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Expert Rev Neurother. 2017 Mar;17(3):263-276. doi: 10.1080/14737175.2016.1227705. Epub 2016 Sep 20.

Management and therapeutic perspectives in amyotrophic lateral sclerosis.

Author information

1
a Department of Neurology , Neuro-Muscular Unit and ALS Center, CHU de Bordeaux, groupe hospitalier Pellegrin , Bordeaux , France.
2
b Department of Neurology , ALS center, Centre de compétence SLA-fédération Tours-Limoges, CHU de Limoges , Limoges , France.
3
c Department of Neurology, CHU Poitiers , University of Poitiers , Poitiers , France.
4
d Department and Laboratory of Neurology, Centre de Référence 'neuropathies périphériques rares' , University Hospital of Limoges , Limoges , France.
5
e Department of Neurology, ALS center , Centre de compétence SLA-fédération Tours-Limoges, CHU de Tours , Tours , France.

Abstract

Amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disorder affecting both upper and lower motor neurons. Despite much research and effort, no clear insights into a unifying hypothesis for the pathogenesis has so far emerged for this disease. Areas covered: We review the main pathophysiological hypotheses and the potential therapeutic targets in ALS, as well as the management of these patients (in order to improve their survival and quality of life). Expert commentary: ALS is a complex neurodegenerative disease, these days considered as a multisystem disorder with predominant motor symptoms (and various clinical forms). Further comprehension of the pathophysiology of this disease is required, although pathophysiological mechanisms (such as TDP-43) show promise in the search for new therapies. There is still no curative treatment for ALS, but the emergence of multidisciplinary specialized ALS clinics has increased both the quality of life and the survival of these patients.

KEYWORDS:

ALS; Amyotrophic lateral sclerosis; animal models; management; riluzole; treatment

PMID:
27644548
DOI:
10.1080/14737175.2016.1227705
[Indexed for MEDLINE]

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