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1.
BMC Neurol ; 21(1): 107, 2021 Mar 09.
Article En | MEDLINE | ID: mdl-33750332

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is usually a benign, yet underdiagnosed clinical condition associated with subacute to acute neurological manifestations primarily affecting white matter. PRES is reversible when recognized promptly and treated early by removal of the insulting factor; however, can lead to irreversible and life-threatening complications such as cerebral hemorrhage, cerebellar herniation, and refractory status epilepticus. METHODS: We utilized the National Inpatient Sample database provided by the Healthcare Cost and Utilization Project (HCUP-NIS) 2017 to investigate the demographic variables (age, sex, and race) for patients with PRES, concomitant comorbidities and conditions, inpatient complications, inpatient mortality, length of stay (LOS), and disposition. RESULTS: A total of 635 admissions for patients aged 18 years or older with PRES were identified. The mean age was 57.2 ± 0.6 years old with most encounters for female patients (71.7%, n = 455) and white as the most prevalent race. Half the patients in our study presented with seizures (50.1%, n = 318), sixty-three patients (9.9%) presented with vision loss, and sixty-four patients (10.1%) had speech difficulty. In addition, 45.5% of patients had hypertensive crisis (n = 289). 2.2% of hospitalizations had death as the outcome (n = 14). The mean LOS was 8.2 (±0.3) days, and the mean total charges were $92,503 (±$5758). Inpatient mortality differed between males and females (1.7% vs. 2.4%) and by race (3.6% in black vs. 1.8% in white) but was ultimately determined to be not statistically significant. Most patients who present with vision disturbance have a high risk of intracranial hemorrhage. Furthermore, end-stage renal disease, atrial fibrillation, and malignancy seemed to be linked with a very high risk of mortality. CONCLUSION: PRES, formerly known as reversible posterior leukoencephalopathy, is a neurological disorder with variable presenting symptoms. Although it is generally a reversible condition, some patients suffer significant morbidity and even mortality. To the best of our knowledge, this is the largest retrospective cohort of PRES admissions that raises clinician awareness of clinical characteristics and outcomes of this syndrome.


Posterior Leukoencephalopathy Syndrome , Cerebral Hemorrhage , Female , Humans , Length of Stay/statistics & numerical data , Male , Middle Aged , Posterior Leukoencephalopathy Syndrome/complications , Posterior Leukoencephalopathy Syndrome/diagnosis , Posterior Leukoencephalopathy Syndrome/epidemiology , Posterior Leukoencephalopathy Syndrome/mortality , Retrospective Studies
2.
Neurologist ; 25(6): 162-167, 2020 Nov.
Article En | MEDLINE | ID: mdl-33181724

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is an acute neurological condition with unknown global incidence, variable clinical presentation, and prognosis. OBJECTIVES: To describe a cohort of patients with PRES with a focus on brain magnetic resonance imaging (MRI) patterns and their relationship with short-term clinical outcomes. METHODS: Retrospective cohort study. The authors included patients if they were older than 15 years and had a PRES diagnosis on the basis of a positive brain MRI at any time during the in-hospital stay. RESULTS: Forty-four patients were included in the present analysis. The median age was 57 years (interquartile range, 32.0-68.5) and 70.5% were women. Hypertension (59.1%), history of transplantation (27.3%), previous chemotherapy (27.3%), chronic renal failure (38.6%), and autoimmune disease (15%) were the main comorbid conditions present. The classic triad of seizures, headache, and visual impairment was present in 18.0% of the cases. Eighty-six percent of patients were admitted to the intensive care unit, with 36.0% needing invasive life support. Brain MRI showed a dominant parieto-occipital pattern in 26 patients, whereas cytotoxic edema and bleeding were present in 27.3% and 29.6%, respectively. In-hospital mortality was 11.4%. The median modified Rankin Scale at hospital discharge was 1 (0-2.5). Risk factors associated with low modified Rankin Scale scores were: headache, visual impairment, and parieto-occipital pattern. Decreased level of consciousness and mechanical ventilation requirement were associated with greater discharge disability. CONCLUSIONS: Characteristic symptoms and signs of PRES and classic MRI patterns are associated with better clinical outcomes.


