A Double-blind Randomised, Placebo-controlled Clinical Trial to Test Ambroxol Treatment in ALS

Inclusion Criteria:

1. Must have given written informed consent before any study related assessments areperformed and must be able to understand purpose of the study, including any possiblerisks and adverse events.
2. ALS as diagnosed according to the recently proposed Gold Coast diagnostic criteria.
3. First symptom of ALS less than or equal to 18 months prior to screening. Thequalifying first symptoms of ALS are limited to manifestations of weakness inextremity, bulbar, or respiratory muscles. Cramps, fasciculations, or fatigue shouldnot be taken in isolation as a first symptom of ALS.
4. Forced vital capacity (FVC) greater than or equal to 60% of predicted value asadjusted for gender, height and age at the Screening Visit.
5. Male or female patients aged 18 years or greater (inclusive) and less than 85 years atthe time of ALS diagnosis.
6. Able to swallow liquid.
7. Able to perform reproducible pulmonary function tests
8. Female patients must be post-menopausal or sterilized or must not be breastfeeding,have no intention to become pregnant during the study, and use acceptable methods ofcontraception or abstain from intercourse.
9. Male patients who have not had a vasectomy and confirmed zero sperm count must agreeafter receiving the first dose of study drug either to use acceptable methods ofcontraception or abstain from intercourse.
10. If on riluzole, stable dosing for 30-days prior to screening.
11. Pre-study ALSFRS-R progression between disease onset and screening of greater than orequal to 0.5 points/month (calculated by ALSFRS-R total score decline from 48 dividedby the months since onset of ALS symptoms).

Exclusion Criteria:

1. Use of non-invasive ventilation (NIV) support for ALS only or gastrostomy tube at timeof screening.
2. Exposure to investigational drug within 12-weeks prior to screening.
3. At screening of any medically significant cardiac, pulmonary, GI, musculoskeletal, orpsychiatric illness that might interfere with the patient's ability to comply withstudy procedures or that might confound the interpretation of clinical safety or data.
4. Patient with a history of significant other major medical conditions based on theInvestigator's judgment.
5. Based on the investigator's judgment, patients who may have difficulty complying withthe protocol and/or any study procedures.
6. Any person who is an employee or an Investigator or Sponsor, or an immediate relativeof an Investigator.