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Posterior reversible encephalopathy syndrome in children with kidney diseases
Kenji Ishikura1, Yuko Hamasaki, Tomoyuki Sakai
1Department of Nephrology, Tokyo Metropolitan Children's Medical Center, 2-8-29 Musashidai, Fuchu-city, Tokyo, 183-8561, Japan. kenzo@ii.e-mansion.com
Insights
Posterior reversible encephalopathy syndrome (PRES) is a condition often seen in children with kidney disease. Early diagnosis and management of PRES symptoms like seizures and altered consciousness are crucial for recovery.
Area of Science:
- Neurology
- Nephrology
- Radiology
Background:
- Posterior reversible encephalopathy syndrome (PRES) is characterized by reversible leukoencephalopathy, often associated with renal insufficiency, hypertension, or immunosuppressive therapy.
- PRES is notably prevalent in pediatric patients with kidney diseases, highlighting the importance of awareness among pediatric nephrologists.
Purpose of the Study:
- To provide a comprehensive overview of Posterior Reversible Encephalopathy Syndrome (PRES) relevant to pediatric nephrology.
- To emphasize diagnostic approaches, predisposing factors, clinical manifestations, and imaging modalities for PRES.
Main Methods:
- Review of existing literature on PRES, focusing on its association with renal diseases and immunosuppressive therapies.
- Analysis of diagnostic criteria, including clinical symptoms and imaging findings, particularly cranial magnetic resonance (MR) imaging.
Main Results:
- Hypertension and calcineurin inhibitors are identified as major predisposing factors for PRES.
- Nephrotic syndrome is a common underlying condition; frequent symptoms include altered consciousness, seizures, headache, and visual disturbances.
- Cranial MR imaging is the primary diagnostic tool, with diffusion-weighted imaging and ADC mapping showing future potential for improved specificity.
Conclusions:
- PRES diagnosis requires a comprehensive evaluation of predisposing factors, clinical presentation, and imaging.
- Prompt management of PRES typically leads to symptom resolution within weeks.
- Further research into PRES pathophysiology and advanced imaging techniques is warranted for enhanced diagnostic accuracy.
Abstract:
Posterior reversible encephalopathy syndrome (PRES) was originally used to describe a reversible, predominantly posterior leukoencephalopathy in patients who had renal insufficiency, hypertension, or who received immunosuppressive therapy. Since PRES is prevalent in children with kidney diseases, awareness and understanding of it is important for practicing pediatric nephrologists. A comprehensive approach to the diagnosis of PRES includes thorough determination of predisposing factors, clinical symptoms, and mandatory appropriate imaging. Unfortunately, the pathophysiology of PRES is still obscure and specificity of radiological examination has not yet been established. Two major predisposing factors, namely hypertension and calcineurin inhibitors, are well recognized. In addition, nephrotic syndrome is a common underlying condition for development of PRES. Frequent symptoms include altered consciousness (coma, stupor, lethargy, confusion), seizure, headache, and visual disturbance. Most of these symptoms usually develop abruptly and resolve within a few weeks after proper management. Cranial magnetic resonance (MR) imaging is the first-line modality of imaging studies for detecting PRES. Diffusion-weighted imaging with quantification of apparent diffusion coefficient (ADC) values by ADC mapping may provide more accurate and specific images in the future.
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