Radiological findings of Posterior Reversible Encephalopathy Syndrome in transplanted children previous affected by hemoglobinopathy: A neuroimaging retrospective analysis

Abstract

To evaluate, by Magnetic Resonance Imaging, if there is a typical pattern or severity of PRES in transplanted children for hemoglobinopathy. Secondary point was to investigate the pattern and severity of PRES in children with thalassemia-THAL and sickle-cell disease-SCD after autologous hematopoietic stem cell transplantation (aHSCT). Finally, we evaluate the presence of atypical PRES presentation and the involved area of central nervous system. Two neuroradiologists analyzed retrospectively MRI of 21 transplanted children for THAL or SCD treated with CI, with neurological symptoms and signs of PRES. The Bartynski and Boardman classification has been used for PRES pattern while McKinney scale for PRES severity. Fisher Exact Probability test or Chi-square test were used to compare the categorical data. In the 21 transplanted children the PRES severity was typically mild (85.7%) without preferring radiological pattern at MRI. The analysis didn't show significant association between PRES pattern or PRES severity and previous hemoglobinopathy (THAL or SCD). No atypical PRES presentation has been found. PRES severity in transplanted children for hemoglobinopathy is typically mild. Notwithstanding children affected by SCD have a damage on the capillary endothelium, after aHSCT our data didn't show a different PRES severity and pattern than THAL children.

Keywords: Hemoglobinopathy; MRI; PRES; Sickle-cell disease; Thalassemia; aHSCT.

Fig. 1

Mild PRES with Superior Frontal Sulcus pattern in 15 years-old male child previous affected by Sickle Cells Disease. T2-Fluid Attenuated Inversion Recovery (FLAIR) at basal ganglia level (a and c) and over the semioval centers (b and d). The MRI performed at day 0 (a and b) showed T2-FLAIR hyperintensity “U-fibers” of right occipital lobe and into iuxta-cortical white matter of left frontal lobe. At day 30 (c and d) MRI showed no signal abnormalities.

Fig. 2

Moderate PRES with Holohemispheric Watershed pattern in 12 years-old male child previous affected by Thalassemia. T2-Fluid Attenuated Inversion Recovery (FLAIR) at cerebellum (a and d), basal ganglia (b and e) and over the semioval centers (c and f). The MRI performed at day 0 (a, b and c) showed T2-FLAIR hyperintensity in cerebellum, slightly at pons, into left thalamus and at “U-fibers” in the frontal and parietal lobes. At day 28 (d, e and f) MRI showed no signal abnormalities.

Fig. 3

Mild PRES with Holohemispheric Watershed pattern in 7 years-old male child previous affected by Thalassemia. MRI at day 0. Diffusion Weighted Imaging (DWI), T2-FLAIR and T1 after administration of contrast media at mid-brain (a, e and i), lateral ventricles (b, f and j), into (c, g and k) and over (d, h and l) semioval centers. The exam showed T2-FLAIR hyperintensity in cortical-subcortical white matter of frontal, parietal and occipital lobes with alteration on Diffusion Weighted Imaging (pseudorestriction). Focal After administration of Gd-contrast agent diffuse subcortical and patchy enhancement has been detected.

Fig. 4

MRI at day 32 of the same patients showed in Fig. 3. The MRI showed reduction of signal abnormalities on Diffusion Weighted Imaging (a, b, c, d) and T2-FLAIR (e, f, g, h) imaging. After administration of Gd-contrast agent no enhancement has been detected (i, j, k, l). T2-Fast Field Echo imaging (m, n, o, p) showed focal hypointensity at cortical-subcortical white matter of left parietal lobe (o) with moderate atrophy of left superior parietal lobule gyrus.