Safety and Biomarker Study of EPI-589 in Subjects With ALS

Open label with 30-day run in phase to establish baseline parameters and a 90-day withdrawal
phase to determine duration of treatment response.

Inclusion Criteria:

- Dx. of ALS by E1 Escorial Criteria

- FVC >= 70% years of age

- Weakness onset within 3 years

- Agreement to use contraception when age appropriate

- Willingness and ability to comply with study procedures

- Stable regimen of dietary supplements and /or riluzole for at least 30 days prior to
study

- Absention from use of other investigative or non-approved drugs

- Subject must be able to swallow size 0 tablets

Exclusion Criteria:

- Allergy to EPI-589

- Use of ventilation

- Participation in other intervention studies

- Inability to undergo MRI or MRS

- Dx with any other neurologic disease

- Malignancy within the past 2 years

- Hx. of stroke

- Hx. of brain injury

- Hepatic insufficiency (LFTs > 3 xs ULN)

- Renal insufficiency requiring dialysis

- End stage cardiac failure

- Participation in a trial of a device, drug or other tx. for ALS within 3 months