Outcome Assessment, Health Care , Posterior Leukoencephalopathy Syndrome/pathology , Posterior Leukoencephalopathy Syndrome/physiopathology , Adult , Aged , Comorbidity , Female , Headache/etiology , Hospital Mortality , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Posterior Leukoencephalopathy Syndrome/complications , Posterior Leukoencephalopathy Syndrome/mortality , Retrospective Studies , Risk Factors , Seizures/etiology , Vision Disorders/etiology
3.
Pregnancy Hypertens ; 12: 35-39, 2018 Apr.
Article En | MEDLINE | ID: mdl-29674196

OBJECTIVE: To study the clinical and imaging profile of patients with new-onset seizures with a presumptive diagnosis of eclampsia. METHODS: This was a cross-sectional study, conducted in a tertiary teaching hospital, on pregnant women presenting with new onset seizures with presumptive diagnosis of eclampsia excluding those with pre-existing neurological conditions. Demographic details, medical and obstetric examination findings were noted. All women underwent neuroimaging within 5 days of onset seizures. RESULTS: Presumptive diagnosis of eclampsia was made in 0.7% (n = 186) of women delivering during the time period. Most women (55.4%) presented with seizures in the antenatal period. Neuroimaging is performed in 130 cases and it was found to be abnormal in 45.4% of women (59/130). Most common associated neurological condition was Posterior Reversible Encephalopathy Syndrome in 20% (n = 26) followed by Cerebral Venosus Sinus Thrombosis in 10% (n = 14). All six women with primary intracerebral haemorrhage succumbed to the disease. CONCLUSION: New-onset seizures may be the initial presentation of uncommon and unpredictable complication of pregnancy with serious maternal/ fetal morbidity and mortality. Neuroimaging will help in these patients to avoid the delay or misdiagnosis, resulting in early initiation of specific treatment which will help to improve and optimize outcomes.


Eclampsia/diagnostic imaging , Neuroimaging/methods , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Seizures/diagnostic imaging , Sinus Thrombosis, Intracranial/diagnostic imaging , Adult , Cross-Sectional Studies , Diagnosis, Differential , Eclampsia/mortality , Eclampsia/therapy , Female , Hospitals, Teaching , Humans , Maternal Mortality , Posterior Leukoencephalopathy Syndrome/complications , Posterior Leukoencephalopathy Syndrome/mortality , Posterior Leukoencephalopathy Syndrome/therapy , Predictive Value of Tests , Pregnancy , Prognosis , Prospective Studies , Risk Factors , Seizures/etiology , Seizures/mortality , Seizures/therapy , Sinus Thrombosis, Intracranial/complications , Sinus Thrombosis, Intracranial/mortality , Sinus Thrombosis, Intracranial/therapy , Tertiary Care Centers , Young Adult
4.
J Pediatr Hematol Oncol ; 40(6): 433-437, 2018 08.
Article En | MEDLINE | ID: mdl-29697577

Posterior reversible encephalopathy syndrome (PRES) has diverse etiologies and is closely linked to hematopoietic stem cell transplant (HSCT). Headache and seizures are the most common clinical presentations. Although near total recovery is seen in the majority of patients with appropriate management, the implications of its occurrence in the setting of an HSCT is much more than the residual neurological deficits. Graft rejection and occurrence of graft versus host disease has been reported. We analyzed retrospectively our data of 35 pediatric HSCT recipients over the last 2 years at our center. In total, 17% (n=6) patients developed PRES. Headache and seizures were the most common clinical presentations. All patients were on calcineurin inhibitors at the onset of symptoms. The median time after HSCT to the onset of PRES was 21 days. In total, 34% (n=2) patients developed residual neurological deficit. One patient died of acute graft versus host disease at a later date, and 50% (n=3) patients had graft rejection and return to transfusion dependence. The implications of PRES on HSCT outcomes are grave, and better immunosuppression transition protocols need to be developed.


