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Also called amyotrophic lateral sclerosis (ALS), Charcot disease is a rare neurodegenerative disease that affects motor neurons. What are the first signs of the disease? Let’s take stock with Dr Wilfrid Casseron, neurologist in Aix-en-Provence.
A rare disease, amyotrophic lateral sclerosis, which is more commonly called Charcot disease, is scary. With a poor prognosis, it leads to progressive muscle weakness, through damage to motor neurons.
Charcot disease, a rare pathology
To discuss the first symptoms of Charcot’s disease in the clearest possible way, Doctissimo interviewed Dr Wilfrid Casseron, a neurologist in Aix-en Provence. “Although amyotrophic lateral sclerosis is, it is true, a serious pathology, it remains quite rare and affects an average of 7,000 patients per year in France, ten times less than multiple sclerosis, for example.” he immediately recalls.
“As the first symptoms of the disease are quite general, it is important to remember that it is important to consult a doctor if you notice them, but not to panic unnecessarily.” he emphasizes again.
What are the first symptoms of the disease?
Patients affected by the disease often notice asymmetrical muscle weakness first. “In addition to this asymmetric muscle weakness, there may also be muscle atrophy and fasciculations. Fasciculations are spontaneous movements of the muscles, a bit like having worms under the skin. These three symptoms can be accompanied by cramps” indicates the specialist.
According to the expert, it is often the fasciculations that worry the patients who come to see him. “They did research on the internet and found that it could be linked to Charcot’s disease and consulted very worried. In reality, there are many other causes that can explain them“.
No sensory signs in Charcot disease
In some patients, it will be the ENT area that will be affected. “In 30% of cases, we find what we call a bulbar form of the disease. At this time, the patient presents difficulties with swallowing, speaking, chewing, with hypersalivation. There may be false routes and voice modification” further indicates Dr Casseron.
“But it’s something very marked, it’s not when it happens once and it disappears, it’s what we call paralytic dysarthria“. Finally, specifies the neurologist, “as it is the motor neurons which are affected, there are therefore never any sensory signs, in Charcot’s disease: no tingling for example.
Consult if these disorders appear
Wilfrid Casseron finally advises patients who present this type of disorder to consult a doctor quickly. “A neurologist will then prescribe the necessary tests to understand what is happening. Most often, he will order an electromyogram, with a blood test and an MRI of the brain and spinal cord. he further details.
“The differential diagnosis that is frequently found is compression of the cervical cord, which gives symptoms similar to those described previously. he concludes.