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Published on: September 20, 2024
Children with posterior reversible encephalopathy syndrome associated with atypical diffusion-weighted imaging and
Kenji Ishikura1, Yuko Hamasaki, Tomoyuki Sakai
1Department of Pediatric Nephrology, Tokyo Metropolitan Children's Medical Center, 2-8-29 Musashidai, Fuchu, Tokyo 183-8561, Japan. kenzo@ii.e-mansion.com
Insights
Posterior reversible encephalopathy syndrome (PRES) can mimic irreversible brain damage on MRI in children. However, these PRES cases fully recovered, highlighting imaging limitations in early diagnosis.
Area of Science:
- Neurology
- Pediatric Nephrology
- Radiology
Background:
- Posterior reversible encephalopathy syndrome (PRES) is a neurological condition characterized by reversible leukoencephalopathy, often associated with hypertension, renal insufficiency, or immunosuppressants.
- PRES typically affects the posterior brain regions and can present with diverse clinical and radiological findings.
Observation:
- Two pediatric cases of PRES secondary to idiopathic nephrotic syndrome and lupus nephritis were analyzed.
- Cranial magnetic resonance (MR) imaging revealed strong hyperintense signals on diffusion-weighted imaging (DWI) with restricted apparent diffusion coefficient (ADC) values, predominantly in posterior brain regions.
Findings:
- The observed MR imaging findings, particularly restricted diffusion, are rarely reported in pediatric PRES and initially suggested irreversible brain damage.
- Despite initial imaging concerns, both children achieved complete clinical and radiological recovery.
- This suggests that DWI/ADC findings in PRES may not always correlate with irreversible injury.
Implications:
- Cranial MR imaging, including DWI with ADC mapping, has limitations in accurately diagnosing early-stage PRES and predicting patient outcomes.
- Further research and experience are needed to refine the diagnostic accuracy and prognostic value of advanced MR techniques in pediatric PRES.
- Clinicians should consider the potential for reversible changes in PRES, even with concerning initial imaging findings.
Abstract:
Posterior reversible encephalopathy syndrome (PRES) is a reversible, predominantly posterior, leukoencephalopathy associated with renal insufficiency, hypertension, or immunosuppressant drugs. We describe two children with PRES whose primary diagnoses were idiopathic nephrotic syndrome and lupus nephritis. Cranial magnetic resonance (MR) imaging at the onset of PRES showed strong hyperintense signals on diffusion-weighted imaging with restricted apparent diffusion coefficient values predominantly in the posterior region. Such findings have been rarely reported in children with PRES and initially suggested irreversible brain damage; however, both children fully recovered clinically as well as radiologically. Our findings suggest the limitations of cranial MR imaging for diagnosing PRES. Further experience with cranial MR imaging, including diffusion-weighted imaging with apparent diffusion coefficient mapping, is required to improve diagnostic accuracy and the ability to predict outcomes in patients with early-stage PRES. At present, initial imaging studies do not necessarily provide sufficient evidence for a firm diagnosis of PRES or the prediction of outcomes.
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