Trial on the Biological and Clinical Effects of Acetyl-L-carnitine in ALS

Inclusion Criteria: 1. Age 18+; 2. ALS diagnosis according to the Gold Coast Criteria; 3. Disease duration \< 24 months from symptom onset, as indicated by limb weakness or bulbar symptoms, at the randomization/baseline visit\*; 4. Self-sufficiency \[Satisfactory bulbar and spinal function (score 3+ on the ALSFRS-R for swallowing, cutting food and handling utensils, and walking)\]; 5. Satisfactory respiratory function (FVC ≥80% of predicted); 6. Documented progression of symptoms as measured by the ALSFRS-R scale. Disease progression rate (DFS) must be\>= 0.33. DFS =(48- ALSFRS-R at screening)/months from onset to screening. 7. Ability to understand and comply with the study requirements; 8. Ability to give written informed consent personally or, as an alternative, via a legally authorized representative; 9. Treatment with riluzole 50 mg twice/day for at least 4 weeks prior to randomization visit; 10. Intact cognitive function, again determined by the Principal Investigator. * The qualifying first symptoms of ALS are limited to manifestations of weakness in extremity, bulbar, or respiratory muscles. Cramps, fasciculations, or fatigue should not be taken in isolation as a first symptom of ALS. Exclusion Criteria: 1. Antecedent polio infection or other active infection; 2. Motor neuron disease (MND) other than ALS; 3. Involvement of other systems possibly determining a functional impairment (as measured by the endpoints) for the entire duration of the study; 4. Other severe clinical conditions (e.g., cardiovascular disorders, neoplasms) with an impact on survival or functional disability in the next 12 months; 5. Previous use of ALCAR for any reason; 6. Poor compliance with previous treatments; 7. Other experimental treatments in the three months prior to the screening visit (if a subject is receiving another experimental drug, a 3-month wash-out period before participating in the present clinical trial will be required); 8. Women who are lactating or able to become pregnant (e.g. who are not post-menopausal, surgically sterile, or using inadequate birth control) and men unable to practice contraception for the duration of the treatment and three months after its completion; 9. Inability to understand and comply with the study requirements; 10. Unwillingness or inability to take riluzole.