2024-03-19 05:15:00
Amyotrophic lateral sclerosis (ALS), better known as Charcot diseaseconcerns 8,000 patients in France.
It is a rare neurodegenerative pathology which is characterized by the destruction of the neurons responsible for motor skills, the motor neurons.
Most often occurring between the ages of 50 and 70, it leads to progressive paralysis and the death of patients in just two to five years.
Problem, as Inserm points out, “the diagnosis of ALS is difficult to make. Indeed, the manifestations are heterogeneous at the start of the disease: weakness or cramps in an arm, a leg, swallowing or joint difficulties, etc.
Furthermore, there is no specific biomarker for the disease.
Thus, the diagnosis results from the elimination of other pathologies that could cause motor disorders, which generally takes one to two years after the onset of symptoms, delays the implementation of therapeutic measures and reduces the chances of inclusion in early-stage clinical trials. »
By testing the potential of electroencephalography, a method of cerebral exploration which measures the electrical activity of the brain using electrodes placed on the scalp, French researchers noted in patients affected by ALS “an imbalance between two types waves respectively associated with the activity of excitatory and inhibitory neurons.
This imbalance, in favor of greater activity of excitatory neurons to the detriment of inhibitory neurons, reflects cortical hyperexcitability. »
For scientists, this phenomenon is not a surprise since it had already been described.
On the other hand, the techniques to achieve this observation are difficult to implement and only work at the very beginning of the disease. Whereas electroencephalography “is very minimally invasive, very inexpensive, and can be used at different times during the disease. »
“If these initial results are confirmed, electroencephalography could in the future serve as a prognostic tool for patients already diagnosed in order to evaluate, for example, the response to drug treatment, or even as a diagnostic tool in the event of symptoms suggestive of disease,” the researchers indicate.
Hope for treatment
Furthermore, scientists observed a deficiency of norepinephrine in the brains of patients and mice with ALS and compared to healthy brains.
To verify the role of this neuromodulator, they blocked its production in healthy animals, and showed that this causes cortical hyperexcitability, like that observed in the disease.
And conversely, by administering molecules stimulating the action of norepinephrine in a mouse model of ALS, this reduced hyperexcitability and restored brain activity equivalent to that of healthy mice.
“This discovery could mark the opening of a new therapeutic avenue in ALS provided that cortical hyperexcitability is indeed associated with the progression of the disease,” concludes Caroline Rouaux of the Strasbourg Biomedicine Research Center.
“Indeed, to date, in our study we have observed an association between the two but no causal link has yet been established. This is what we will check in the coming months. »
Source : Inserm
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