From the Guidelines
Amyotrophic Lateral Sclerosis (ALS) symptoms typically begin with muscle weakness that progressively worsens over time, often starting with muscle twitching, cramping, stiffness, or weakness in a limb, and can also include slurred speech or difficulty swallowing, as noted in the ESPEN guideline on clinical nutrition in neurology 1. The disease presents in two main forms: bulbar progressive paresis (bulbar onset, 25-35% of patients) or spinal motor neuron injury (limb onset or peripheral onset) 1.
- Early signs of ALS often include:
- Muscle twitching (fasciculations)
- Cramping
- Stiffness
- Weakness in a limb
- Slurred speech
- Difficulty swallowing
- As the disease advances, patients experience increasing muscle weakness and atrophy throughout the body, leading to difficulty moving, speaking, swallowing, and eventually breathing, with a mean survival of 3-5 years, and 5-10% living longer than 10 years 1.
- Cognitive changes occur in some patients, ranging from mild impairment to frontotemporal dementia, affecting 20-50% of cases, mainly frontotemporal dementia 1.
- Notably, ALS typically does not affect sensory nerves, so touch, sight, hearing, smell, and taste usually remain intact, and the disease also spares the bladder and bowel functions, as well as eye movements.
- The disease progression varies significantly between individuals, with some experiencing rapid decline while others have a slower progression over several years, and patients with bulbar onset and older age have the shortest life expectancy 1. Dysphagia is a severe and invalidating symptom for ALS patients, leading to swallowing disorders that disturb the ability to move food or liquids safely and efficiently from the mouth through the pharynx into the esophagus, and can be managed with artificial nutrition by feeding tubes, with gastrostomy being preferable 1.
From the Research
ALS Symptoms
- Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease caused by the death of motor neurons 2
- ALS causes a wide range of symptoms, including pain, cramps, spasticity, sialorrhoea, and respiratory insufficiency 3
- The evidence base for the effectiveness of symptomatic treatment therapies for ALS is limited 3
Treatment of ALS Symptoms
- Riluzole is a benzothiazole derivative that blocks glutamatergic neurotransmission in the CNS, which is thought to exert neuroprotective effects 2
- Riluzole was approved by the US FDA in 1995 as the first drug to treat ALS, although its efficacy in ALS is modest, prolonging tracheostomy-free survival by only 2-3 months 2
- Non-invasive ventilation (NIV) probably improves median survival and quality of life in people with respiratory insufficiency and normal to moderately impaired bulbar function compared to standard care 3
- Botulinum toxin A (BoTox-A) is safe and could be beneficial in the short term and long term in a subset of ALS patients with moderate-to-severe spasticity 4
Prognostic Factors
- Treatment with riluzole is an independent prognostic factor in ALS, with patients treated with riluzole exhibiting a 7-month longer median survival than those who did not receive riluzole 5
- The relationship between riluzole use and extended survival varied across disease progression strata, being only evident among fast progressors 5
- Early use of non-invasive ventilation prolongs survival in subjects with ALS, with a median time from ALS diagnosis to death significantly longer in the early NPPV group 6