Perampanel Single Ascending Dose Transcranial Magnetic Stimulation Biomarker Study in Amyotrophic Lateral Sclerosis

To measure the effect on motor threshold (MT) by transcranial magnetic stimulation (TMS)
after a single dose of perampanel at two dose levels.

Inclusion Criteria:

1. A probable laboratory supported, probable or definitive ALS diagnosis by revised El
Escorial criteria.

2. Sporadic or familial ALS.

3. Ages of 18-70.

4. Agree to use reliable contraception

5. Randomization will occur after a baseline MT has been established; any subject in whom
a MT cannot be established will be excluded.

6. Caregiver willing to report adverse behavioral events. -

Exclusion Criteria:

1. History of epilepsy.

2. Significant laboratory abnormality (AST or alanine aminotransferase >3x upper limit of
normal, or glomerular filtration rate <60)

3. History of aggressive behavior.

4. Subject unwilling to abstain from alcohol for 2 weeks after each dosing.

5. History of drug abuse in the last 5 years

6. Other severe medical conditions, including psychiatric conditions, which would cause
an increased risk in the opinion of the investigator, including but not limited to
renal failure and liver failure.

7. Skull defect or other physical contraindication for TMS

8. Pacemaker or implanted defibrillator

9. Inability to take study capsule by mouth

Females only: Subject is pregnant [as confirmed by a positive serum human chorionic
gonadotropin (hCG) test for females of reproductive potential (FRP) only], subject is
breastfeeding, or subject is of reproductive potential and does not agree to follow use of
reliable contraception.