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      Posterior reversible encephalopathy syndrome in pediatric acute leukemia: Case series and literature review

      case-report

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          Abstract

          Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic state coupled with a unique radio imaging appearance. We describe this rare, mostly reversible condition in five cases undergoing similar treatment under preset protocol (MCP-841) for acute lymphoblastic leukemia (ALL) at our centre. Hypertension is a well-known adverse effect of high-dose corticosteroid therapy primarily mediated by its effects on the mineralocorticoid receptor especially in pediatric population and we hypothesize that this may be the etiology of PRES in two of these patients.

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          Posterior reversible encephalopathy syndrome: incidence of atypical regions of involvement and imaging findings.

          Alexander McKinney, James Roswell Short, Charles L Truwit … (2007)
          Posterior reversible encephalopathy syndrome (PRES) is classically characterized as symmetric parietooccipital edema but may occur in other distributions with varying imaging appearances. This study determines the incidence of atypical and typical regions of involvement and unusual imaging manifestations. Seventy-six patients were eventually included as having confirmed PRES from 111 initially suspected cases, per imaging and clinical follow-up. Two neuroradiologists retrospectively reviewed each MR image. Standard sequences were unenhanced FLAIR and T1- and T2-weighted images in all patients, with diffusion-weighted imaging (n = 75) and contrast-enhanced T1-weighted imaging (n = 69) in most. The regions involved were recorded on the basis of FLAIR findings, and the presence of atypical imaging findings (contrast enhancement, restricted diffusion, hemorrhage) was correlated with the severity (extent) of hyperintensity or mass effect on FLAIR. The incidence of regions of involvement was parietooccipital, 98.7%; posterior frontal, 78.9%; temporal, 68.4%; thalamus, 30.3%; cerebellum, 34.2%; brainstem, 18.4%; and basal ganglia, 11.8%. The incidence of less common manifestations was enhancement, 37.7%; restricted diffusion, 17.3%; hemorrhage, 17.1%; and a newly described unilateral variant, 2.6%. Poor correlation was found between edema severity and enhancement (r = 0.072), restricted diffusion (r = 0.271), hemorrhage (r = 0.267), blood pressure (systolic, r = 0.13; diastolic, r = 0.02). Potentially new PRES causes included contrast-related anaphylaxis and alcohol withdrawal. This large series of PRES cases shows that atypical distributions and imaging manifestations of PRES have a higher incidence than commonly perceived, and atypical manifestations do not correlate well with the edema severity.
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            Posterior reversible encephalopathy syndrome in infection, sepsis, and shock.

            W Bartynski, J Boardman, Z Zeigler … (2015)
            The cause of "posterior reversible encephalopathy syndrome" (PRES) is not established. We recently encountered several patients who developed PRES in the setting of severe infection. In this study, we comprehensively reviewed the clinical and imaging features in a large cohort of patients who developed PRES, with particular attention to those with isolated infection, sepsis, or shock (I/S/S). The clinical/imaging features of 106 patients who developed PRES were comprehensively evaluated. In 25 of these patients, PRES occurred in association with severe I/S/S separate from transplantation. The clinical/imaging features (computer tomography, MR imaging, and MR angiography [MRA]) of the patients with I/S/S were further evaluated, including organ/tissue/blood culture results, mean arterial blood pressure (MAP) at toxicity, extent of cerebral edema, and presence of vasospasm. PRES occurred in association with I/S/S in 25 of 106 patients (23.6%), in addition to 4 other major clinical settings, including cyclosporine/FK-506 (post-transplant) neurotoxicity (46.2%), autoimmune disease (10.4%), postchemotherapy (3.7%), and eclampsia (10.4%). In the 25 patients with I/S/S, available cultures demonstrated a predominance of gram-positive organisms (84%). Blood pressure was "normal" at toxicity in 10 patients (MAP, 95 mm Hg); "severe" hypertension was present in 15 patients (MAP, 137 mm Hg). Extent of brain edema graded on imaging studies was greater in the normal MAP group compared with the severe hypertension group (P < .05). MRA demonstrated vasospasm in patients with severe hypertension and vessel "pruning" in the normal MAP group. Infection/sepsis/shock may be an important cause of PRES, particularly in relation to infection with gram-positive organisms.
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              Clinical spectrum of reversible posterior leukoencephalopathy syndrome.

              Soonhak Kwon, Jahoon Koo, Sangkwon Lee (2001)
              Reversible posterior leukoencephalopathy syndrome is a recently recognized disorder with characteristic radiologic findings that mainly involve the white/gray matter of the parieto-occipital lobes. This complex syndrome is associated with cyclosporine A therapy or a variety of other conditions in which blood pressure rises acutely. Twelve patients from a variety of conditions who met the diagnostic criteria for this syndrome were studied. Interestingly, three of these patients had intra-abdominal neurogenic tumors, which have rarely been reported. Initial cranial magnetic resonance imaging scans revealed fairly symmetric areas of increased T(2) signal involving both white and gray matter of parieto-occipital lobes in the majority of the patients. However, the lesions were often located outside the parieto-occipital regions. Four patients had occipital region magnetic resonance spectroscopy during the acute phase, which revealed high lactate peak and normal N-acetyl aspartate/creatine and choline peaks. With appropriate treatment, most patients recovered from this syndrome and experienced almost complete resolution of brain lesions on follow-up magnetic resonance imaging. One patient, however, continued to have small residual hemosiderin deposits on a follow-up magnetic resonance imaging with neurologic sequellae. In conclusion, a better understanding of this complex syndrome may obviate unnecessary investigations and allow management of the associated problems in prompt and appropriate ways.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Indian J Med Paediatr Oncol
                Journal ID (iso-abbrev): Indian J Med Paediatr Oncol
                Journal ID (publisher-id): IJMPO
                Title: Indian Journal of Medical and Paediatric Oncology : Official Journal of Indian Society of Medical & Paediatric Oncology
                Publisher: Medknow Publications & Media Pvt Ltd (India )
                ISSN (Print): 0971-5851
                ISSN (Electronic): 0975-2129
                Publication date (Print): Jan-Mar 2014
                Volume: 35
                Issue: 1
                Pages: 79-82
                Affiliations
                [1 ] Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bangalore, India
                [2 ] Department of Pediatric Oncology, Kidwai Memorial Institute of Oncology, Bangalore, India
                Author notes
                Address for correspondence: Dr. Sandhya Appachu M, C/O Department of Medical Oncology, Kidwai Institute of Medical Oncology, Bangalore, India. E-mail: sandhyaappachu@ 123456yahoo.co.in
                Article
                Publisher ID: IJMPO-35-79
                DOI: 10.4103/0971-5851.133727
                PMC ID: 4080669
                SO-VID: c73d4aa1-e252-40d7-a47d-bd4a3662c7b8
                Copyright © Copyright: © Indian Journal of Medical and Paediatric Oncology
                License:

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Oncology & Radiotherapy
                Keywords: encephalopathy syndrome,pres,steroid induced encephalopathy
                Data availability:
                ScienceOpen disciplines: Oncology & Radiotherapy
                Keywords: encephalopathy syndrome, pres, steroid induced encephalopathy

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