Table 1.

Human pharmacological trials in amyotrophic lateral sclerosis. Summary of all clinical trials mentioned in the text with indication of the exact doses applied, the outcome measure, duration of intake as well as results.

Drug and doses	Outcome measure	Duration of intake	Results	Remark	Reference
Alpha-tocopherol 500 mg/day	Deterioration of function, measured by the modified Norris limb scale; survival	12 months	No effect of survival or motor function	Patients with riluzole plus alpha-tocopherol remained longer in milder states of the ALS Health State scale	Desnuelle et al. (2001)
Alpha-tocopherol 5000 mg/day	Survival, calculating time to death, tracheostomy or permanent assisted ventilation, according to the WFN-Criteria of clinical trials	18 month	No influence by alpha-tocopherol intake	Functional assessments showed a marginal trend in favour of vitamin E, without reaching significance.	Graf et al. (2005)
Alpha-tocopherol 15 mg/day	Risk of developing ALS	10 years	Less than half the risk of death from ALS than non-users.	Vitamin E supplementation could have a role in ALS prevention	Ascherio et al. (2005)
Alpha-tocopherol 22 mg/day	Risk of developing ALS	12 month	High intake of vitamin E is associated with a 50-60% decreased risk of developing ALS	Intake of moderate vitamin E dosages decreased the risk of ALS/MND as a preventive measure	Veldink et al. (2007)
Minocycline 400 mg/day	Difference in rate of change in the revised ALS functional rating scale (ALSFRS-R)	9 month	ALSFRS-R score deterioration was faster in the minocycline group than in the placebo group	Minocycline has a harmful effect on patients with ALS	Gordon et al. (2007)
High energy diet	Investigation whether lipid contents may have an impact on disease progression and survival	12 month	Frequency of hyper lipidemia as revealed by increased plasma levels of total cholesterol or LDL was twofold higher in patients with ALS than in control subjects.	Hyperlipidemia is a significant prognostic factor for survival of patients with amyotrophic lateral sclerosis.	Dupuis et al. (2008)
Arimoclomol 300 mg/day	Safety and tolerability	9 month	Well tolerated and safe	Arimoclomol was shown to be safe. It crosses the blood-brain barrier.	Cudkowicz et al. (2008)
IGF-1 0.1 mg/kg/day	Safety and efficacy	9 month	No significant difference between treated groups	IGF-1 appeared to be safe and well tolerated.	Borasio et al. 1998)
IFG-1 0.1 mg/kg/day	Change in the manual muscle testing score	2 years	No significant difference between treated groups	More adverse reactions with thromboembolic events in the treated group	Sorenson et al. (2008)
Lithium 300 mg/day, plasma levels ranging from 0.4 to 0.8 mEq/liter	Progression of the ALSFRS and Norris scale	15 month	Lithium delays ALS progression in human patients.	Decreases in ALS-FRS-R and Norris scales were not statistically significant in the group treated with lithium.	Fornai et al. 2008)