Neurological PRESentations in Sickle Cell Patients Are Not Always Stroke: A Review of Posterior Reversible Encephalopathy Syndrome in Sickle Cell Disease

• Published in: Pediatric blood & cancer Vol. 63; no. 6; pp. 983 - 989
• Main Authors: Solh, Ziad, Taccone, Michael S., Marin, Samantha, Athale, Uma, Breakey, Vicky R.
• Format: Journal Article
• Language: English
• Published: United States Blackwell Publishing Ltd 01.06.2016; Wiley Subscription Services, Inc
• Subjects: Anemia, Sickle Cell - complications; Diagnosis, Differential; Hematology; Humans; Oncology; Pediatrics; Posterior Leukoencephalopathy Syndrome - diagnosis; Posterior Leukoencephalopathy Syndrome - etiology; posterior reversible encephalopathy syndrome; Sickle cell disease; Stroke; Stroke - diagnosis; transfusion; posterior reversible encephalopathy syndrome; transfusion; stroke; sickle cell disease
• ISSN: 1545-5009; 1545-5017
• DOI: 10.1002/pbc.25932

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.