[Update on fundamental and clinical research in amyotrophic lateral sclerosis].
This paper from a group of French experts in amyotrophic lateral sclerosis (ALS) presents an update of recent advances in fundamental, epidemiological and clinical research in ALS. Recent development in the pathogenesis of ALS suggests that motor neuron degeneration is a multifactorial and noncell autonomous process. Research has been advanced through the identification of the TAR-DNA-binding protein (TDP-43) as a common neuropathological marker of ALS and frontotemporal lobar degeneration with ubiquitin-positive inclusions. Recently, mutations in the TDP-43 gene have been described in individuals with familial and sporadic ALS. Fundamental research in ALS is expected to lead to the disclosure of new diagnostic markers and therapeutic targets. A small trial has suggested that lithium carbonate may slow ALS progression but larger trials will be needed to confirm these results.
Centre SLA de Paris, fédération des maladies du système nerveux, groupe hospitalier Pitié-Salpêtrière, AP-HP, 47, boulevard de l'Hôpital, 75651 Paris cedex 13, France. pierre-francois.pradat@psl.ap-hop-paris.fr
P-F
PF
Pradat
J-P
JP
Camdessanché
L
L
Carluer
P
P
Cintas
P
P
Corcia
V
V
Danel-Brunaud
A
A
Echaniz-Laguna
J
J
Gonzalez
G
G
Nicolas
N
N
Vandenberghe
A
A
Verschueren
D000690:Amyotrophic Lateral Sclerosis / Q000453:epidemiology / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*; D004594:Electrophysiology; D006801:Humans; D018696:Neuroprotective Agents / Q000627:therapeutic use; D018529:Nutritional Support; D015656:Respiratory Mechanics / Q000502:physiology
D018696:Neuroprotective Agents