This neurosteroid, being a positive allosteric modulator of GABAA receptors, is also used in the treatment of epilepsy related to PCDH19 clusters, status epilepticus accompanying Dravet syndrome, and infant convulsions following hypoxic brain damage. Here, PCDH19 is linked to encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.