7 research outputs found
Management of amyotrophic lateral sclerosis in clinical practice: Results of the expert consensus using the Delphi methodology
International audienceAmyotrophic lateral sclerosis (ALS) is a rare disease characterized by a progressive and irreversible degeneration of upper and lower motor neurons leading to death. In France, limited data exist describing the criteria used in clinical practice for diagnosis and follow-up, and how novel therapies may fit in. The objective of this Delphi panel was to obtain an overview of current French practices in ALS diagnosis, management, and follow-up by determining the scales and criteria used in clinical practice outside of clinical trials, as well as the place of a future treatment like AMX0035, acting on endoplasmic reticulum (ER) stress and mitochondrial dysfunction, in the current therapeutic strategies. A questionnaire was administered to 24 ALS healthcare providers practicing in ALS centers in France. Two rounds of remote voting were organized, before proposition of final consensus statements. Consensus was considered reached when at least 66% of the voters agreed. Consensus were obtained to define the new Gold Coast criteria as the ones used in clinical practice to establish the diagnosis of ALS, thus replacing the revised El Escorial criteria, considered too complex and now mainly used to characterize the patient populations to be included in clinical trials. The clinical factors considered to establish ALS diagnosis are mainly the demonstration of progression of the motor deficit and elimination of differential diagnoses. The ALSFRS-R scale is used in daily clinical practice to assess patient's functional impairment in terms of number of points lost, with the bulbar, respiratory, and fine motor subscores being the most important to evaluate independently. A critical medical need was identified regarding the provision of new therapeutic alternatives in ALS. The panel members would support the earliest management of patients. In this landscape, based on data from a very encouraging phase II (Centaur trial), AMX0035 represents a new tool of choice in current treatment strategies for all patients for whom experts are confident in the diagnosis of ALS, in combination with riluzole. These results will need to be confirmed by the ongoing phase III trial (Phoenix trial)
Experimental evidence of shock wave measurements with low-velocity (<100 m s â1 ) and fast dynamics (<10 ns) capabilities using a coupled photonic Doppler velocimetry (PDV) and triature velocity interferometer system for any reflector (VISAR) diagnostic
International audienceWe present a series of shock-wave measurements on aluminum based on the use of a simultaneous Photon Doppler Velocimetry (PDV) and triature velocity interferometer system for any reflector. Our dual setup can accurately measure shock velocities, especially in the low-speed range (<100 m s â1 ) and fast dynamics (<10 ns) where measurements are critical in terms of resolution and unfolding techniques. Especially, the direct comparison of both techniques at the same measurement point helps the physicist in determining coherent settings for the short time Fourier transform analysis of the PDV, providing increased reliability of the velocity measurement with a global resolution of few m s â1 in velocity and few ns FWHM in time. The advantages of such coupled velocimetry measurements are discussed, as well as new opportunities in dynamic materials science and applications