Childhood Posterior Reversible Encephalopathy Syndrome: Clinicoradiological Characteristics, Managements, and Outcome

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a novel clinicoradiological syndrome characterized by convulsions, headache, altered mentality, and impaired vision, which are usually accompanied by hypertension. As its nomination, PRES is usually diagnosed according to the presence of typical neuroimage showing vasogenic edema predominately involving the posterior brain area. With the widespread utilization of magnetic resonance imaging (MRI), PRES is becoming more perceptible in different medical fields. Compared to adult cases, childhood PRES seems to have a broader clinical and neuroradiological spectrum. PRES can be associated with various underlying comorbidities, medication use, and therapeutic modalities in children with diverse neurological manifestations. Moreover, pediatric patients with PRES have a more significant propensity for atypical MRI findings beyond the typically posterior cerebral areas. The knowledge of typical and atypical presentations in children is essential to avoid misdiagnosing or missing PRES, which is a potentially treatable entity. Early supportive care is the mainstay of treatment, with particular attention to the treatment of hypertension with rigorous attention to all body systems. Prompt identification and symptom-directed management are imperative to achieve a reversible prognosis in childhood PRES. Future studies specially designed for the child population are required to determine potential outcome predictors, and further, to develop novel strategies of neuroprotection in childhood PRES.

Keywords: childhood; hypertension; neuroimgaging; posterior reversible encephalopathy syndrome; seizures.

Figure 1

Typical neuroimaging of PRES in a 9-year-old male with underlying nephritic syndrome. (A) Initially, unenhanced brain CT showed mildly cortico-subcortical hypodensities, particularly in bilateral parieto-occipital regions. (B) At the acute stage, these cerebral lesions showed hypointensities on T1-weighted MRI in bilateral parieto-occipital regions (C) T2-weighted MRI, and (D) T2-FLAIR MRI showed symmetrical hyperintensities in bilateral parieto-occipital regions. (E) ADC map shows a hyperintense signal in the aforementioned corresponding areas. (F) FLAIR images 2 months later, showing a complete resolution of the abnormal signal.

Figure 2

Atypical neuroimaging findings of PRES. (A,B) In a 17-year-old female with underlying systemic lupus erythematosus, lesions diffusely involving cerebral hemispheres, basal ganglia, and brain stem, with a prominent asymmetry on FLAIR images. (C) In a 12-year-old female with nephritic syndrome, MRI showed paradoxically hyperintense lesions on DWI (restricted diffusion) in bilateral occipital lobes. (D) In a 14-year-old male with membranoproliferative glomerulonephritis who showed prominent lesions of bilateral cerebellar hemispheres in FLAIR images (E,F) In an 8-year-old male with nephrotic syndrome, MRI showed occipital lesions hyperintensities on FLAIR, which also demonstrate post-contrast enhancement on T1-weighted image (white arrows).

Figure 3

Characteristic neuroimaging findings of a 15-year-old male with posterior reversible encephalopathy syndrome with spinal cord involvement (PRES-SCI). (A) Axial view of brain MRI showing T2-weighted hyperintensity in the bilateral parieto-occipital and frontal lobes. (B) Coronal view of MRI showing T2 hyperintensity in the brain stem, as well as in bilateral frontoparietal lobes (white arrows). (C) Sagittal cervicothoracic spine demonstrating longitudinally T2-weighted hyperintensity originating at cervicomedullary junction (white arrows).