Can clinical and MRI findings predict the prognosis of variant and classical type of posterior reversible encephalopathy syndrome (PRES)?

A 67‐Year‐Old Woman Receiving Tumor Necrosis Factor α Inhibitor Therapy Presenting With Neurologic Dysfunction

BACKGROUND: This study was planned with an aim to identify the nature and distribution of cranial lesions on magnetic resonance imaging (MRI) and its correlation with clinical and laboratory data in eclampsia and severe preeclampsia. MATERIAL AND METHODS: 40 patients admitted for indication of severe preeclampsia or eclampsia with or without neurological signs were first stabilized and then underwent cranial MRI. Following MRI they were divided into two groups; Group MP (n=24) including patients with positive finding on the cranial MRI and Group MN (n=16) which included patients with normal Cranial MR imaging. Nature and distribution of the lesions were documented and statistical comparison was made on the basis of clinical findings, arterial blood pressure and laboratory data in both the groups. Patient with cerebral changes in the MRI were also called back for repeat MRI postnatal after two 2 months. RESULTS: Out of the 40 patients who underwent cranial MRI 24 patients had cerebral changes (Group MP) whereas 16 patients had normal scan (Group MN). In 21 out of 24 (87.5%) MRI finding positive patients the finding was consistent with diagnosis of posterior reversible encephalopathy syndrome (PRES). The most commonly involved areas in patients with PRES were parietal (85.7%,), frontal (71.42%) and occipital lobe (71.42), followed by temporal lobe (38.09) and basal ganglia (33.33) and cerebellum. All the patients who were diagnosed with PRES had a normal MR scan on the follow up at two months after the initial presentation. There was a significantly greater incidence of seizures and neurological disturbances in patients with positive MRI findings as compared to patients with no MRI findings (p<0.001). There was no statistical difference between the blood pressure measurements of the two groups. Markers of endothelial dysfunction like Serum LDH (p=0.002) Serum creatinine (p=0.006) and abnormal red blood cell morphology (0.002) was significantly higher in patients with positive MRI findings compared to MRI Finding Negative group. CONCLUSION: Our study suggests that PRES is the core component of the pathogenesis of cerebral findings of eclampsia and development of PRES is associated with endothelial dysfunction and not elevated blood pressure alone.

This study investigated the clinical characteristics, cranial magnetic resonance imaging (MRI) features, MRI follow-up, and prognosis of children with acute lymphoblastic leukemia (ALL) who developed posterior reversible encephalopathy syndrome (PRES) during remission induction chemotherapy. We analyzed the age, gender, PRES symptoms and signs, cranial MRI findings, therapeutic effect, and prognosis of children with ALL who developed PRES during chemotherapy from January 2010 to December 2013 at the Hematology Oncology Center of Beijing Children's Hospital. Changes in cranial MRI findings were analyzed, and intelligence (IQ) and cognitive function were evaluated using the Wechsler Scale and the Wisconsin Card Score Test after the children completed chemotherapy. There were 850 children with newly diagnosed ALL in this period; 13 (1.5%), 6 boys and 7 girls, developed PRES. All were diagnosed as B-cell ALL. The median age at PRES onset was 7 years (2-11 years). The median day of PRES onset was day 28 (day 17-34) of remission induction chemotherapy. Of the 13 children with PRES onset and seizures, 4 had visual disturbances and 2 had consciousness disturbances. Cranial MRI showed hyperintensity in the subcortical white matter on T2-weighted axial and fluid-attenuated inversion recovery (FLAIR) images. The lesion locations were as follows: occipital lobe, 12 (92.3%) patients; frontal lobe, 7 (53.8%) patients; temporal lobe, 5 (38.4%) patients; parietal lobe, 3 (23.1%) patients; and cerebellum, 1 (7.7%) patient. There were 8 (61.5%) patients with vasogenic edema and 5 (38.5%) with cytotoxic edema. After treatment, all children recovered within one month, when their PRES symptoms were relieved, they continued to receive chemotherapy. However, 1 child (1.07%) died of severe central nervous system infection one year after PRES treatment, and 3 (25%) had recurrent seizures and were diagnosed with epilepsy after three months of PRES treatment. Their cranial MRIs showed cytotoxic edema, which was acute stage on day 15, with aggravated lesions on cranial MRI. The cranial MRI lesions returned to normal at one month in 3 (23.1%) patients, at three months in 6 (46.1%) patients, at one year in 8 (61.5%) patients, and at two years in 12 (92.3%) patients. The 12 surviving children all returned to school, and their full-scale, verbal, and performance IQs were normal, with no significant differences in intelligence or cognitive function compared with children with ALL without PRES during the same period. PRES can occur during remission induction chemotherapy treatment of children with ALL, but the incidence is low. Cranial MRI can be used for diagnosis and to characterize lesions. The children recover about a month after treatment, and cranial MRI lesions return to normal within two years. The time for complete resolution of MRI lesions differs, and children with cytotoxic edema have worse prognosis with sequelae, such as epilepsy, which requires close monitoring. PRES does not affect intelligence or cognitive development.

