title: Neurological PRESentations in Sickle Cell Patients Are Not Always Stroke: A Review of Posterior Reversible Encephalopathy Syndrome in Sickle Cell Disease.
abstract: Acute neurological changes in sickle cell disease (SCD) patients often raise the suspicion for stroke. Posterior reversible encephalopathy syndrome (PRES) can mimic stroke in its clinical presentation. We aimed to (i) review the PRES literature in SCD patients including clinical presentation, risk factors, pathophysiology, and management and (ii) elucidate the distinction between PRES and stroke in SCD. The exact pathophysiology of PRES in SCD remains elusive but is likely multifactorial and related to sickling, ischemia, and chronic anemia predisposing to vasogenic edema. PRES and stroke in SCD are distinguishable conditions. Our review may help elucidate a clinical approach to this distinction.
authors.affiliation: Division of Pediatric Hematology and Oncology, Department of Pediatrics, McMaster Children's Hospital, McMaster University, Hamilton, Ontario, Canada.
authors.forename: Ziad
authors.initials: Z
authors.lastname: Solh
authors.affiliation: Division of Neurosurgery, Department of Surgery, The Ottawa Hospital, University of Ottawa, Ottawa, Ontario, Canada.
authors.forename: Michael S
authors.initials: MS
authors.lastname: Taccone
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics, Health Sciences Centre, University of Manitoba, Winnipeg, Manitoba, Canada.
authors.forename: Samantha
authors.initials: S
authors.lastname: Marin
authors.affiliation: Division of Pediatric Hematology and Oncology, Department of Pediatrics, McMaster Children's Hospital, McMaster University, Hamilton, Ontario, Canada.
authors.forename: Uma
authors.initials: U
authors.lastname: Athale
authors.affiliation: Division of Pediatric Hematology and Oncology, Department of Pediatrics, McMaster Children's Hospital, McMaster University, Hamilton, Ontario, Canada.
authors.forename: Vicky R
authors.initials: VR
authors.lastname: Breakey
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications*; D003937:Diagnosis, Differential; D006801:Humans; D054038:Posterior Leukoencephalopathy Syndrome / Q000175:diagnosis* / Q000209:etiology*; D020521:Stroke / Q000175:diagnosis
keywords: posterior reversible encephalopathy syndrome; sickle cell disease; stroke; transfusion