Onset Of Posterior Reversible Encephalopathy Syndrome Research Articles

Clinical relevance of reversible cerebral vasoconstriction syndrome in pregnant women with posterior reversible encephalopathy syndrome: review of case reports in Japan.

We systematically reviewed case reports of posterior reversible encephalopathy syndrome (PRES), and investigated the characteristics of PRES in pregnant Japanese women and the clinical relevance of reversible cerebral vasoconstriction syndrome (RCVS) in pregnant women with PRES. Articles were collected using the PubMed/Medline and Ichushi-Web databases. This review was ultimately conducted on 121 articles (162 patients). The clinical characteristics of PRES, individual sites of PRES lesions, edema types, and clinical characteristics of RCVS in PRES cases were examined. The most common individual site of PRES lesion was the occipital lobe (83.3%), followed by the basal ganglia, parietal lobe, frontal lobe, brain stem, cerebellum, temporal lobe, thalamus, and splenium corpus callosum (47.5, 42.6, 24.7, 16.1, 9.3, 5.6, 4.3, and 0.0%, respectively). Edema types in 79 cases with PRES were mainly the vasogenic edema type (91.1%), with very few cases of the cytotoxic edema type (3.8%) and mixed type (5.1%). Among 25 PRES cases with RCVS, RCVS was not strongly suspected in 17 (68.0%) before magnetic resonance angiography. RCVS was observed at the same time as PRES in 13 cases (approximately 50%), and between days 1 and 14 after the onset of PRES in the other 12. These results suggest that the basal ganglia is a frequent site of PRES lesions in pregnant women. RCVS may occur at or after the onset of PRES, even if there are no symptoms to suggest RCVS.

Posterior reversible encephalopathy syndrome in children with acute lymphoblastic leukemia during remission induction chemotherapy: a single-center retrospective study.

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.

Clinical and radiological findings of posterior reversible encephalopathy syndrome in children: About 16 children hospitalized in the pediatric department of a Tunisian tertiary care hospital.

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological entity most frequently described in young- or middle-aged adults with a rare occurrence among children. To determine the clinical, radiological features and outcome of PRES in children admitted to a Tunisian tertiary care pediatric department. we retrospectively reviewed records of all children under 18 years old diagnosed with PRES and admitted to the PICU of the Pediatric department of Sahloul University Hospital from January 2000 to August 2021. Sixteen patients were enrolled in this study. The mean age of the study population at PRES onset was 10 years (range: 4-14 years) and the male female ratio was 3. The most frequent neurological signs were seizures (n=16 cases), headache (n=8 cases), and impaired level of consciousness (7 cases). Visual disturbances were found in one patient. Arterial hypertension was the most underlying cause (16 cases). Brain MRI showed vasogenic edema, mostly localized in the parietal (13 cases) and occipital (11 cases) lobes. Moreover, cytotoxic edema (2 cases), pathologic contrast enhancement (1 case), and hemorrhage (3 cases) were isolated on MRI. The outcome after specific management was favorable after the first onset in 13 cases and death occurred in 3 patients. Relapses were observed in 4 patients. Clinical features presented by children with PRES are variable and non-specific. MRI typically shows reversible posterior cerebral edema. However, in some cases, atypical neuro-imaging findings, such as cytotoxic edema infarction, hemorrhage and contrast enhancement can be observed.

Comparison of Clinical Manifestations, Laboratory, Neuroimaging Findings, and Outcomes in Children With Posterior Reversible Encephalopathy Syndrome (PRES) in Children With and Without Renal Disease

BackgroundTo demonstrate and compare the clinical manifestations, laboratory findings, and neuroimaging findings of posterior reversible encephalopathy syndrome (PRES) in children with and without underlying renal disease. MethodsThe study included 23 children with a diagnosis of PRES from January 2009 to March 2019. All data, including clinical manifestations, laboratory findings, underlying medical illness, and neuroimaging results, were obtained. ResultsSixteen had underlying renal disease. The median age of PRES onset was 10.3 years in children with renal disease and 9.8 years in children without renal disease. Higher blood pressure at the baseline, on admission, and at the onset of PRES was found in the renal disease group more than in the nonrenal disease group (P < 0.05). Seizures were likely seen in the renal disease group compared with the nonrenal disease group (P = 0.03). Generalized tonic-clonic seizures were the most common seizure type in both groups. An initial CT scan revealed vasogenic edema in 75% of the renal group and 85.7% of the nonrenal group. During a long-term follow-up, all children recovered without significant neurological deficits or subsequent epilepsy. ConclusionsHypertension and higher baseline blood pressure are more common in children with renal disease who develop PRES compared with nonrenal disease. Seizures are more common in the renal disease group. A computed tomographic (CT) scan can help with PRES diagnosis when magnetic resonance imaging is not available. All children with PRES recovered without significant neurological deficits or subsequent epilepsy.