This study investigated the clinical characteristics, cranial magnetic resonance imaging (MRI) features, MRI follow-up, and prognosis of children with acute lymphoblastic leukemia (ALL) who developed posterior reversible encephalopathy syndrome (PRES) during remission induction chemotherapy. We analyzed the age, gender, PRES symptoms and signs, cranial MRI findings, therapeutic effect, and prognosis of children with ALL who developed PRES during chemotherapy from January 2010 to December 2013 at the Hematology Oncology Center of Beijing Children's Hospital. Changes in cranial MRI findings were analyzed, and intelligence (IQ) and cognitive function were evaluated using the Wechsler Scale and the Wisconsin Card Score Test after the children completed chemotherapy. There were 850 children with newly diagnosed ALL in this period; 13 (1.5%), 6 boys and 7 girls, developed PRES. All were diagnosed as B-cell ALL. The median age at PRES onset was 7 years (2-11 years). The median day of PRES onset was day 28 (day 17-34) of remission induction chemotherapy. Of the 13 children with PRES onset and seizures, 4 had visual disturbances and 2 had consciousness disturbances. Cranial MRI showed hyperintensity in the subcortical white matter on T2-weighted axial and fluid-attenuated inversion recovery (FLAIR) images. The lesion locations were as follows: occipital lobe, 12 (92.3%) patients; frontal lobe, 7 (53.8%) patients; temporal lobe, 5 (38.4%) patients; parietal lobe, 3 (23.1%) patients; and cerebellum, 1 (7.7%) patient. There were 8 (61.5%) patients with vasogenic edema and 5 (38.5%) with cytotoxic edema. After treatment, all children recovered within one month,when their PRES symtoms were relieved, they continued to receive chemotherapy. However, 1 child (1.07%) died of severecentral nervous system infection one year after PRES treatment, and 3 (25%) had recurrent seizures and were diagnosed with epilepsy after three months of PRES treatment. Their cranial MRIs showed cytotoxic edema, which was acute stage on day 15, with aggravated lesions on cranial MRI. The cranial MRI lesions returned to normal at one month in 3 (23.1%) patients, at three months in 6 (46.1%) patients, at one year in 8 (61.5%) patients, and at two years in 12 (92.3%) patients. The 12 surviving children all returned to school, and their full-scale, verbal, and performance IQs were normal, with no significant differences in intelligence or cognitive function compared with children with ALL without PRES during the same period. PRES can occur during remission induction chemotherapy treatment of children with ALL, but the incidence is low. Cranial MRI can be used for diagnosis and to characterize lesions. The children recover about a month after treatment, and cranial MRI lesions return to normal within two years. The time for complete resolution of MRI lesions differs, and children with cytotoxic edema have worse prognosis with sequelae, such as epilepsy, which requires close monitoring. PRES does not affect intelligence or cognitive development.

