Clinical and Radiological Characteristics of Classical and Variant Type of Posterior Reversible Encephalopathy Syndrome on Prognosis Following Hematopoietic Stem Cell Transplantation in Pediatric Patients: A Single-Center Experience.

OBJECTIVES

Hematopoietic stem cell transplant is increasingly being used as a curative therapeutic option for patients with hematologic malignancies and nonmalignant diseases. Here, we aimed to determine the frequency and features of typical and atypical central variants of posterior reversible encephalopathy syndrome in children who had undergone an allogeneic hematopoietic stem cell transplant procedure for any indication at a single center.

MATERIALS AND METHODS

We retrospectively analyzed 101 pediatric patients between aged 6 months and 18 years who had undergone hematopoietic stem cell transplant for any indication between 2010 and 2023. We evaluated morbidity and mortality rates and several risk factors, therapies, and outcomes associated with posterior reversible encephalopathy syndrome. Medical data were obtained from patients' electronic health records.

RESULTS

In 101 patients who underwent hematopoietic stem cell transplant, the most common diagnosis was acute lymphoblastic leukemia (n = 46; 45.5%). Median time to onset of posterior reversible encephalopathy syndrome was 20 days (range, 10-108 days) posttransplant. Most cases of posterior reversible encephalopathy syndrome were typical, and 16 patients (15.8%) had the central variant. Headache was the most common clinical manifestation (79.2%). Rates of steroid use and complete remission in patients with the central variant were significantly (P < .05) lower than in the group with the typical variant. Mortality rates were significantly higher in patients with the central variant (n = 10, 62.5%) compared with those with the typical variant (n = 5, 5.9%) (P < .001).

CONCLUSIONS

Patients with the central variant of posterior reversible encephalopathy syndrome showed significantly increased risk of mortality. Because this clinical-radiological condition frequently manifests with atypical findings on magnetic resonance imaging, consideration of posterior rever-sible encephalopathy syndrome as a potential diagnosis is crucial when the clinical presentation is consistent but radiological findings are not typical to posterior reversible encephalopathy syndrome.