The objective of this trial is to evaluate the efficacy and tolerability of EH301 in patients with amyotrophic lateral sclerosis. Patients with ALS are randomized to receive either EH301 or placebo daily and undergo active evaluation for 6 months.

Inclusion Criteria:

• 18 years or older
• Diagnosis of probable or definite (sporadic or familial) ALS by El Escorial criteria
• Onset of symptomatology for more than 6 months
• If female: not lactating; negative pregnancy test; agree to use an effective method of birth control throughout study

Exclusion Criteria:

• Tracheostomy, invasive ventilation, or non-invasive positive pressure ventilation
• Gastrostomy
• Evidence of major psychiatric disorder or clinically evident dementia
• Diagnosis of a neurodegenerative disease in addition to ALS
• Current medication apart from riluzole that in the opinion of the investigator would make the patient unsuitable for study participation
• Recent history (within the previous 6 months) or current evidence of alcohol or drug abuse
• Concurrent unstable disease involving any system (e.g. carcinoma other than basal cell carcinoma), any cardiac dysrhythmia, myocardial infarction, clinical or ECG signs of myocardial ischemia, cardiac insufficiency, angina symptoms, current symptoms of coronary artery disease, or any other condition that in the opinion of the investigator would make the patient unsuitable for study participation
• Baseline QTc (Bazett) > 450 msec for males and > 470 msec for females
• Known hepatitis B/C or HIV positive serology
• Renal impairment defined as blood creatinine > 2x ULN
• Hepatic impairment and/or liver enzymes (ALT or AST) > 3x ULN
• Hemostasis disorders or current treatment with oral anticoagulants
• Participated in any other investigational drug or therapy study with a non-approved medication, within the previous 3 months
• No medical insurance