• Subjects must provide a signed and dated informed consent form (ICF) to participate in the study. Subjects must be able (in the judgment of the Investigator) to understand the nature of the study and all risks involved with participation in the study. Subjects must be willing to cooperate and comply with all protocol restrictions and requirements.

• Subjects will be male or female, ≥ 18 to 75 years of age at the time the ICF is signed.

• Subjects will be diagnosed with Definite ALS or Probable ALS according to the El Escorial revised criteria for the diagnosis of ALS.

• Subjects with a baseline score ≥ 2 points on each individual item of the ALSFRS- R at screening and baseline visits.

• Subjects have a screening and baseline %forced vital capacity (FVC) ≥ 70%.

• Subjects with 1- to 4-point decline for 8 weeks (±7 days) in ALSFRS-R total score between screening and baseline visits.

• Subjects whose first symptom of ALS has occurred within 2 years of providing written informed consent.