Arimoclomol in Amyotropic Lateral Sclerosis

Screening of up to 4 weeks Treatment of up to 76 weeks

Inclusion Criteria:

- Subject meets revised El Escorial criteria for clinically possible, clinically
probable / Clinically probable ALS laboratory-supported or clinically definite ALS, or
familial ALS

- 18 months or less since first appearance of weakness (e.g. limb weakness, dysarthria,
dysphagia, shortness of breath).

- ALSFRS-R equal to or above 35 and erect (seated) SVC% predicted equal to or above 80%
at screening

Exclusion Criteria:

- Tracheostomy or use of non-invasive ventilation for more than 2 hours during waking
hours at the time of screening or baseline

- pregnant or breast-feeding

- current or anticipated use of diaphragmatic pacing

- Any other relevant medically significant condition which could present risk to the
subject or interfere with the assessment of safety or has an increased risk of causing
death during the trial