"Critical Care A Day"
Saturday May 24, 2025
Saturday May 24, 2025
Q: Which is more common in Sickle cell disease (SCD)? - Select one
A) PRES
B) Stroke
Answer: B
Posterior reversible encephalopathy (PRES), though usually described in accelerated hypertension with its constellation of symptoms like confusion, headache, visual symptoms, and seizures, may occur in other conditions, such as endothelial dysfunction. Its most interesting association is found in pediatric SCD patients with recent transfusion or hematopoietic stem cell transplantation. No clear explanation is available.
In SCD patients, stroke is more prevalent than PRES. This is the reason many times, PRES in SCD patients is misdiagnosed as stroke (see reference # 1). These patients are also prone to early dementia.
#neurology
#hematology
#pediatrics
References:
1. Solh Z, Taccone MS, Marin S, et al. Neurological PRESentations in Sickle Cell Patients Are Not Always Stroke: A Review of Posterior Reversible Encephalopathy Syndrome in Sickle Cell Disease. Pediatr Blood Cancer 2016; 63:983.
2. BinAmir HA, AlAhmari A, AlQahtani A, Mohamed G, Alotaibi F, AlShamrani M, AlSaeed A, AlGhanmi S, Heji A, Alreshaid A, AlKawi A, AlHazzani A, AlZawahmah M, Shuaib A, Al-Ajlan F, AlMohareb F. Posterior reversible encephalopathy syndrome post stem cell transplantation in sickle cell disease: case series and literature review. Front Med (Lausanne). 2024 May 1;11:1330688. doi: 10.3389/fmed.2024.1330688. PMID: 38751974; PMCID: PMC11094324.
3. Abboud MR. Cerebral vasculopathy in patients with sickle cell disease and stroke: now you see it, now you don't. Haematologica. 2024 Oct 1;109(10):3108-3109. doi: 10.3324/haematol.2024.285383. PMID: 38752274; PMCID: PMC11443402.