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Posterior reversible encephalopathy syndrome in childhood cancer
P de Laat1, M L Te Winkel, A S Devos
1Department of Pediatric Oncology/Hematology, Erasmus MC-Sophia Children’s Hospital, University Medical Center, Rotterdam, The Netherlands.
Insights
Posterior reversible encephalopathy syndrome (PRES) in children with cancer is often triggered by hypertension and presents with seizures. While symptoms typically resolve, some patients may experience lasting neurological issues.
Area of Science:
- Neurology
- Pediatric Oncology
- Radiology
Background:
- Posterior reversible encephalopathy syndrome (PRES) is a neurological condition characterized by seizures, headaches, altered mental status, cortical blindness, and transient MRI lesions.
- PRES is increasingly recognized in pediatric cancer patients, often associated with specific treatments and underlying malignancies.
Purpose of the Study:
- To investigate the incidence, clinical presentation, and outcomes of PRES in children undergoing cancer treatment.
- To identify potential triggers and associated factors of PRES in this vulnerable population.
Main Methods:
- A retrospective review of seven childhood cancer patients with PRES was conducted.
- A comprehensive literature search identified and analyzed 49 additional well-documented cases of childhood cancer-associated PRES.
Main Results:
- Fifty-six cases of pediatric cancer-associated PRES were identified, with a mean age of onset at 9 years.
- Acute lymphoblastic leukemia was the most common primary diagnosis (31 cases), followed by solid tumors (13 cases).
- Hypertension (49 patients) was the most frequent trigger, and seizures (50 patients) were the most common symptom. Approximately 86% of patients showed reversible clinical and radiological symptoms.
Conclusions:
- PRES occurs in children with both leukemia and solid tumors, not exclusively in leukemia patients.
- Hypertension is a critical factor in PRES development during childhood cancer therapy.
- While most cases resolve, a small percentage (around 10%) may have persistent neurological deficits.
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.
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