Imaging Patterns and Outcome of Posterior Reversible Encephalopathy Syndrome During Childhood Cancer Treatment

Abstract

Background Diagnosis of posterior reversible encephalopathy syndrome (PRES) requires presence of headache, seizures, impaired vision, or altered mentation accompanied by specific imaging findings. We aimed to study long-term clinical and radiologic outcome of PRES in children with cancer to augment limited available data. Procedure Retrospective review of children with cancer who were diagnosed with PRES. Results We identified PRES in 21 males and 16 females amongst the 5,217 children treated during the study period. Median time from cancer diagnosis to PRES was 6.6 months in 25 leukemia (1.6%), 5 brain tumor (0.3%), and 7 other solid tumor (0.4%) patients; p=<0.0001 for leukemia versus all other tumors. Symptoms included seizures (97%), headaches (40%), altered mentation (68%), and vision impairment (27%). Hypertension in 97% and steroids use was seen in 78%. Headaches, visual disturbance, and mental status resolved within a median of <3 days, while epilepsy developed in 19%. T2 hyperintense signal was present in 100% of occipital, 47% of temporal, 75% of parietal, and 55% of frontal lobes, as well as 22% of cerebellum and 5% of basal ganglia. Follow-up MRI in 34 patients showed partial or complete T2 resolution in 79%, development of laminar necrosis in 5, microhemorrhages in 6, and focal atrophy in 3. Conclusion PRES in children is more common in hematological malignancy compared to other tumors and is associated with hypertension and steroid use. Seizure is the most common acute manifestation. Most MRI changes resolve, but persistent imaging abnormality and epilepsy may develop in a significant minority.

Posterior reversible encephalopathy syndrome in children with acute lymphoblastic leukemia: Clinical characteristics, risk factors, course, and outcome of disease

“…The present study showed that PRES was a common neurotoxicity during early ALL treatment with the NOPHO ALL2008 protocol, in line with previously reported data . Older age, T‐cell leukemia, CNS involvement at diagnosis, and high‐risk block treatment were significant risk factors for PRES.…”

“…Posterior reversible encephalopathy syndrome (PRES), first described in 1996, has been reported in children with acute lymphoblastic leukemia (ALL), usually occurring during the three first months of therapy . The reported incidence of PRES in pediatric ALL varies from 1.6% to 4.5% .…”

“…Posterior reversible encephalopathy syndrome (PRES), first described in 1996, 1 has been reported in children with acute lymphoblastic leukemia (ALL), usually occurring during the three first months of therapy. [2][3][4][5][6][7] The reported incidence of PRES in pediatric ALL varies from 1.6% to 4.5%. [2][3][4][5][6][7] Endothelial and blood-brain barrier dysfunction are the main underlying pathophysiological mechanisms causing PRES, 8 and it has been speculated that hypertension and the cytotoxic effect of pharmacological treatments constitute triggering factors.…”

Neurotoxicity including posterior reversible encephalopathy syndrome after initiation of calcineurin inhibitors in transplanted methylmalonic acidemia patients: Two case reports and review of the literature

“…With early recognition and treatment of CNI‐induced neurotoxicity, including PRES, the majority of patients will have no remaining neurological symptoms . However, life‐threatening complications may occur and acquired neurological damage may persist . In the three MMA patients with PRES some remaining clinical abnormalities were found (case 1) …”

“…28 However, life-threatening complications may occur 32 and acquired neurological damage may persist. 33,34 In the three MMA patients with PRES some remaining clinical abnormalities were found (case 1). 11,24 4.2 | FGF-21 and CNI-induced neurotoxicity, including PRES, in transplanted MMA patients…”