A Study With SAGE-547 for Super-Refractory Status Epilepticus

Inclusion Criteria:

- Subjects two (2) years of age and older

- Subjects who have:

- Failed to respond to the administration of at least one first-line agent (e.g.,
benzodiazepine or other emergent initial anti-epileptic drug [AED] treatment),
according to institution standard of care, and;

- Failed to respond to at least one second-line agent (e.g., phenytoin,
fosphenytoin, valproate, phenobarbital, levetiracetam or other urgent control
AED), according to institution standard of care, and;

- Not previously been administered a third-line agent but have been admitted to an
intensive care unit with the intent of administering at least one third-line
agent for at least 24 hours; or who have previously failed zero, one or more wean
attempts from third-line agents and are now on continuous intravenous infusions
of one or more third-line agent and in an EEG burst or seizure suppression
pattern; or who have previously failed one or more wean attempts from third-line
agents and are now either not on a continuous intravenous infusion of at least
one third-line agent or are on a continuous intravenous infusion of one or more
third-line agent but not in an EEG burst or seizure suppression pattern

Exclusion Criteria:

- Subjects with SRSE due to anoxic/hypoxic encephalopathy with highly malignant/
malignant EEG features

- Children (subjects aged less than 17 years) with an encephalopathy due to a rapidly
progressing underlying neurological disorder

- Subjects who have any of the following:

1. a glomerular filtration rate (GFR) low enough to warrant dialysis but for
whatever reason, dialysis is not planned or non-continuous dialysis planned (that
would not adequately remove Captisol®);

2. severe cardiogenic or vasodilatory shock requiring two or more pressors that is
not related to third-line agent use;

3. fulminant hepatic failure;

4. no reasonable expectation of recovery (for instance, a likely outcome is
persistent vegetative state) or life-expectancy, in the experience of the
investigator, is less than 30 days.

- Subjects who are being administered more than three third-line agents concomitantly or
in whom the qualifying wean cannot be completed per protocol