Calcineurin Inhibitors/administration & dosage , Graft Rejection/drug therapy , Graft vs Host Disease/drug therapy , Hematopoietic Stem Cell Transplantation , Posterior Leukoencephalopathy Syndrome/drug therapy , Child , Female , Graft Rejection/etiology , Graft Rejection/mortality , Graft vs Host Disease/etiology , Graft vs Host Disease/mortality , Humans , Male , Posterior Leukoencephalopathy Syndrome/etiology , Posterior Leukoencephalopathy Syndrome/mortality , Retrospective Studies
5.
Biol Blood Marrow Transplant ; 23(9): 1531-1540, 2017 Sep.
Article En | MEDLINE | ID: mdl-28602890

Posterior reversible encephalopathy syndrome (PRES) is a serious adverse event associated with calcineurin inhibitors used for graft-versus-host disease (GVHD) prophylaxis. We compared the incidence of PRES in children with thalassemia (n = 222, 1.4 to 17.8 years old) versus sickle cell disease (SCD; n = 59, 2 to 17 years old) who underwent hematopoietic cell transplantation from HLA-matched siblings or alternative donors and analyzed the risk factors for PRES. Overall, 31 children developed calcineurin inhibitor-related PRES (11%), including 30 patients with seizures and 1 patient without seizures. PRES incidence was significantly higher in SCD patients (22%; 95% confidence interval [CI], 10% to 32%) than in thalassemia patients (8%; 95% CI, 5% to 12%;P = .002). In multivariate analysis, factors associated with PRES were hypertension (hazard ratio [HR], 5.87; 95% CI, 2.57 to 13.43; P = .0001), SCD (HR, 2.49; 95% CI, 1.25 to 4.99; P = .009), and acute GVHD (HR 2.27; 95% CI, 1.06 to 4.85; P= .031). In the entire cohort overall survival (OS) was significantly higher in patients without versus with PRES (90% versus 77%; P = .02). In a subgroup analysis that including matched sibling transplants, OS and disease-free survival (DFS) were similar in thalassemia patients without PRES (92% and 88%, respectively) and with PRES (82% and 73%, respectively), whereas SCD patients with PRES had significantly lower OS (67%) and DFS (67%) than patients without PRES (94% and 94%, respectively; P = .008). Thus, SCD patients had a significantly higher incidence of PRES than thalassemia patients, and hypertension and GVHD were the 2 main risk factors for PRES in patients with hemoglobinopathies. Although PRES did not significantly influence survival in patients with thalassemia, patients with SCD had significantly lower survival after PRES.


Anemia, Sickle Cell/therapy , Calcineurin Inhibitors/adverse effects , Hematopoietic Stem Cell Transplantation , Immunosuppressive Agents/adverse effects , Posterior Leukoencephalopathy Syndrome/therapy , Seizures/therapy , beta-Thalassemia/therapy , Acute Disease , Adolescent , Anemia, Sickle Cell/immunology , Anemia, Sickle Cell/mortality , Anemia, Sickle Cell/pathology , Calcineurin Inhibitors/administration & dosage , Child , Child, Preschool , Cohort Studies , Female , Graft vs Host Disease/immunology , Graft vs Host Disease/mortality , Graft vs Host Disease/pathology , Graft vs Host Disease/prevention & control , Humans , Hypertension/diagnosis , Hypertension/physiopathology , Immunosuppressive Agents/administration & dosage , Infant , Male , Posterior Leukoencephalopathy Syndrome/chemically induced , Posterior Leukoencephalopathy Syndrome/immunology , Posterior Leukoencephalopathy Syndrome/mortality , Risk Factors , Seizures/chemically induced , Seizures/immunology , Seizures/mortality , Siblings , Survival Analysis , Transplantation, Homologous , Unrelated Donors , beta-Thalassemia/immunology , beta-Thalassemia/mortality , beta-Thalassemia/pathology
6.
Ir J Med Sci ; 186(3): 699-705, 2017 Aug.
Article En | MEDLINE | ID: mdl-28155100

