Posterior Reversible Encephalopathy Syndrome in Children:: A Prospective Follow-up Study

2019 ◽  
Vol 35 (1) ◽  
pp. 55-62 ◽  
Author(s):  
Amira Hamed Darwish

Aim: To evaluate clinical and radiologic presentation, and neurologic outcome of pediatric posterior reversible encephalopathy syndrome (PRES). Patients and Methods: The study included 24 children (14 males and 10 females) diagnosed with PRES who were prospectively followed for 2 years. They were evaluated using Wechsler Intelligence Scale, electroencephalograph (EEG), and brain magnetic resonance imaging (MRI). Results: The mean age of the studied patients at the time of diagnosis of PRES was 6 years (±2.2). Chemotherapy for cancer represented 66.7% of the causes of PRES in the studied children, followed by renal disorders and immunosuppressive agents for hematopoietic stem cell transplantation. Twenty-seven attacks of PRES were reported as 3 children developed a second attack of PRES. Normal intelligence quotient was found in 95.8% of studied children after PRES. Residual abnormalities in follow-up MRI were demonstrated in 3 children. Epilepsy and residual MRI lesions were reported in 2 of the 3 children with recurrent PRES. Residual lesions in follow-up MRI and epilepsy were more significantly reported after recurrent PRES ( P < .05). Conclusions: Neoplastic, renal disorders and hematopoietic stem cell transplantation represent the main disorders associated with PRES in children. Chemotherapeutic drugs, immunosuppressants, and hypertension are the main risk factors for pediatric PRES. The outcome of pediatric PRES is good, but long-term neurologic sequelae can occur, mainly epilepsy and residual MRI abnormalities. Recurrence of PRES is infrequently reported in children receiving chemotherapeutic or immunosuppressive drugs. Recurrent PRES is a risk factor for long-term neurologic sequelae.

2021 ◽  
Author(s):  
Ali Onder Atca ◽  
Berrin Erok ◽  
Selime Aydogdu

Abstract Background: Hematopoietic stem cell transplantation (HSCT) is the intravenous infusion of hematopoietic progenitor cells derived from bone marrow, umblical cord blood or peripheral blood to restore hematologic and immunologic functions of bone marrow. HSCT is the only curative treatment in many childhood disorders and is used worldwide. However, serious complications are still an important concern of management. Central nervous system (CNS) complications in pediatric HSCT recipients can be associated with high morbidity and mortality. Posterior reversible encephalopathy syndrome (PRES) is the most common of these neuroimaging abnormalities in patients having neurological symptoms following HSCT. The most common presentation is posterior encephalopathy in which the lesions are located at the posterior parts of cerebral hemispheres, namely parietooccipital lobes. However, it can also be found in non-posterior distribution and can be accompanied by various atypical imaging manifestattions like restricted diffusion. Methods: In this study, we aimed to describe the typical and also uncommon neuroimaging manifestations of PRES following HSCT in our pediatric patients.We evaluated the medical records of 300 pediatric HSCT recipients.Results: We detected 14 cases of PRES, of which there were 3 cases of atypical neuroimaging manifestations, regarding both the sites of involvement and also the magnetic resonance imaging (MRI) manifestations. Conclusion: Such different presentations should not dissuade the diagnosis of PRES in the appropriate clinical settings in order to manage these patients timely and accurately.


2015 ◽  
Vol 135 (1) ◽  
pp. 1-10 ◽  
Author(s):  
Volker Schmidt ◽  
Tino Prell ◽  
Anne Treschl ◽  
Anne Klink ◽  
Andreas Hochhaus ◽  
...  

Background: Posterior reversible encephalopathy syndrome (PRES) is a rare but serious complication after allogeneic hematopoietic stem cell transplantation (alloHSCT). Among others, calcineurin inhibitors (CNI) for prophylaxis of graft-versus-host disease (GvHD) may promote the development of PRES, but the pathomechanism is still controversial. Discontinuation of CNI facilitates remission of symptoms but might contribute to the unfavorable prognosis of PRES due to an elevated incidence of GvHD. Methods: This is a case series of 7 patients with PRES from a retrospective analysis of 146 consecutive patients who received alloHSCT for hematologic malignancies. Results: At the onset of PRES, all patients presented a systemic infection, while no influence was seen for underlying disease, conditioning regimen, donor type, or GvHD. Discontinuation of CNI and control of the blood pressure reversed neurological symptoms in 6 patients, while 1 patient died from septic multiorgan failure. After bridging with prednisolone and/or mycophenolic acid, replacement of CNI by the mammalian target of rapamycin (mTOR) inhibitor everolimus effectively prevented severe GvHD without recurrence of PRES. Conclusions: A systemic infection/inflammation may be an important cause of PRES. Prophylaxis of GvHD by the mTOR inhibitor everolimus in case of PRES after alloHSCT demonstrated promising results but needs to be validated in larger cohorts.


2011 ◽  
Vol 5 (6) ◽  
pp. 543-549 ◽  
Author(s):  
Daniel W. Hommes ◽  
Marjolijn Duijvestein ◽  
Zuzana Zelinkova ◽  
Pieter C.F. Stokkers ◽  
Maartje Holsbergen-de Ley ◽  
...  

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