Prolonged refractory status epilepticus with early and persistent restricted hippocampal signal MRI abnormality.

We report a series of four children with a prolonged refractory status epilepticus with an early persistent and restricted hippocampal signal MRI abnormality but otherwise no proven etiology. The mean duration of status epilepticus was 53 days (range, 20-91 days) with a mean length of stay in Pediatric Intensive Care Unit (PICU) of 2 months (range, 26-115 days). Neurological outcome showed epilepsy disease in all, and mild to severe disability. In long-term follow-up, the initial MRI signal abnormality developed into a cortical atrophy in all cases. The establishment of the diagnosis, etiology, and options for treatment are discussed.