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is associated with variable predisposing risk factors including preeclampsia and eclampsia since it proposed. However, studies of large case series focusing on pregnancy-related PRES are still limited. We performed a large retrospective study of patients with pregnancy-related PRES admitted to our institution. METHODS: This was a single-center, 2010-2015 retrospective cohort study of patients with pregnancy-related PRES who underwent neuroimaging via magnetic resonance imaging or computerized tomography from mainland China. RESULTS: 26 of 28 women with eclampsia and 7 of 59 women with preeclampsia had confirmed PRES. A total of 36 patients were finally included as confirmed pregnancy-related PRES in this research. Acute hypertension was present in 31 patients (86%). Headache was the most common presenting symptom (81%) followed by seizures (73%), altered mental status (57%), nausea/vomiting (47%) and visual disturbance (33%). Atypical involved regions included frontal lobe (72%), temporal lobe (67%), basal ganglia (50%), cerebellum (47%), brain stem (14%) and thalamus (8%). Atypical neuroimaging features included restricted diffusion (33%), contrast enhancement (19%) and hemorrhage (19%). Comorbidities included thrombocytopenia (25%), pulmonary infection (25%), anemia (19%), fever (17%), acute renal failure (8%), HELLP syndrome (6%), DIC (6%). Most of patients recovered completely with timely diagnosis and treatment. Two patients who suffered DIC finally died. CONCLUSIONS: Patients with pregnancy-related PRES may present with atypical neuroimaging findings. Moreover, our data supported the view that nearly all imaged patients with eclampsia had clinical and radiologic findings of PRES.


Posterior Leukoencephalopathy Syndrome/etiology , Adolescent , Adult , China , Cohort Studies , Female , Humans , Posterior Leukoencephalopathy Syndrome/mortality , Posterior Leukoencephalopathy Syndrome/pathology , Pregnancy , Retrospective Studies , Risk Factors , Young Adult
7.
J Neurol ; 264(2): 237-242, 2017 Feb.
Article En | MEDLINE | ID: mdl-27815684

Although often reversible, fatal outcome in posterior reversible encephalopathy syndrome (PRES) is well known. However, data on predictors of PRES-associated in-hospital death are scarce. In this study, we aimed to investigate predictors of in-hospital death in a large cohort. Radiological report databases between January 1999 and February 2015 were retrospectively searched for patients with PRES. Patients were included if they met criteria for PRES after detailed investigation of clinical charts and imaging studies. Various clinical, paraclinical and brain MRI data as well as data on in-hospital mortality were analyzed. 151 patients were included into the study, 64% were female. Seventeen (11.2%) patients died during hospital stay. In univariate analyses, higher age (p = 0.04), higher levels of C-reactive protein (p < 0.001), etiology of PRES (sepsis and chemotherapy; p = 0.02), altered coagulation (p = 0.002), altered mental state at onset (p = 0.03), and subarachnoid hemorrhage (SAH; p = 0.01) were related to in-hospital death. In multivariate analyses adjusted for age and sex, elevated CRP levels (OR 1.1 95% CI 1.1-1.2), altered coagulation (OR 5.1 95% CI 1.8-14.7), subarachnoid hemorrhage (OR 10.1 95% CI 2.2-46.1) and altered mental state (OR 3.3; 95% CI 1.1-9.4) were independently associated with in-hospital death. Altered mental state, subarachnoid hemorrhage as well as the higher levels of CRP and altered coagulation were significantly more frequent in patients who died in hospital. However, prospective studies are warranted to establish predictors of fatality in patients with PRES.


