Prolonged refractory status epilepticus with early and persistent restricted hippocampal signal MRI abnormality
Laurent Chevret1, Beatrice Husson, Seraphin Nguefack
1Pediatric Intensive Care Unit, CHU Bicêtre, 78, rue du Gal Leclerc, 94275 Le Kremlin Bicêtre, France. laurent.chevret@bct.aphp.fr
Insights
Four children experienced prolonged refractory status epilepticus with early hippocampal MRI abnormalities. This condition led to persistent epilepsy and disability, with MRI changes evolving into cortical atrophy over time.
Area of Science:
- Pediatric Neurology
- Neuroradiology
- Epileptology
Background:
- Status epilepticus (SE) in children can be prolonged and refractory, posing significant management challenges.
- Identifying the etiology of SE is crucial for effective treatment and prognosis.
- Hippocampal abnormalities on MRI are common in SE but their early, persistent nature warrants further investigation.
Observation:
- A series of four pediatric patients presented with prolonged refractory status epilepticus (mean 53 days).
- All patients exhibited early, persistent, and restricted hippocampal signal abnormalities on MRI.
- No definitive etiology was identified for the SE in these cases.
Findings:
- Patients required extended stays in the Pediatric Intensive Care Unit (PICU) (mean 2 months).
- All children were left with epilepsy and varying degrees of neurological disability.
- Long-term follow-up revealed the initial hippocampal MRI signal abnormality progressed to cortical atrophy in all cases.
Implications:
- This case series highlights a distinct pattern of SE with specific MRI findings.
- The findings suggest a potential neurodegenerative component or a unique disease process.
- Further research is needed to elucidate the etiology and optimize treatment strategies for this severe pediatric epilepsy syndrome.
Abstract:
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.
