From the Guidelines
Amyotrophic Lateral Sclerosis (ALS) symptoms typically begin with muscle weakness that progressively worsens over time, and early recognition of these symptoms is crucial for timely diagnosis and management. The symptoms of ALS can be divided into several categories, including:
- Muscle weakness and atrophy, which can lead to difficulty with fine motor tasks, tripping, and falling
- Speech and swallowing difficulties, such as dysarthria and dysphagia, which can lead to malnutrition and dehydration
- Breathing difficulties, which can lead to respiratory failure
- Cognitive changes, ranging from mild impairment to frontotemporal dementia
- Other symptoms, such as muscle twitching, cramping, and stiffness.
According to the ESPEN guideline clinical nutrition in neurology 1, patients with ALS should be advised to fractionate and enrich their meals with energy or deficient nutrients to manage symptoms of dysphagia and malnutrition. The guideline also recommends dietetic counseling to adapt the texture of solid and liquids to facilitate swallowing and avoid aspiration in patients with moderate dysphagia 1. Additionally, postural maneuvers, such as chin-tuck posture, should be recommended to protect the airway during swallowing in patients with moderate dysphagia 1.
The natural history of oropharyngeal dysphagia in ALS patients is characterized by a progressive decline in swallowing function, leading to malnutrition, dehydration, and respiratory complications 1. The use of videofluoroscopy, fiberoptic endoscopic evaluation of swallowing, and other diagnostic tools can help identify swallowing abnormalities and guide management decisions 1. Overall, the management of ALS symptoms requires a multidisciplinary approach, including nutritional support, speech and language therapy, and respiratory care, to improve quality of life and slow disease progression.
From the Research
ALS Symptoms and Treatment
- ALS (Amyotrophic Lateral Sclerosis) is a progressive neurodegenerative disease that affects the motor neurons, leading to increasing disability and eventually death 2.
- The symptoms of ALS include muscle weakness, atrophy, and paralysis, as well as difficulties with speech, swallowing, and breathing 3.
- Currently, there are only two approved drugs for the treatment of ALS: riluzole and edaravone 4, 5.
- Riluzole has been shown to prolong tracheostomy-free survival by 2-3 months, although its efficacy is modest 5, 3.
- Edaravone, on the other hand, has been found to be effective in halting ALS progression during early stages, and is considered a promising new treatment option 4.
Riluzole Mechanism of Action
- Riluzole works by blocking glutamatergic neurotransmission in the central nervous system, which is thought to exert neuroprotective effects 3, 6.
- The exact mechanism of action of riluzole is still not fully understood, but it is believed to involve the inhibition of persistent sodium current, potentiation of calcium-dependent potassium current, and inhibition of neurotransmitter release 6.
- Riluzole has been shown to have a wide range of effects on neural activity, including the inhibition of repetitive firing, potentiation of glutamate transporters, and promotion of neuronal survival or growth factors 6.
Clinical Efficacy and Safety
- Riluzole has been found to be generally safe and well-tolerated in clinical practice, although it can cause side effects such as increased serum alanine transferase levels 5, 3.
- The clinical efficacy of riluzole has been demonstrated in several studies, which have shown that it can prolong survival and delay the use of surrogates such as tracheostomy and mechanical ventilation 5, 3.
- Edaravone has also been found to be safe and effective in clinical trials, and is considered a promising new treatment option for ALS 4.