Posterior Leukoencephalopathy Syndrome/mortality , Adult , Age Factors , Berlin , Biomarkers/metabolism , C-Reactive Protein/metabolism , Factor Analysis, Statistical , Female , Hospital Mortality , Humans , Logistic Models , Male , Middle Aged , Multivariate Analysis , Posterior Leukoencephalopathy Syndrome/metabolism , Posterior Leukoencephalopathy Syndrome/therapy , Retrospective Studies , Young Adult
8.
Clin Lymphoma Myeloma Leuk ; 15(10): 606-11, 2015 Oct.
Article En | MEDLINE | ID: mdl-26184063

BACKGROUND: Central nervous system complications (CNSC) can be the cause of morbidity and mortality in patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT). We aimed to determine the incidence of CNSC and its impact on survival. PATIENTS AND METHODS: This retrospective cohort study included patients with hematologic disorders who received allo-HSCT between 2002 and 2011 at the University of Nebraska Medical Center. RESULTS: Of the 351 patients identified, 45 developed CNSC (12.8%). The 100-day cumulative incidence of CNSC was 8% (95% confidence interval, 8-15). The most common CNSC included posterior reversible encephalopathy syndrome (40%), stroke or transient ischemic attack (24%), seizures (20%), and infection (9%). The 5-year overall survival was significantly lower among patients with versus without CNSC (14% vs. 44%, P = .0004). In multivariate analysis, the risk of mortality for patients with versus without CNSC was significantly higher (hazard ratio, 1.56; 95% confidence interval, 1.03-2.36; P = .04). CONCLUSION: The occurrence of CNSC after allo-HSCT was associated with reduced survival. Identifying patients at risk, monitoring, early detection, and management of CNSC after allo-HSCT are needed to improve outcomes.


Hematopoietic Stem Cell Transplantation/adverse effects , Leukemia, Myeloid, Acute/therapy , Posterior Leukoencephalopathy Syndrome/etiology , Seizures/etiology , Stroke/etiology , Adolescent , Adult , Aged , Child , Child, Preschool , Female , Humans , Infant , Leukemia, Myeloid, Acute/mortality , Male , Middle Aged , Multivariate Analysis , Posterior Leukoencephalopathy Syndrome/mortality , Proportional Hazards Models , Retrospective Studies , Risk Factors , Seizures/mortality , Stroke/mortality , Transplantation, Homologous , Treatment Outcome , Young Adult
9.
Biol Blood Marrow Transplant ; 21(11): 2028-32, 2015 Nov.
Article En | MEDLINE | ID: mdl-26226408

Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome characterized by vision changes, altered mental status, and seizures, typically caused by an acute rise in blood pressure. PRES has been reported after hematopoietic stem cell transplantation (HSCT) in association with hypertension from calcineurin inhibitors and corticosteroids. The imaging evaluation of PRES after HSCT in children and young adults has not been well described. We performed a retrospective review of all HSCT recipients presenting to the intensive care unit with new neurologic symptoms. A neuroradiologist reviewed all radiologic images and compared computed tomography (CT) versus magnetic resonance imaging (MRI) findings indicative of diagnosis of PRES. Alternative imaging diagnoses explaining the patients' symptoms were also recorded. Fifty-four transplant recipients were admitted to the intensive care unit with new neurologic symptoms. Thirty-nine percent (21 of 54) of subjects had imaging findings consistent with PRES, 24% (13 of 54) had imaging findings consistent with an alternative diagnosis, 9% (5 of 54) had a nonspecific finding, and 28% (15 of 54) had no acute imaging findings. PRES was diagnosed at a median of 49 days (interquartile range, 29 to 94) after HSCT. The presenting symptom for the majority of patients with PRES was seizures (86%), whereas 14% presented with acute encephalopathy. Ninety-five percent of subjects diagnosed with PRES (20 of 21) underwent a head CT as their initial imaging evaluation. CT scan was diagnostic of PRES in 40% (8 of 20). Subsequently, 16 patients underwent brain MRI with 12 additional patients being diagnosed with PRES on MRI. The median time elapsed between negative CT and a positive MRI examination was 20 hours (range, 3.6 hours to 9 days). CT serves as an excellent screening test for acute pathology, such as intracranial hemorrhage; however, it lacks sensitivity for the diagnosis of PRES. Patients with clinical symptoms suggestive of PRES who have a negative CT should be treated appropriately for PRES and should undergo MRI of the brain as soon as clinically stable to confirm the diagnosis.


