Amyotrophic lateral sclerosis (ALS): Charcot's disease
Understanding a rare neurodegenerative disease
Dr. Antoine Mercier
Neurologist
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.
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