Clinical features and prognostic analysis of posterior reversible encephalopathy syndrome in children.

We aimed to investigate the clinical features and prognosis of posterior reversible encephalopathy syndrome (PRES) in children. Clinical data of children with PRES diagnosed at the Children's Hospital of Chongqing Medical University from June 2015 to May 2019 were retrospectively analyzed. The study enrolled 47 patients with a mean age at diagnosis of 8.79 ± 3.72 years (range, 2-15 years). PRES causes included renal disorder (29/47), hematological disease (13/47), and hypertension (5/47). PRES manifested as seizure (43/47), headache (28/47), visual impairment (18/47), dizziness (18/47), vomiting (18/47), and mental and behavioral abnormalities (17/47). Forty-six children had hypertension (46/47) at PRES onset. Magnetic resonance imaging (MRI) mainly involved the parietal and occipital lobes (42/47), 38 cases were mild (38/47), seven were moderate (7/47), and two were severe (2/47). The clinical symptoms of 41 patients (41/47) were relieved within 1-2weeks. Thirty-seven children were followed up for 7-54 months (modified Rankin Scale). Twenty-five children had favorable outcomes (25/37). Twelve children had adverse outcomes (12/37), including epilepsy, disorders of consciousness, visual impairment, and mental decline. Analysis of single factors revealed that severity on MRI, length of in-hospital stay, and mental and behavioral abnormalities were related to adverse outcomes after PRES. Analysis of multiple factors revealed that severity on MRI and length of in-hospital stay were independent risk factors for PRES. Pediatric PRES is a clinical radiographic syndrome with multiple etiologies. Most patients have a good prognosis. Severity on MRI and length of in-hospital stay are independent risk factors.

Long-Term Follow-Up of Patients with a Diagnosis of Posterior Reversible Encephalopathy Syndrome.

Objective:The essential characteristics of posterior reversible encephalopathy syndrome (PRES) are the presence of acute onset neurologic symptoms, focal vasogenic edema at neuroimaging, and reversible clinical and/or radiologic findings. This study aimed to evaluate the clinical findings, causes, radiologic findings, and prognoses of patients with PRES.Methods:Patients with PRES confirmed with clinical and radiologic findings by a pediatric neurologist were evaluated retrospectively.Results:Seventeen patients with PRES were evaluated (mean age at onset, 10.23 ± 4.65 years; range, 2-17 years; girls, 29.4% [n = 5]). The mean length of follow-up was 6 ± 2.3 years (range, 3.4-10 years). Mortality due to primary disease occurred in 4 patients (23.5%) during follow-up. PRES was derived from renal diseases in 10 patients (58.8%), hematologic diseases in 6 patients (35.3%), and liver disease in one patient (5.9%). Hypertension was present in 16 patients (94.1%) at onset of PRES (>99th percentile). Seizure, the most frequent initial symptom, was observed in 82.4% (n = 14). Blurred vision and headache were the initial symptoms in 3 patients (17.6%). Sequelae were observed at magnetic resonance imaging (MRI) in 6 patients. Development of epilepsy was determined as a sequela in 4 patients (23.5%) and mental motor retardation in 2 patients (11.8%).Conclusion:Epilepsy is uncommon in patients who have recovered from PRES. The presence of gliosis on MRI and interictal epileptic discharges on electroencephalograms are major risk factors for the development of epilepsy. Antiepileptic treatment can be stopped in the early period in patients with normal MRI and electroencephalogram by eliminating the factors that trigger the seizures.

Posterior reversible encephalopathy syndrome in children with acute lymphoblastic leukemia during remission induction chemotherapy: a single-center retrospective study.

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.