Hematopoietic Stem Cell Transplantation , Hypertension/diagnosis , Myeloablative Agonists/therapeutic use , Posterior Leukoencephalopathy Syndrome/diagnosis , Transplantation Conditioning , Adolescent , Anemia, Aplastic , Blood Pressure , Bone Marrow Diseases , Bone Marrow Failure Disorders , Child , Child, Preschool , Female , Graft vs Host Disease/immunology , Graft vs Host Disease/mortality , Graft vs Host Disease/pathology , Graft vs Host Disease/therapy , Hematologic Neoplasms/immunology , Hematologic Neoplasms/mortality , Hematologic Neoplasms/pathology , Hematologic Neoplasms/therapy , Hemoglobinuria, Paroxysmal/immunology , Hemoglobinuria, Paroxysmal/mortality , Hemoglobinuria, Paroxysmal/pathology , Hemoglobinuria, Paroxysmal/therapy , Humans , Hypertension/diagnostic imaging , Hypertension/mortality , Hypertension/therapy , Immunologic Deficiency Syndromes/immunology , Immunologic Deficiency Syndromes/mortality , Immunologic Deficiency Syndromes/pathology , Immunologic Deficiency Syndromes/therapy , Intensive Care Units , Magnetic Resonance Imaging , Male , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Posterior Leukoencephalopathy Syndrome/mortality , Posterior Leukoencephalopathy Syndrome/therapy , Retrospective Studies , Survival Analysis , Tissue Donors , Tomography, X-Ray Computed , Transplantation, Homologous
10.
Oncologist ; 20(7): 806-11, 2015 Jul.
Article En | MEDLINE | ID: mdl-26032137

BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is characterized by neurologic symptoms with typical lesions on neuroimaging and may be associated with chemotherapy and immunosuppressive agents used in patients with cancer. We described the spectrum of PRES at a major cancer center. METHODS: We reviewed charts of adults with PRES between 2005 and 2011 at Memorial Sloan Kettering Cancer Center for clinical information and outcome. RESULTS: We identified 21 women (68%) and 10 men (median cohort age: 58 years). Solid tumors (n = 22, 71%) were more common than hematologic (n = 8) or primary brain malignancies (n = 1). Prior brain irradiation (16%) and central nervous system metastases (10%) were uncommon. There were 55% who received chemotherapy or targeted therapy within the month preceding PRES, including 6 patients who received bevacizumab; PRES followed allogeneic stem cell transplantation in 5 (16%). Presenting symptoms included confusion (71%), seizure (58%), and headache (48%). Maximum systolic and diastolic blood pressures were similar among patients grouped by cancer type, chemotherapy or bevacizumab use, and atypical imaging. Moreover, 37% of patients with both magnetic resonance imaging (MRI) and computed tomography (CT) had normal CT concurrent with PRES on MRI, and 84% returned to neurologic baseline at a median of 7.5 days (range: 1-167 days) from onset. Successful anticonvulsant taper was achieved in 51%. Chemotherapy rechallenge was attempted in 41% without recurrent PRES. Autopsy revealed nonspecific changes isolated to radiographically affected areas in one of two patients. CONCLUSION: Recent chemotherapy, particularly bevacizumab, is common in cancer patients with PRES. Clinical and radiographic presentations may vary; MRI appears more sensitive than CT. Anticonvulsant taper and chemotherapy rechallenge is often possible. IMPLICATIONS FOR PRACTICE: Posterior reversible encephalopathy syndrome is characterized by neurologic symptoms with typical lesions on neuroimaging and may be associated with chemotherapy and immunosuppressive agents used in patients with cancer. Clinical and radiographic presentations are protean, and magnetic resonance imaging is more sensitive than computed tomography. Recovery is common, and many patients can be successfully rechallenged with the apparently offending chemotherapy agent or regimen.


