ALS is a devastating neurodegenerative disorder with no effective treatment. In 
the present study, we found that daily doses of lithium, leading to plasma 
levels ranging from 0.4 to 0.8 mEq/liter, delay disease progression in human 
patients affected by ALS. None of the patients treated with lithium died during 
the 15 months of the follow-up, and disease progression was markedly attenuated 
when compared with age-, disease duration-, and sex-matched control patients 
treated with riluzole for the same amount of time. In a parallel study on a 
genetic ALS animal model, the G93A mouse, we found a marked neuroprotection by 
lithium, which delayed disease onset and duration and augmented the life span. 
These effects were concomitant with activation of autophagy and an increase in 
the number of the mitochondria in motor neurons and suppressed reactive 
astrogliosis. Again, lithium reduced the slow necrosis characterized by 
mitochondrial vacuolization and increased the number of neurons counted in 
lamina VII that were severely affected in saline-treated G93A mice. After 
lithium administration in G93A mice, the number of these neurons was higher even 
when compared with saline-treated WT. All these mechanisms may contribute to the 
effects of lithium, and these results offer a promising perspective for the 
treatment of human patients affected by ALS.