Posterior reversible encephalopathy syndrome (PRES) is a clinico-neuroradiological entity, characterized by typical neurological deficits, distinctive magnetic resonance imaging (MRI) features, and a usually benign clinical course. Although frequently seen in association with hypertensive conditions, many other predisposing factors, notably cytotoxic and immunosuppressant drugs have been associated with PRES. The aim of this study was to determine differences in the MR appearance of PRES according to various risk factors. Thirty consecutive patients with clinical and MRI findings consistent with PRES were included. We identified 24 patients with hypertension-related conditions, including 14 patients with preeclampsia-eclampsia, and six patients without hypertension, in whom PRES was associated with exposition to neurotoxic substances. Lesion distribution, extent of disease, and number of affected brain regions were compared between patients with PRES with and without hypertension, and patients with PRES with and without preeclampsia-eclampsia, respectively. No statistically significant differences in distribution of lesions and extent of disease were observed between patients with PRES with or without hypertension, and patients with or without preeclampsia-eclampsia, respectively. The number of affected brain regions was significantly higher in patients with preeclampsia-eclampsia (p = 0.046), and the basal ganglia region was more frequently involved in these patients (p = 0.066). Apart from a significant higher number of involved brain regions and a tendency for basal ganglia involvement in patients with PRES associated with preeclampsia-eclampsia, the MRI appearance of patients with PRES does not seem to be influenced by predisposing risk factors.

Posterior reversible encephalopathy syndrome from induced hypertension during endovascular thoracoabdominal aortic aneurysm repair

Posterior reversible encephalopathy syndrome (PRES) is characterized by typical neurological deficits and magnetic resonance imaging (MRI) findings. A 17-year-old nulliparous woman with 34-week-old pregnancy was diagnosed with preeclampsia and magnesium sulphate infusion was initiated. Despite this medication and an emergency cesarean section, the patient had repeating seizures. Cranial MRI showed hyperintense signal alterations in the cortical and subcortical regions of the right angular gyrus and, thus, a diagnosis of PRES was made. Presenting with headache, altered mental status and severe systolic hypertension in young women, preeclampsia associated PRES can be easily diagnosed by MRI findings. Conservative management with close monitorization of vital and clinical findings and administration of anti-hypertensive and anti-convulsant drugs would provide the reversibility of PRES related radiological changes in most cases. The clinicians dealing with preeclampsia should be aware of this clinical entity and they should appeal to MRI whenever there is a high index of suspicion.

Longitudinal multivoxel MR spectroscopy study of pediatric diffuse pontine gliomas treated by radiotherapy

Posterior reversible encephalopathy syndrome (PRES) and acute toxic leukoencephalopathy (ATL) are both potentially reversible clinicoradiologic entities. Although their magnetic resonance imaging (MRI) findings differ, rarely both may occur simultaneously in acutely encephalopathic patients. Our aim was to determine the incidence and causes of concomitant "ATL-PRES." Retrospective search of suspected acutely encephalopathic adults since 1998 throughout our picture archiving and communication system revealed 167 patients with PRES and 106 patients with ATL. Images of these patients were retrospectively evaluated by two neuroradiologists and a fellow to identify the cases which carry both features of PRES and ATL. Imaging findings were scored based on previously reported scoring system as mild, moderate, and severe. The clinical outcome of the patients was determined according to the modified Rankin scale. Our search revealed a series of 6 patients (%2.2) in 273 patients who presented acutely with either encephalopathy or seizures, caused by various etiologies, including immunosuppression following transplantation (n = 2), hypertensive crisis (n = 2), chemotherapy (n = 1), and sepsis (n = 1). MRI demonstrated findings consistent with both PRES and ATL simultaneously on FLAIR and diffusion weighted imaging. Severity of imaging findings of concomitant "ATL-PRES" was concordant with each other (rho ≈ 1.0, P < .00001), and each patient eventually returned to clinical baseline. This finding, along with their similar etiologies, raises the possibility of an underlying common pathophysiologic thread, perhaps being endothelial toxicity. Concomitant "ATL-PRES" was found in 2.2% of the patients in a large cohort of ATL and PRES. Etiologies varied. Clinical symptoms and MRI findings were potentially reversible.