Neoplasms/complications , Posterior Leukoencephalopathy Syndrome/etiology , Posterior Leukoencephalopathy Syndrome/therapy , Adult , Aged , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Blood Pressure , Electroencephalography , Female , Headache/chemically induced , Headache/etiology , Humans , Kaplan-Meier Estimate , Magnetic Resonance Imaging , Male , Middle Aged , Neoplasms/mortality , Neoplasms/physiopathology , Posterior Leukoencephalopathy Syndrome/chemically induced , Posterior Leukoencephalopathy Syndrome/mortality , Seizures/drug therapy , Tomography, X-Ray Computed , Young Adult
11.
Ren Fail ; 37(6): 974-9, 2015 Jul.
Article En | MEDLINE | ID: mdl-25974726

AIM: The clinical course and outcome of children with thrombotic thrombocytopenic purpura and posterior reversible encephalopathy has not been observed and studied till date. The aim of the present study was to know the clinical course and outcome of children with thrombotic thrombocytopenic purpura and posterior reversible encephalopathy. Results from our observation invite potential insight for further research on this subject. METHODS: From January 2005 to February 2013, seven children diagnosed with thrombotic thrombocytopenic purpura and posterior reversible encephalopathy syndromes were admitted in a tertiary care hospital in Srinagar, Kashmir. The demographic parameters, clinical characteristics and laboratory data were noted. The outcome was defined in the form of complete recovery or death. Thrombotic thrombocytopenic purpura was diagnosed on clinical grounds, laboratory parameters and renal biopsy. The diagnosis was established after an expert opinion from a hematologist. Posterior reversible encephalopathy syndrome was defined on neuroimaging. RESULTS: The common clinical parameters which were shared by all the patients were hypertension and altered sensorium. Four (57.1%) patients showed clinical deterioration and died within one week of admission even after intensive management. Three (42.8%) patients improved clinically and recovered fully and were discharged in stable clinical condition. Repeat imaging on discharge was normal. CONCLUSION: This series of seven pediatric patients is the first series on this subject. The presence of posterior reversible encephalopathy syndrome in pediatric patients with thrombotic thrombocytopenic purpura complicates the clinical course and worsens the prognosis.


Posterior Leukoencephalopathy Syndrome/complications , Posterior Leukoencephalopathy Syndrome/diagnosis , Purpura, Thrombotic Thrombocytopenic/complications , Purpura, Thrombotic Thrombocytopenic/diagnosis , Adolescent , Child , Child, Preschool , Combined Modality Therapy , Critical Care/methods , Disease Progression , Female , Follow-Up Studies , Hematologic Tests , Humans , Immunohistochemistry , Intensive Care Units, Pediatric , Magnetic Resonance Imaging/methods , Male , Posterior Leukoencephalopathy Syndrome/mortality , Posterior Leukoencephalopathy Syndrome/therapy , Purpura, Thrombotic Thrombocytopenic/mortality , Purpura, Thrombotic Thrombocytopenic/therapy , Retrospective Studies , Risk Assessment , Sampling Studies , Severity of Illness Index , Survival Rate , Tomography, X-Ray Computed/methods , Treatment Outcome
13.
J Neurol Sci ; 347(1-2): 59-65, 2014 Dec 15.
Article En | MEDLINE | ID: mdl-25271189

