Status epilepticus amauroticus and posterior reversible encephalopathy syndrome in children

Transient cortical visual impairment is a common aura of occipital lobe seizures. However, status amauroticus for hours has rarely been described as a manifestation of occipital lobe status epilepticus.

To describe status amauroticus caused by focal status epilepticus of occipital lobe origin in two children with MRI findings consistent with posterior reversible encephalopathy syndrome.

Retrospective review of clinical records of two children who developed status amauroticus in association with posterior reversible encephalopathy syndrome, identified through the EEG database at British Columbia Children's Hospital.

Case 1: A 15-year-old boy with chronic renal failure on hemodialysis developed acute headache, hypertension, vomiting, and blurred vision followed by severe visual impairment and a secondarily generalized clonic seizure. EEG demonstrated independent right and left occipital seizures. After phenytoin therapy, visual acuity was 20/20 within 48 hours. Case 2: A 10-year-old girl with beta-thalassemia treated with allogenic bone marrow transplantation developed acute headache, hypertension, severe visual impairment (light perception), encephalopathy, and intermittent dysconjugate gaze. EEG showed status epilepticus of biposterior quadrant origin. After phenytoin treatment, vision normalized within 72 hours.

Reversible status amauroticus because of occipital lobe status epilepticus may be the presenting symptom of posterior reversible encephalopathy syndrome and EEG recording should be performed.