To predict tumor recurrence in patients who underwent surgical resection of ampullary adenocarcinoma using preoperative magnetic resonance (MR) imaging findings combined with clinical findings. In this multicenter study, a total of 113 patients (mean age, 62.9 ± 9.8 years; 58 men and 55 women) with ampullary adenocarcinoma who underwent preoperative MR imaging and surgery with margin-negative resection between 2006 and 2017 were retrospectively included. The MR imaging findings were evaluated by two radiologists. Preoperative clinical findings were obtained. Cox proportional regression analyses were used to identify the independent prognostic factors for recurrence-free survival (RFS). A nomogram was created based on the multivariable analysis and was internally validated. Multivariable analysis revealed that presence of infiltrative tumor margin (hazard ratio [HR]: 2.18, p = 0.019), adjacent organ invasion (HR: 3.31, p = 0.006), adjacent vessel invasion (HR: 5.42, p = 0.041), peripancreatic lymph node enlargement (HR: 2.1, p = 0.019), and jaundice (HR: 1.93, p = 0.043) were significantly associated with worse RFS of ampullary adenocarcinoma after surgical resection. These MR imaging and clinical findings were used to construct a nomogram. On internal validation, the calibration plots showed excellent agreement between the predicted probabilities and the actual rates of tumor recurrence, with Harrell's c-index of 0.746. Combination of preoperative MR imaging and clinical findings can be useful for predicting tumor recurrence after surgical resection of ampullary adenocarcinoma. Identifying these features before surgery may aid in better treatment planning and management of these patients. A predictive nomogram using preoperative MR imaging and clinical findings can be useful in estimating the recurrence-free survival after surgical resection of ampullary adenocarcinoma. • Presently, tumor size on imaging is the only non-invasive factor that correlates with recurrence-free survival from ampullary adenocarcinoma; other factors are obtained postoperatively. • Infiltrative tumor margin, adjacent organ invasion, adjacent vessel invasion, peripancreatic lymph node enlargement on MRI, and jaundice are significant predictors for recurrence. • A nomogram incorporating significant MR imaging and clinical findings showed good performance in predicting recurrence-free survival, which can help in treatment planning.

Since solid organ and hematopoietic stem cell transplantations have been performed increasingly recently, use of immunosuppressive agents such as cyclosporine and tacrolimus has also risen significantly. Posterior reversible encephalopathy syndrome (PRES) is known to be a serious complication of immunosuppressive therapy use following solid organ or stem cell transplants. Clinical findings of the syndrome involve headache, mental status changes, focal neurological deficits, as well as visual disturbances, which are also associated with characteristic imaging features of subcortical white matter lesions on computed tomography (CT) or magnetic resonance imaging (MRI). Despite the fact that the alterations in subcortical white matter might be secondary to potentially reversible vasogenic edema, conversion to irreversible cytotoxic edema has also been described. These imaging findings appear to be prevalent in the territory of the posterior cerebral artery. In most earlier studies, it has been reported that the neurotoxicity associated with tacrolimus may occur at therapeutic levels. The sypmtom complex in most cases in PRES can be reversed through decreasing the dosage or withholding the drug for a few days. PRES is an uncommon complication; however, it may lead to significant morbity and mortality if ir is not diagnosed instantly. The study sheds light on the importance of MRI in prompt recognition of this syndrome, which provides us with the best chance to avoid long-run sequelae. This report highlights the value of MRI in prompt recognition of this entity, which offers the best chance of avoiding long-term sequelae. We aimed to present our 9 year-old renal transplant patient in whom we observed PRES following tacrolimus treatment in the light of clinical and MRI findings. Similar findings did not recur after tacrolimus use. Drug was not changed.

Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome with numerous etiologies, mostly characterized by magnetic resonance imaging (MRI) abnormalities in the posterior cerebral white and gray matter and acute neurological symptoms. To examine the predisposing factors, clinical results, and radiological features of PRES in children diagnosed with malignancy. The study included 20 patients (7 F/13 M) aged 4-16 years at the time of diagnosis who were diagnosed with malignancy and developed PRES during chemotherapy. All the patients were diagnosed as having PRES both clinically and radiographically during chemotherapy. The time from the initiation of the chemotherapy to the onset of PRES ranged from 7-675 days. Hypertension was detected in nine patients, seizure was the most common presenting symptom - had involvement in the occipital and parietal lobes on MRI (n=14)/followed by headache (n=8)/altered consciousness (n=5)/visual impairment (n=4). Hydrocephalus and tentorial herniation were observed in one patient. Most of the lesions on MRI resolved within 10-33 days and the EEG findings within 9 months. Clinical symptoms of PRES also disappeared completely the 5-year Press frequency was found to be 2.48%. PRES may complicate the oncological treatment in children. Hypertension is a leading risk factor for PRES, while it should be kept in mind that the blood pressure may be normal in chemotherapy-induced PRES cases. PRES should be included in the differential diagnosis of all patients receiving chemotherapy and presenting with acute neurological symptoms.

