ALL-113: Pattern, Risk Factors, and Outcome Predictors of Posterior Reversible Encephalopathy Syndrome in Pediatric Cancer Patients

Abstract

Context: Although PRES is considered to have a benign clinical course, some studies have reported associated mortality of 5–19% and severe functional impairments in 44% of patients. Objective: To describe the risk factors and clinical and radiological patterns of PRES and to assess different predictors related to its outcome in pediatric cancer patients. Design/Setting: A retrospective study included pediatric cancer patients who developed PRES during their treatment at the Children’s Cancer Hospital Egypt (CCHE-57357) from January 2013 to June 2017. Patients or Other Participants: This study included 50 patients (32 hematologic malignancies, 13 solid tumors, and 5 post-BMT. ALL was the most common diagnosis encountered in 19 patients (38%), followed by lymphoma in 8 patients (16%) and AML in 5 (10%). Main Outcomes Measures: Clinical outcome of PRES was assessed using the Modified Rankin scale (mRS) after 2 weeks and 3 months from the episode. Patients were classified either good outcome; those who had complete recovery from all PRES symptoms without or with mild neurological deficit (mRS 0–2), or bad outcome for those who had significant neurological deficit (mRS 3–5), and those who died due to PRES (mRS 6). Results: Convulsions were the most common presenting clinical finding (found in 45 patients), and initial motor affection was encountered in 6 patients. Hypertension was reported in 86% at the time of onset. Regarding the MRI findings, occipital affection was the most common (92%), followed by frontal and temporal lobe involvement in 32% and 22%, respectively, and advanced PRES was described in 8 patients. Among all patients, 80% had complete clinical resolution, and 60% showed complete radiological resolution at 2 weeks’ evaluation, and 2 patients died out of PRES. An unfavorable outcome was associated with those who had motor dysfunction (p=0.0001) and status epilepticus (p=0.004) at presentation. Moreover, frontal lobe and thalamic affection and atypical PRES were also associated with poor outcome. Conclusions: Most children with cancer who develop PRES have favorable clinical and radiologic outcomes, but exceptionally persistent brain damage and even death may occur. Unfavorable outcome is associated with many factors, including motor dysfunction, status epilepticus, frontal lobe or thalamic affection, and atypical PRES. Although PRES is considered to have a benign clinical course, some studies have reported associated mortality of 5–19% and severe functional impairments in 44% of patients. To describe the risk factors and clinical and radiological patterns of PRES and to assess different predictors related to its outcome in pediatric cancer patients. A retrospective study included pediatric cancer patients who developed PRES during their treatment at the Children’s Cancer Hospital Egypt (CCHE-57357) from January 2013 to June 2017. This study included 50 patients (32 hematologic malignancies, 13 solid tumors, and 5 post-BMT. ALL was the most common diagnosis encountered in 19 patients (38%), followed by lymphoma in 8 patients (16%) and AML in 5 (10%). Clinical outcome of PRES was assessed using the Modified Rankin scale (mRS) after 2 weeks and 3 months from the episode. Patients were classified either good outcome; those who had complete recovery from all PRES symptoms without or with mild neurological deficit (mRS 0–2), or bad outcome for those who had significant neurological deficit (mRS 3–5), and those who died due to PRES (mRS 6). Convulsions were the most common presenting clinical finding (found in 45 patients), and initial motor affection was encountered in 6 patients. Hypertension was reported in 86% at the time of onset. Regarding the MRI findings, occipital affection was the most common (92%), followed by frontal and temporal lobe involvement in 32% and 22%, respectively, and advanced PRES was described in 8 patients. Among all patients, 80% had complete clinical resolution, and 60% showed complete radiological resolution at 2 weeks’ evaluation, and 2 patients died out of PRES. An unfavorable outcome was associated with those who had motor dysfunction (p=0.0001) and status epilepticus (p=0.004) at presentation. Moreover, frontal lobe and thalamic affection and atypical PRES were also associated with poor outcome. Most children with cancer who develop PRES have favorable clinical and radiologic outcomes, but exceptionally persistent brain damage and even death may occur. Unfavorable outcome is associated with many factors, including motor dysfunction, status epilepticus, frontal lobe or thalamic affection, and atypical PRES.