Open-Label, Safety and Tolerability Extension Study of KNS-760704 in Amyotrophic Lateral Sclerosis (ALS) (CL211)

Patients who complete the Part 2 Week 28 visit in study KNS-760704-CL201 and patients with
ALS who are actively receiving RTPB
[(6R)-4,5,6,7-tetrahydro-N6-propyl-2,6-benzothiazolediamine dihydro-chloride monohydrate]
under Research IND #60,948 may be eligible to participate in this study.

Eligible patients will receive 1 tablet of KNS-760704 150 mg every 12 hours (Q12H) (300 mg
total daily dose) for up to 180 weeks.

Inclusion Criteria:

1. Patient has provided signed informed consent for this trial before the commencement of
any study-related procedure

2. Patient is actively participating in Knopp Protocol KNS-760704-CL201 and has completed
the Part 2 Week 28 visit in that study or patient is actively receiving RTPB under
Research IND #60,948

Exclusion Criteria:

1. Patient did not participate in Knopp Protocol KNS-760704-CL201 or patient did not
receive RTPB under Research IND #60,948.

2. Patient discontinued from KNS-760704-CL201 for any reason other than enrollment into
this study (applies to patients enrolled in KNS-760704-CL201 only)

3. Patient was not taking RTPB under Research IND #60,948 prior to April 30, 2009
(applies to patients enrolled under Research IND #60,948 only)