Prognostic factors and seizure outcome in posterior reversible encephalopathy syndrome (PRES) in children with hematological malignancies and bone marrow failure: A retrospective monocentric study

PurposeThe aim of this study was to evaluate seizure outcome in children with hematological malignancies and PRES and to identify prognostic factors that could help manage the syndrome. MethodWe retrospectively reviewed the report data of 21 patients diagnosed with hematological malignancy or aplastic anemia and PRES between 2008 and 2018. Basic demographic data, oncology treatment, presymptomatic hypertension before PRES manifestation, neurological status, seizure type, and EEG and MRI findings at PRES onset and at the one-year follow-up visit were studied. Patients who developed remote symptomatic seizures or epilepsy were identified. ResultsWe included 21 children (11 females and 10 males) in the study. Sixteen patients (76.2%) were diagnosed with ALL and the rest individually with AML, CML, T-lymphoma, Burkitt lymphoma, and severe aplastic anemia. Presymptomatic hypertension (PSH) was evaluated in 19 patients and was present in 18 (94.7%). The duration was 9 h and more in 16 patients (88.8%); the severity was grade II in 12 patients (66.7%). Seizures as the initial symptom of PRES were present in 17 patients (80.9%). Four patients (19.0%) were assessed with remote symptomatic seizures. Two of them (9.5%) had ongoing seizures at the one-year follow-up visit and were diagnosed with epilepsy. The presence of gliosis on follow-up MRI indicated worse outcome with development of epilepsy (without statistical significance). ConclusionsPRES syndrome has an overall good prognosis and the evolution to epilepsy is rare. The severity and duration of PSH or seizure severity and EEG findings at PRES onsetwere not associated with worse neurological outcomes in this study.

Clinical features, outcome, and associated factors for posterior reversible encephalopathy in Thai patients with systemic lupus erythematosus: a case-control study.

Posterior reversible encephalopathy syndrome (PRES) in patients with systemic lupus erythematosus (SLE) has been recognized increasingly. This study aimed to determine the prevalence, clinical features, brain imaging findings, outcomes, and associated factors of PRES in Thai SLE patients. SLE patients with PRES were identified from the lupus cohort of Chiang Mai University. Controls were SLE patients with a hospital number close to and actually had SLE diagnosis within 5 years of the case(case:control ratio = 1:4). Of 1,332 SLE patients, 30 episodes of PRES were identified in 24 female SLE patients (prevalence 1.80%). The mean ± SD age at SLE diagnosis and at onset of PRES was 25.02 ± 13.78 and 28.31 ± 12.61years, respectively. Seizure was the most common presenting symptom, as seen in 28 episodes, followed by acute severe headache in 17, alteration of consciousness in 17, nausea and vomiting in 10, blurred vision in 11, and hemiparesis in 3. Abrupt increase in blood pressure and active nephritis were seen in 29 and 26 of the episodes, respectively. Urine protein/creatinine ratio >1.00 (OR 15.72, 95% CI 3.12-79.12, p=0.001) and hemoglobin <10 gm/dL (OR 5.12, 95% CI 1.37-19.15, p=0.015) were associated factors for developing PRES. During the observation period, 7 patients in the PRES group and 8 in the control group died (p=0.015). PRES was uncommon in SLE patients, but associated with a high mortality rate. Active nephritis and anemia were associated factors of PRES in Thai SLE patients.

Posterior reversible encephalopathy syndrome in cancer patients: a single institution retrospective study.

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiologic entity. Its management and outcome in the oncology population is limited because it is still difficult to identify despite an increasingly recognized occurrence. This is the largest retrospective study of PRES in cancer patients reported from a single institution. We explore the clinical manifestations and radiologic features to comprehensively assess PRES in order to prevent permanent neurologic deficits and mortality. We retrospectively identified 69 patients with cancer who developed PRES at MDACC between 01/2006 to 06/2012. Clinical and radiographic data were abstracted from their records and reviewed for our analysis. Mean age at PRES onset was 52±17.8years. Fifty-two (75%; p<0.001) patients were women. Most common diagnoses were leukemia (30%) and lymphoma (12%). Forty-eight (70%) patients were treated with chemotherapy, 21 (30%) bone marrow transplant and 14 (20%) tacrolimus. Most common clinical presentation was seizures (67%). PRES was associated with hypertension in 62 (90%) patients. On brain MRI, 33 (44%) patients had some evidence of hemorrhage, 22 (73%) of these were thrombocytopenic. Thirty-five (51%) patients fully recovered and 19 (28%) had permanent neurological deficits. Morbidity and mortality were associated with continuation with offending agent, thrombocytopenia, variations in mean arterial pressure ≥20mmHg, electrographic seizures at onset, atypical MRI pattern and delay in diagnostic imaging (7.4±4.8days vs. 1.9±1.8days; p=0.031) as half of them did not receive a prompt intervention. Special attention should be given to patients who present with high-risk factors in order to prevent development of PRES or decrease patient morbidity and mortality. Management of PRES should be guided by the radiographic findings. Overall, early recognition, discontinuation of the offending agents, correction of thrombocytopenia and blood pressure control are still the main strategies to manage PRES.

