mostly in Phase II or III trials, and patients can check eligibility for participation through clinical trial registration systems.
When experiencing unexplained muscle weakness, muscle atrophy, or increased twitching, immediate medical attention should be sought to confirm whether these are signs of disease progression. If respiratory function indices (such as forced vital capacity (FVC) dropping below 50%) or unexplained weight loss exceeding 5% occur, the treatment plan needs to be reassessed. Side effects from medications, such as abnormal liver enzymes or increased fatigue, should also be communicated to the physician promptly.
If family caregivers notice that the patient is experiencing worsening swallowing difficulties, decreased blood oxygen levels during sleep, or early symptoms of respiratory failure (such as difficulty breathing while lying flat), an emergency evaluation should be arranged. Adjustments to treatment plans should be led by a neurologist and should not involve self-modifying medication dosages.
The assessment of treatment effectiveness is primarily conducted through neurological assessment scales (such as ALSFRS-R) to track symptom progression, while also observing muscle strength, maintenance of daily functions, and changes in respiratory and swallowing functions. Physicians will also regularly conduct blood tests and imaging studies to evaluate disease control.
What is the purpose of speech therapy for ALS patients? What specific difficulties can it improve?Speech therapy aims to delay the decline of communication abilities by training oral muscle usage techniques to extend verbal expression capabilities. Therapists guide patients in using assistive communication devices, such as speech synthesizers or picture boards, to help patients effectively express their needs even after language function impairment.
How should ALS patients adjust their diet in daily life to prolong their nutritional intake capabilities?It is recommended to adopt a high-calorie, high-protein diet and adjust the food texture (such as pureed or liquid) based on the severity of swallowing difficulties. If necessary, enteral nutrition through a gastrostomy tube may be required to avoid malnutrition or the risk of aspiration pneumonia. Dietitians will create personalized meal plans based on the patient's eating functional stage.
What potential benefits and risks can participating in clinical trials for ALS bring to patients?Clinical trials may provide new treatment options that are not yet widely available, such as gene therapy or neuroprotective agents, but they may also carry unknown side effects. Patients should discuss the trial design, expected benefits, and risks in detail with their medical team and assess whether it aligns with their disease stage and quality of life needs.
What key measures are involved in respiratory function management for ALS patients in the end stage of the disease?End-stage patients often require non-invasive ventilation (NIV) or tracheostomy to assist breathing. The medical team will regularly monitor lung capacity (such as negative pressure tests) and adjust ventilation modes based on blood oxygen saturation and respiratory muscle strength to delay the progression of respiratory failure and improve quality of life.
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