It has been suggested that amyotrophic lateral sclerosis (ALS), a neurodege
nerative disorder resulting in motor neuron death, is associated with oxida
tive damage induced by free radicals. Our study aimed to get an assessment
of the blood oxidative stress status in a population of 167 ALS patients (a
ged 59+/-13 years), treated or not with riluzole, compared with 62 age-matc
hed healthy control subjects (aged 60+/-11 years) simultaneously included i
n the study. We determined the level of plasma lipid peroxidation (thiobarb
ituric acid-reactive substances, TBARS); the status of the major lipophilic
plasma antioxidant defenses (vitamin E, vitamin A and beta-carotene); the
activities of erythrocyte Cu,Zn-superoxide dismutase (Cu,Zn-SOD) and of pla
sma and erythrocyte glutathione peroxidase (GSH-Px). Plasma selenium was al
so determined as a trace element essential to the activity of the GSH-Px. I
n comparison with controls, we observed in ALS patients (mean+/-S.D.) signi
ficantly higher TEARS values (ALS=1.34+/-0.28 mu mol/l; controls=1.11+/-0.2
0 mu mol/l) and a significant enhancement of the erythrocyte SOD activity (
ALS=710+/-114 U/g Hb; controls=667+/-93 U/g Hb). No differences were observ
ed for selenium level, GSH-Px activity, plasma vitamin E, beta-carotene and
Vitamin A concentrations. These data confirm the presence of an oxidative
stress in blood of ALS patients. The elevated plasma TEARS, without any def
iciency in plasma lipophilic antioxidants such as vitamin E, vitamin A and
beta-carotene, suggest an enhancement in the production of free radicals. N
o correlation was found in our study between the level of any of the blood
oxidative stress markers and the disease duration. Comparison between patie
nts treated or not with riluzole did not display any modification of the pl
asma TEARS concentration, but we observed a slight decrease of erythrocyte
SOD activity in treated patients (treated=705+/-113 U/g Hb; not treated=725
+/-118 U/g Hb), suggesting a possible activity of riluzole on the oxygenate
d free radical production. (C) 2000 Elsevier Science B.V. All rights reserv
ed.