Epileptic Ictal Hyperperfusion on Arterial Spin Labeling Perfusion and Diffusion-Weighted Magnetic Resonance Images in Posterior Reversible Encephalopathy Syndrome

The hemodynamic state of the posterior dominant vasogenic edema in posterior reversible encephalopathy syndrome (PRES) is controversial. The aim of this retrospective study was to examine the contribution of epileptic ictal hyperperfusion in patients with PRES using combined magnetic resonance perfusion imaging with arterial spin labeling (ASL) and diffusion-weighted magnetic resonance imaging (MRI). A detailed review of chronological MRI findings in 2 patients, including diffusion-weighted imaging (DWI) and ASL, with special reference to clinical and electroencephalographic findings, was performed. At the onset of PRES, both patients developed secondary generalized seizures. At the first PRES episode in Case 1, ASL and DWI clearly depicted "ictal hyperperfusion" and prolonged epilepsy-induced cytotoxic edema in the left parieto-occipital lobe cortex, located around the vasogenic edema of the PRES lesion in the left occipital lobe (hypoperfused area). At the second and third episodes (2 and 7 months after the first episode, respectively), although recurrent PRES was ruled out, ASL and DWI clearly demonstrated ictal hyperperfusion in the left posterior temporal and parieto-occipital lobes associated with partial nonconvulsive status epilepticus, which developed around the PRES-related old hematoma lesion. In Case 2, peri-ictal MRI findings of ictal ASL hyperperfusion and cortical hyperintensity on DWI were also noted in the left parieto-occipital lobe, but were mild compared with Case 1. Combined use of DWI and ASL can provide information on hemodynamic state associated with epileptic ictal hyperperfusion in the various phases of PRES.

Primary brain tumors and posterior reversible encephalopathy syndrome.

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic encephalopathic state associated with reversible cerebral vasogenic edema. It is an increasingly recognized occurrence in the oncology population. However, it is very uncommon in patients with primary brain tumors (PBTs). The aim of this study was to analyze the clinicoradiological features and report the clinical outcomes of PRES in PBT patients. We identified 4 cases with PBT who developed PRES at MD Anderson Cancer Center (MDACC) between 2012 and 2014. Clinical and radiological data were abstracted from their records. In addition, we also solicited 8 cases from the literature. The median age at PRES onset was 19 years, male-to-female ratio was 1:1, and the syndrome occurred in patients with ependymoma (n = 4), glioblastoma (n = 3), diffuse intrinsic pontine glioma (DIPG; n = 3), juvenile pilocytic astrocytoma (n = 1), and atypical meningioma (n = 1). Two glioblastomas and 2 DIPG cases received bevacizumab and vandetanib before the onset of symptoms, respectively. The most common clinical presentation was seizures (n = 7). Three MDACC patients recovered completely in 3-4 weeks after the onset of symptoms. One patient died due to active cancer and several comorbidities including PRES. Hypertension seems to be the most important coexisting risk factor for development of PRES; however, the potential effects of chemotherapeutic agents in the pathogenesis of PRES should also be examined. The clinicoradiological course of PRES in PBT patients did not vary from the classical descriptions of PRES found in other causes. PRES must be considered as part of the differential diagnosis in patients with PBTs presenting with seizures or acute encephalopathy.

