Amyotrophic lateral sclerosis (ALS): Charcot's disease
Understanding a rare neurodegenerative disease
Dr. Antoine Mercier
Neurologist
In simple terms
Progressive weakness, muscle atrophy: ALS, its warning signs and management.
Motor neuron involvement
ALS destroys central and peripheral motor neurons, leading to progressive paralysis of all voluntary muscles (limbs, swallowing, speech, breathing), while preserving cognitive, sensory, and sphincter functions.
Warning signs
Asymmetrical limb weakness, cramps, fasciculations (visible muscle twitching), speech disorders (dysarthria) or swallowing difficulties.
Diagnosis
Clinical + electromyography (EMG). Revised El Escorial criteria. Average diagnostic delay: 12 months.
Treatments
Riluzole: the only drug that moderately slows progression. Edaravone in some countries. Very active research: antisense (tofersen for SOD1 forms), gene therapies.
Supportive care
Essential multidisciplinary approach: physiotherapy, speech therapy, nutrition (gastrostomy), non-invasive ventilation, early palliative care. Median survival: 3-5 years, but 10% live >10 years.
The words explained
Medical terms used above, in everyday language.
- Diagnosis
- The moment a doctor identifies which illness is causing your symptoms.
- Cognitive
- Related to thinking: memory, attention, learning and decision-making.
When should you see a doctor?
- Your symptoms last more than a few weeks or keep coming back.
- They get worse quickly, or stop you doing everyday things.
- You notice sudden pain, breathing trouble, fainting or bleeding — seek urgent care.
- You already take medication and want to change anything about it.
This article explains general health information. It does not replace advice from your own doctor.
Frequently asked questions
- What is Amyotrophic lateral sclerosis (ALS): Charcot's disease?
- Progressive weakness, muscle atrophy: ALS, its warning signs and management.
- What are the main symptoms or signs of Amyotrophic lateral sclerosis (ALS): Charcot's disease?
- Common signs vary from person to person. The article above lists the symptoms to watch for, how they progress, and when they need medical attention.
- When should you see a doctor for Amyotrophic lateral sclerosis (ALS): Charcot's disease?
- See a doctor if symptoms last more than a few weeks, get worse quickly, stop you doing everyday things, or if you notice sudden pain, breathing trouble, fainting or bleeding.
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