Table 2 Inclusion criteria for patient participation in the Extension Study

1. Completed Visit 6 of the IB1001-201 / IB1001-202 / IB1001-203 Parent Study

2. The Principal Investigator determines further treatment with IB1001 to be in patient’s best interest

3. Written informed consent signed by the patient and/or their legal representative/parent/ impartial witness for participation in the Extension Phase

4. Patients are willing to continue to remain without the following prohibited medication from Visit 6 throughout the duration the Extension Phase:

 a) Aminopyridines (including sustained-release form);

 b) N-Acetyl-DL-Leucine (e.g. Tanganil®);

 c) N-Acetyl-L-Leucine (prohibited if not provided as investigational medicinal product [IMP]);

 d) Riluzole;

 e) Gabapentin;

 f) Varenicline;

 g) Chlorzoxazone;

 h) Sulfasalazine;

 i) Rosuvastatin.