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Prognostic factors in children with PRES and hematologic diseases
N Tambasco1, E Mastrodicasa2, C Salvatori3
1Department of Neurology, Perugia General Hospital and University of Perugia, Perugia, Italy. n.tambasco@libero.it.
Insights
Posterior reversible encephalopathy syndrome (PRES) is a neurological condition often linked to cancer in children. Prompt recognition in transplant recipients is crucial to prevent severe complications or death.
Area of Science:
- Neurology
- Pediatrics
- Hematology
Background:
- Posterior reversible encephalopathy syndrome (PRES) is a neurological disorder presenting with seizures, headache, confusion, and focal neurological deficits.
- Cancer is a common predisposing factor for PRES in pediatric populations.
- Stem cell transplantation is a potential trigger for PRES in children with hematological diseases.
Objectives:
Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiological entity characterized by focal neurological signs, headache, confusion, and seizure, associated with transitory lesions in the posterior areas of the brain detectable with neuroimaging. Among children, one of the most common causes of PRES is cancer.
Materials And Methods:
In this review, we present the cases of 5 children developing PRES after stem cell transplantation for hematological disease and review all the cases reported in English literature to investigate outcomes and associated risk factors.
Results:
One hundred and eleven cases were reported. Hypertension was very frequent (80%). Clinical features included seizures (80.1%), headache (44.1%), visual disturbance (26.1%), and mental change (48.6%). EEG was abnormal in 27 of 32 patients. MRI revealed characteristic lesions in all patients even in early stages. Abnormal MRI findings in late stages were associated with neurological sequelae. Nineteen patients died (17.1%) of which 2 of PRES. Among alive patients, 17 had neurological sequelae. Four cases of PRES relapse were described.
Conclusions:
Thus, all transplant recipients with symptoms consistent with PRES should be promptly recognized to avoid long-term complications or even death.
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