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiologic syndrome characterized clinically by headache, seizures, and altered sensorium and radiological changes which are usually reversible. The purpose of this study was to describe the spectrum of magnetic resonance imaging (MRI) findings in childhood PRES, to determine the common etiologies for childhood PRES, and to have an insight into the pathophysiology of PRES. The MRI results of 20 clinically diagnosed cases of PRES between July 2011 and June 2013 were reviewed. The final diagnosis of PRES was based on the clinical presentation and the MRI features at the time of presentation, which resolved on the follow-up imaging. The medical records of the patients were reviewed to determine the underlying medical disease. Eight out of the 20 patients included in the study were on cyclosporine or tacrolimus based immunosuppressant therapy for kidney transplant. Four patients had severe hypertension at presentation. The most common MRI finding was high T2-fluid-attenuated inversion recovery (FLAIR) signal in the cortex and subcortical white matter of both cerebral hemispheres, particularly in the parietal and occipital lobes (n=16). The second most common MRI finding was increased leptomeningeal FLAIR signal (n=7). Out of seven patients with leptomeningeal signal, five demonstrated leptomeningeal enhancement as well. Four out of these seven patients had no other parenchymal findings. Childhood PRES is commonly seen in the setting of immunosuppressant therapy for kidney transplant, severe hypertension and cancer treatment. There was high incidence of increased leptomeningeal FLAIR signal and leptomeningeal enhancement in our study. It supports the current theory of endothelial injury with increased microvascular permeability as the potential pathophysiology of PRES. Also, absence of elevated blood pressure in majority of the patients in our study supports the theory of direct endothelial injury by some agents leading to vasogenic edema.

Analysis of Revision Anterior Cruciate Ligament Reconstruction according to the combined injury, degenerative change, and MRI findings

Purpose: To find out the characteristic MR findings of reversible posterior leukoen-cephalopathy syndrome (RPLS) due to various causes in children. Materials and Methods: Eight children with RPLS underwent MR imaging, and the findings were retrospectively analyzed. All eight were acutely hypertensive at the time of a neurotoxic episode. Three had intra-abdominal tumors (one adrenal pheochromo-cytoma, one para-aortic paraganglioma and one para-aortic ganglioneuroma encasing the left renal artery); three were being treated with cyclosporine; one was being treat-ed with steroid; and one had hemolytic uremic syndrome. Initial cranial MR images were analyzed with particular emphasis on the distribution of the lesions. To assess possible sequelae, follow-up MR images were obtained in seven patients at least one week after the treatment of hypertension. Four underwent proton MR spectroscopy. Results: Characteristic distribution of lesions in the occipital and posterior parietal lobes was identified in all cases regardless of the causes of RPLS. The cerebellum, basal ganglia, anterior parietal, and frontal lobe were involved in four, two, one, and one case, respectively. Cortical gray matter involvement was predominant in six and subcortical white matter involvement predominated in two patients. The distribution of lesions was bilateral and asymmetric. Gyriform enhancement was identified in six cases, and small hemorrhage was noted in one. In seven patients, the clinical and MR findings improved without sequelae on follow-up study. In one, proton MR spectroscopy demonstrated a high lactate peak at the time of the neurologic event. Nearnormal spectra were noted in three children who underwent proton MR spectroscopy after recovery. Conclusion: The MR findings of RPLS are characteristic in that lesions are distributed in the posterior region of the brain and they are reversible on follow-up study. In children with RPLS due to unknown causes, the possibility of intra-abdominal tumors should also be considered.