Elevation of serum lactate dehydrogenase at posterior reversible encephalopathy syndrome onset in chemotherapy-treated cancer patients

The pathophysiology of posterior reversible encephalopathy syndrome (PRES) is incompletely understood; however, an underlying state of immune dysregulation and endothelial dysfunction has been proposed. We examined alterations of serum lactate dehydrogenase (LDH), a marker of endothelial dysfunction, relative to the development of PRES in patients receiving chemotherapy. A retrospective Institutional Review Board approved database of 88 PRES patients was examined. PRES diagnosis was confirmed by congruent clinical diagnosis and MRI. Clinical features at presentation were recorded. Serum LDH values were collected at three time points: prior to, at the time of, and following PRES diagnosis. Student’s t-test was employed. LDH values were available during the course of treatment in 12 patients (nine women; mean age 57.8years [range 33–75years]). Chemotherapy-associated PRES patients were more likely to be normotensive (25%) versus the non-chemotherapy group (9%). LDH levels at the time of PRES diagnosis were higher than those before and after (p=0.0263), with a mean difference of 114.8 international units/L. Mean time intervals between LDH measurement prior to and following PRES diagnosis were 44.8days and 51.4days, respectively. Mean elapsed time between last chemotherapy administration and PRES onset was 11.1days. In conclusion, serum LDH, a marker of endothelial dysfunction, shows statistically significant elevation at the onset of PRES toxicity in cancer patients receiving chemotherapy. Our findings support a systemic process characterized by endothelial injury/dysfunction as a factor, if not the prime event, in the pathophysiology of PRES.

Life-threatening complications of posterior reversible encephalopathy syndrome in children

BackgroundAlthough the posterior reversible encephalopathy syndrome (PRES) is considered to have a benign clinical outcome, the presentation of PRES can be associated with life-threatening complications such as severe cerebral hemorrhage, cerebellar herniation and refractory status epilepticus (SE). The aim of this paper is to report incidence, clinical features and outcome of life-threatening complications related to PRES in children. MethodsPatients who suffered from life-threatening complications were retrospectively identified from a group composed by 27 consecutive children diagnosed with PRES in our hospital between 2000 and 2012. The clinical, radiological and EEG features and the outcome of these patients were evaluated and compared to the characteristics of patients with no complications. ResultsFive patients (18%) presented life-threatening complications: 2 cerebral hemorrhages with mass effect and midline shift (1 massive intraparenchymal hemorrhage and 1 subdural hemorrhage and intraparenchymal hemorrhage), 2 transforaminal cerebellar herniations and 1 refractory SE. Two children died because of complications and 2 children required urgent neurosurgical intervention. The infratentorial involvement at onset of PRES and the observation of focal neurological deficits other than visual disturbances were significantly more frequent in children with life-threatening complications (p < 0.01). ConclusionsPRES is associated with a non-negligible incidence of life-threatening complications. A careful clinical, neuroradiological and EEG monitoring is necessary in order to improve the outcome especially in the case of focal neurological deficits and infratentorial involvement.

Bortezomib-induced Posterior Reversible Encephalopathy Syndrome in a Patient with Newly Diagnosed Multiple Myeloma

Posterior reversible encephalopathy syndrome (PRES) is a known but extremely rare side effect of bortezomib therapy. An unusual case of PRES possibly caused by bortezomib during induction treatment in a patient with multiple myeloma is reported. The patient experienced neither hypertensive crisis nor uremic encephalopathy at the onset of PRES, which are both well-known etiologies of PRES. The patient's PRES-related symptoms resolved completely after discontinuation of bortezomib and administration of a bulk dose of corticosteroids. The importance of early recognition of this potential neurological complication must be emphasized because this new drug is being increasingly prescribed.

Tacrolimus-Associated Ischemic Optic Neuropathy and Posterior Reversible Encephalopathy Syndrome After Small Bowel Transplantation

Tacrolimus-Associated Ischemic Optic Neuropathy and Posterior Reversible Encephalopathy Syndrome After Small Bowel Transplantation

Posterior reversible encephalopathy syndrome in children: Radiological and clinical findings – A retrospective analysis of a German tertiary care center

