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Introduction: Posterior reversible encephalopathy syndrome (PRES) is a serious neurological syndrome that may develop following immunosuppressive therapy for stem cell transplantation (SCT). We report 8 patients with sickle cell disease (SCD) who developed PRES, which is likely to be related to immunosuppression. Methods: This is retrospective cohort analysis of the SCD registry at the King Faisal Specialist Hospital and Research Center (KFSHRC) in Riyadh, Saudi Arabia. Inclusion criteria included all adults SCD patients who underwent SCT from 2011 until 2022. We explored all cases of PRES in patients with SCT. PRES was diagnosed with MRI imaging showing reversible vasogenic cerebral edema associated with neurological symptoms including severe headache, seizures, encephalopathy, delirium, and visual disturbances. Results: During ten years follow-up (2011-2022) we found 8 patients with PRES (age range between 14 to 37 years at diagnosis) PRES occurred 8 to 124 days following SCT in 7 cases and one patient developed PRES 8 months prior to SCT. All patients were on immunosuppressive medications, including tacrolimus, cyclosporine, sirolimus and or mycophenolate mofetil. Headache, seizures, visual hallucinations, confusion, and drowsiness were the most common presenting symptoms. MRI showed abnormalities in the occipital, parietal and frontal lobes in most cases. Recovery was complete in all patients and no recurrences were noted. Two patients had graft versus host disease (GVHD). We compared risk factors for PRES among the 8 cases and 136 SCT in SCD patients who did not develop PRES. There was a significant association between PRES and imaging abnormalities, including previous bi-hemispheric infarctions (p = 0.001), and cerebral microbleeds (CBMs). PRES was strongly associated with presence (p = 0.006), size (p = 0.016) and number (p = 0.005) of CMBs. Conclusion: PRES can develop days to weeks following SCT in patients with SCD, and is associated with immunosuppressive therapy, previous bi-hemispheric infarctions and CMB. Prompt recognition and intervention leads to good recovery.
RESUMO
To prevent radiologists from overlooking lesions, radiology textbooks recommend "systematic viewing," a technique whereby anatomical areas are inspected in a fixed order. This would ensure complete inspection (full coverage) of the image and, in turn, improve diagnostic performance. To test this assumption, two experiments were performed. Both experiments investigated the relationship between systematic viewing, coverage, and diagnostic performance. Additionally, the first investigated whether systematic viewing increases with expertise; the second investigated whether novices benefit from full-coverage or systematic viewing training. In Experiment 1, 11 students, ten residents, and nine radiologists inspected five chest radiographs. Experiment 2 had 75 students undergo a training in either systematic, full-coverage (without being systematic) or non-systematic viewing. Eye movements and diagnostic performance were measured throughout both experiments. In Experiment 1, no significant correlations were found between systematic viewing and coverage, r = -.10, p = .62, and coverage and performance, r = -.06, p = .74. Experts were significantly more systematic than students F2,25 = 4.35, p = .02. In Experiment 2, significant correlations were found between systematic viewing and coverage, r = -.35, p < .01, but not between coverage and performance, r = .13, p = .31. Participants in the full-coverage training performed worse compared with both other groups, which did not differ between them, F2,71 = 3.95, p = .02. In conclusion, the data question the assumption that systematic viewing leads to increased coverage, and, consequently, to improved performance. Experts inspected cases more systematically, but students did not benefit from systematic viewing training.
