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2017 ; 12
(2
): 181-184
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Current view and perspectives in amyotrophic lateral sclerosis
#MMPMID28400790
Mathis S
; Couratier P
; Julian A
; Corcia P
; Le Masson G
Neural Regen Res
2017[Feb]; 12
(2
): 181-184
PMID28400790
show ga
Amyotrophic lateral sclerosis (ALS), identified as a distinct clinical entity by
Charcot since the end of the nineteenth century, is a devastating and fatal
neurodegenerative disorder that affects motor neurons in the brain, brainstem and
spinal cord. Survival of patients with ALS is associated with several factors
such as clinical phenotype, age at onset, gender, early presence of respiratory
failure, weight loss and treatment with Riluzole (the only disease-modifying drug
approved for this disease). Nowadays, there is still no curative treatment for
ALS: palliative care and symptomatic treatment are therefore essential components
in the management of these patients. Nevertheless, the scientific knowledge in
the field of ALS motor neuron degeneration is growing, with the prospect of new
treatments. Based on this physiopathological knowledge, several new therapeutic
targets are being studied, involving various mechanisms such as excitotoxicity,
neuroinflammation, mitochondrial dysfunction, oxidative stress, RNA metabolism
and other attractive concepts. Moreover, it is also important to identify
reliable biomarkers that will be essential components for future therapeutic
development and study design in ALS. In this review, we present the main recent
advances and promising therapeutics and biomarkers in the field of ALS.