title: Management and outcomes of malignant posterior reversible encephalopathy syndrome.
abstract: BACKGROUND
  Recognition of severe forms of posterior reversible encephalopathy syndrome (PRES) has improved. Management of these patients remains challenging, particularly in patients with the combination of edema and hemorrhage.
  
  
  METHODS
  A prospective inpatient neuro-intensive care database was queried for patients with PRES. Malignant PRES was diagnosed by clinical assessments (GCS less than 8 and clinical decline despite standard medical management for elevated intracranial pressure) and radiographic criteria (edema with associated mass effect; brain hemorrhage exerting mass effect; effacement of basal cisterns, transtentorial, tonsillar, or uncal herniation). Malignant PRES was defined as: radiology studies consistent with PRES; GCS less than 8; and clinical decline despite standard elevated intracranial pressure management.
  
  
  RESULTS
  Five cases were identified over a 4 year interval. The following contributing conditions were also present: chemotherapy (1), systemic lupus erythematosis (2), pregnancy (1), and methamphetamines (1). Neurocritical care interventions included: hyperosmolar therapy (5), anticonvulsants (5), management of coagulopathy (5), and ventilatory support (5). Neurosurgical interventions included: craniectomy (5), hematoma evacuation (3), and external ventricular drain (4). Brain biopsy was performed in 5 patients and was negative for vasculitis, demyelinating disease, tumor, or infection. Cyclophosphamide was administered to the two patients with SLE. With long-term follow up, all patients achieved good functional outcomes (modified Rankin score 1-2).
  
  
  CONCLUSIONS
  In contrast to historical reports of high mortality rates (16-29%) for severe and hemorrhagic PRES variants, we had no fatalities and observed favorable functional outcomes with intracranial pressure monitoring and craniectomy for malignant PRES cases who fail medical ICP management.
authors.affiliation: Department of Neurosurgery, The Permanente Medical Group, Kaiser Sacramento Medical Center, Sacramento, USA. Electronic address: Paul.t.akins@kp.org.
authors.forename: Paul T
authors.initials: PT
authors.lastname: Akins
authors.affiliation: Department of Neurosurgery, The Permanente Medical Group, Kaiser Sacramento Medical Center, Sacramento, USA.
authors.forename: Yekaterina
authors.initials: Y
authors.lastname: Axelrod
authors.affiliation: Department of Neurosurgery, The Permanente Medical Group, Kaiser Sacramento Medical Center, Sacramento, USA.
authors.forename: James W
authors.initials: JW
authors.lastname: Silverthorn
authors.affiliation: Department of Neurosurgery, The Permanente Medical Group, Kaiser Sacramento Medical Center, Sacramento, USA.
authors.forename: Kern
authors.initials: K
authors.lastname: Guppy
authors.affiliation: Department of Neurosurgery, The Permanente Medical Group, Kaiser Sacramento Medical Center, Sacramento, USA.
authors.forename: Amit
authors.initials: A
authors.lastname: Banerjee
authors.affiliation: Department of Neurosurgery, The Permanente Medical Group, Kaiser Sacramento Medical Center, Sacramento, USA.
authors.forename: Mark W
authors.initials: MW
authors.lastname: Hawk
mesh_terms: D000328:Adult; D002543:Cerebral Hemorrhage / Q000150:complications / Q000175:diagnosis; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008875:Middle Aged; D054038:Posterior Leukoencephalopathy Syndrome / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D011247:Pregnancy; D011446:Prospective Studies; D016896:Treatment Outcome
chemical_list: D003520:Cyclophosphamide
keywords: Cerebral hemorrhage; Craniectomy; Eclampsia; PRES; Posterior reversible encephalopathy syndrome