Posterior reversible encephalopathy syndrome in childhood cancer

Background: Posterior reversible encephalopathy syndrome (PRES) is characterized by seizures, headaches, altered mental status, cortical blindness and typical transient lesions on magnetic resonance imaging. Patients and methods: We describe seven childhood cancer patients with clinical and radiological symptoms of PRES, and reviewed all well-documented PRES cases reported during childhood cancer treatment. Results: Fifty-six children with PRES, including our 7 cases, were identified in the literature. Mean age at onset was 9 (range: 2-17) years. Primary diagnoses were acute lymphoblastic leukemia (n = 31), acute myeloid leukemia (n = 5), non-Hodgkin lymphoma (n = 7) and solid tumors (n = 13). PRES patients presented with seizures (n = 50), altered mental status (n = 20), visual disturbances (n = 24) and/or headaches (n = 17). PRES was associated with hypertension in 49 patients. About 86% of the patients had both clinical and radiological reversible symptoms. Four patients developed epilepsy, in one patient ataxia remained and one patient had a persistent mydriasis. Conclusion: Although PRES has predominantly been described in leukemia patients, it occurs in children with solid tumors as well. Hypertension seems to be the most important trigger for the occurrence of PRES during childhood cancer treatment. Seizures are the most common accompanying sign. Symptoms and radiological findings normalize in ~90% of the cases, but in 10% neurological symptoms remain.