In 80% of patients, the disease first manifests as muscle weakness in the feet (= drop foot) and hands, followed by atrophy and paralysis. The weakness is accompanied by muscle cramps and spasms, often in the arms and shoulders. There may also be tremors.

After one or two years of evolution, the disorders of a bulbar involvement (described below) appear.

In 20% of patients, the disease first presents with symptoms of damage to the medulla oblongata, namely difficulty speaking (= difficulty in speaking, weak voice, muffled), which is called dysarthria and difficulty chewing and swallowing (dysphagia). Subsequently, patients present with the muscle weakness of the limbs and trunk that we have described above:

• Decreased coordination and dexterity
• Significant fatigue
• emaciation
• Constipation
• Pain, especially muscle pain
• Sialorrhée (hypersalivation)
• Sleeping troubles
• Difficulty in breathing, due to the progressive paralysis of the breathing muscles in the thorax. This damage occurs later in the course of the disease
• Impairment of cognitive functions manifested in 30 to 50% of patients, most often minimal changes in personality, irritability, obsessions, reduced self-criticism and problems with organization and execution of tasks. In about 15% of cases, there is frontotemporal dementia, with significant disorganization and disinhibition

People at risk

Men are slightly more affected than women.

Risk factors

There are hereditary forms of Charcot’s disease (approximately 10% of cases). Age is also a risk factor.