Charcot affects today 5 000 to 7 000 people in France, with an annual incidence (number of new cases per year) close to 2.5 per 100 000 inhabitants. It belongs to the group of rare diseases. At the Meeting, fifty patients is followed each year.
Since November 2017, the department of Neurological Diseases Rare of CHU-South Meeting, led by Dr. Ariane Choumert, has been labelled a Centre of Resources and Skills for ALS and Rare Diseases of the Motor Neuron. The management of these patients is multidisciplinary and multi-professional, focusing on the symptoms of neurological, orthopedic, respiratory, nutritional, and taking into account the psychological and social consequences of the disease. This support must be put in place as soon as possible in order to improve the prognosis and quality of life of patients, but also to anticipate the complications. This work is a close collaboration between the services of pneumology and the Transversal Unit of Clinical Nutrition.
The SLA starts on average at the age of 60-65 years, with a very slight male preponderance. The disease causes a progressive paralysis of the muscles of voluntary body, with damage to a random and non-mandatory members, the motricity oro-facial with swallowing disorders and respiratory conditions.
The diagnosis of ALS is difficult, particularly in the forms of beginning where the signs are incomplete. The degree of evolution of the disease can be evaluated by clinical tests and simple by balance sheets, motors, nutritional, and respiratory regular, in order to assess the degree of involvement and tailor the care. The evolving profile of the disease varies from one patient to another and also in a same patient over time.
Two types of motor neurons located at different levels of the nervous system are concerned, the motor neurons central neurons and peripheral motor. The diagnosis relies on the expertise of the neurologist who will look for clinical signs of impairment of the motor neuron of the central or peripheral, and perform an electromyogram which search for the faults of the suffering of the neuron device. Other tests are performed in order to eliminate differential diagnoses.