title: Status epilepticus amauroticus and posterior reversible encephalopathy syndrome in children.
abstract: BACKGROUND
  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.
  
  
  OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Division of Neurology, Department of Pediatrics, British Columbia Children's Hospital, University of British Columbia, Vancouver, Canada.
authors.forename: Valeria L
authors.initials: VL
authors.lastname: Muro
authors.forename: Samuel
authors.initials: S
authors.lastname: Yip
authors.forename: Linda
authors.initials: L
authors.lastname: Huh
authors.forename: Mary B
authors.initials: MB
authors.lastname: Connolly
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000494:pharmacology; D001766:Blindness / Q000188:drug therapy / Q000209:etiology*; D016026:Bone Marrow Transplantation / Q000009:adverse effects; D002648:Child; D038524:Diffusion Magnetic Resonance Imaging; D004569:Electroencephalography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008140:Lorazepam / Q000494:pharmacology; D008297:Male; D009778:Occipital Lobe / Q000503:physiopathology*; D010672:Phenytoin / Q000494:pharmacology; D054038:Posterior Leukoencephalopathy Syndrome / Q000150:complications* / Q000188:drug therapy / Q000209:etiology; D051437:Renal Insufficiency / Q000150:complications; D012189:Retrospective Studies; D012640:Seizures / Q000188:drug therapy / Q000209:etiology*; D014057:Tomography, X-Ray Computed; D017086:beta-Thalassemia / Q000601:surgery
chemical_list: D000927:Anticonvulsants; D010672:Phenytoin; D008140:Lorazepam