PurposeTo report the radiological and clinical spectrum of posterior reversible encephalopathy syndrome (PRES) in children in a German tertiary referral center. MethodsThe radiological report data bases of the authors' university hospitals were searched for paediatric patients with PRES. Clinical and paraclinical data as well as various imaging features at symptom onset and during follow-up were tabulated in patients fulfilling the criteria for PRES. ResultsA total of 18 paediatric patients with PRES were included into the study. Mean age was 9 years (IQR 7–12), 38.9% were females. Most frequent predisposing causes were renal and haemato-oncologic diseases frequently associated with endotheliotoxic cytostatic medication. Frontal lesions occurred as frequently as parietal lesions followed by occipital lesions. The superior frontal sulcus topographic lesion pattern occurred as frequent as the parieto-occipital one. In 38% of cases residual lesions were encountered with focal laminar necroses being most frequent. Initial clinical syndromes associated with PRES included seizures in 18, altered mental state in 5, and hemiparesis and visual disturbances in 2 children. Mean arterial blood pressure at onset of PRES was 140/85 mmHg (IQR systolic: 124–169, diastolic: 78–93 mmHg). ConclusionPaediatric PRES in this cohort comprises a broad radiological and clinical spectrum. The occurrence of frontal lesions, a superior frontal sulcus associated lesion pattern, and the development of focal laminar necrosis appear to be frequent in children.

A Case of Posterior Reversible Encephalopathy Syndrome Following Hypopharyngeal Cancer Surgery

Posterior reversible encephalopathy syndrome (PRES) features headache, seizures, abnormal mental state, altered visual perception, and hypertension. While rare, it has become increasingly identifiable with clinicoradiological processes in cancer associated with cytotoxic and immunosuppressive agents. Such processes include vasogenic edema mainly involving the white matter of the parietooccipital lobes. PRES is reversible when adequately managed. Failure to quickly recognize it may, however, result in profound, permanent central nervous system dysfunction or death. We reported a 48-year-old woman with advanced hypopharyngeal cancer who developed PRES after total pharyngolaryngoesophagectomy. Her condition gradually improved and she recovered completely without neurological deficit 46 days after PRES onset.

Epilepsy after neuroimaging normalization in a woman with tacrolimus-related posterior reversible encephalopathy syndrome

Posterior reversible encephalopathy syndrome (PRES) is known to occur after solid organ transplantation, and is caused by immunosuppressive agents such as tacrolimus. PRES onset usually occurs within the first 2months after liver transplantation. Clinical findings include seizures, headache, focal neurological deficits, visual disturbances, and altered mental status. These are associated with characteristic imaging features of subcortical white matter lesions on brain MRI. Atypical localizations of this posterior leukoencephalopathy have been reported. Expeditious recognition of the syndrome may lead to a complete recovery. Abnormalities of EEG during follow-up might be associated with unfavorable seizure outcome, even when neuroimaging changes resolve. We report a case of late-onset PRES with atypical localization following liver transplantation. The patient developed epilepsy despite resolution of MRI lesions at 8months of follow-up. EEG was a prognostic factor of seizure persistence, suggesting an incomplete recovery of brain lesions in contrast to neuroimaging findings.

Nephrotic state as a risk factor for developing posterior reversible encephalopathy syndrome in paediatric patients with nephrotic syndrome

Posterior reversible encephalopathy syndrome (PRES) is a distinctive and potentially serious complication of the nephrotic syndrome. The objective of the present study is to characterize the factors predisposing the development of PRES in paediatric patients with nephrotic syndrome. We investigated paediatric patients with idiopathic nephrotic syndrome who developed PRES between 1999 and 2005 in our institution. Patients with steroid-sensitive nephrotic syndrome and those with steroid-resistant nephrotic syndrome that were proven to be idiopathic were eligible. In total, seven patients ranging in age from 1.5 to 15.1 years old were analysed. At the onset of PRES, six of the seven patients were in a nephrotic state. Various degrees of acute renal insufficiency were shown in four patients. The re-administration of cyclosporine after the episodes of PRES was carried out in four patients. During the observation for 17-51 months after the re-administration, the recurrence of PRES did not develop in these patients. The development of PRES occurred at the time of moderate to severe nephrotic state in most of our paediatric patients with nephrotic syndrome. Besides the administration of cyclosporine and having hypertension, there appear to be several additive factors predisposing the development of PRES in these patients, namely low serum albumin level, generalized oedema, increase in vascular permeability, unstable fluid status and renal insufficiency. The re-administration of cyclosporine to those patients with anamnesis of PRES may be considered after the management and close monitoring of these factors as well as hypertension.