PURPOSE: To evaluate the relative impact of clinical data, imaging findings, and CSF laboratory values on clinical outcome in patients with posterior reversible encephalopathy syndrome (PRES). METHODS: 47 patients with PRES who underwent a lumbar puncture were retrospectively evaluated. Fatal outcome was defined as death directly ascribed to PRES toxicity. Univariate and multivariate analyses were used to evaluate the association between fatal outcome and clinical factors (demographics, PRES etiology), imaging findings (signal abnormality severity, atypical distribution, restricted diffusion, hemorrhage, enhancement, angiographic abnormalities), and lumbar puncture results (appearance, cell count, glucose, protein, culture results). RESULTS: Nine patients (19.1%) had a fatal outcome. Odds of a fatal outcome increased nearly 5-fold with hemorrhage on imaging (Adjusted Odds Ratio (AOR) 4.8, 95% CI 3.8-6.0, p=0.03) and nearly doubled with low CSF glucose (AOR 1.9, 95% CI 1.5-2.5, p=0.02). Hypertensive encephalopathy as an etiology was associated with a fatal outcome (AOR 1.6, 95% CI 1.3-2.9, p=0.02), while toxemia of pregnancy was protective, with a 75% decreased risk (AOR 0.25, 95% CI 0.15-0.43, p=0.02). CONCLUSION: Clinical, imaging, and CSF laboratory findings all influence outcome in PRES, with a low CSF glucose, hypertensive encephalopathy, and imaging findings of hemorrhage associated with increased risk of fatal outcome.


Cerebral Hemorrhage/mortality , Glucose/cerebrospinal fluid , Hypertension/complications , Magnetic Resonance Imaging , Posterior Leukoencephalopathy Syndrome/complications , Posterior Leukoencephalopathy Syndrome/mortality , Adolescent , Adult , Aged , Aged, 80 and over , Cerebral Hemorrhage/etiology , Cerebral Hemorrhage/pathology , Female , Humans , Hypertension/mortality , Male , Middle Aged , Posterior Leukoencephalopathy Syndrome/etiology , Posterior Leukoencephalopathy Syndrome/pathology , Risk Assessment , Risk Factors , Young Adult
14.
Clin Neurol Neurosurg ; 125: 52-7, 2014 Oct.
Article En | MEDLINE | ID: mdl-25086431

INTRODUCTION: Recognition of severe forms of posterior reversible encephalopathy syndrome (PRES) has improved. Management of these patients remains challenging, particularly in patients with the combination of edema and hemorrhage. METHODS: A prospective inpatient neuro-intensive care database was queried for patients with PRES. Malignant PRES was diagnosed by clinical assessments (GCS less than 8 and clinical decline despite standard medical management for elevated intracranial pressure) and radiographic criteria (edema with associated mass effect; brain hemorrhage exerting mass effect; effacement of basal cisterns, transtentorial, tonsillar, or uncal herniation). Malignant PRES was defined as: radiology studies consistent with PRES; GCS less than 8; and clinical decline despite standard elevated intracranial pressure management. RESULTS: Five cases were identified over a 4 year interval. The following contributing conditions were also present: chemotherapy (1), systemic lupus erythematosis (2), pregnancy (1), and methamphetamines (1). Neurocritical care interventions included: hyperosmolar therapy (5), anticonvulsants (5), management of coagulopathy (5), and ventilatory support (5). Neurosurgical interventions included: craniectomy (5), hematoma evacuation (3), and external ventricular drain (4). Brain biopsy was performed in 5 patients and was negative for vasculitis, demyelinating disease, tumor, or infection. Cyclophosphamide was administered to the two patients with SLE. With long-term follow up, all patients achieved good functional outcomes (modified Rankin score 1-2). CONCLUSION: In contrast to historical reports of high mortality rates (16-29%) for severe and hemorrhagic PRES variants, we had no fatalities and observed favorable functional outcomes with intracranial pressure monitoring and craniectomy for malignant PRES cases who fail medical ICP management.


Posterior Leukoencephalopathy Syndrome/diagnosis , Posterior Leukoencephalopathy Syndrome/therapy , Adult , Cerebral Hemorrhage/complications , Cerebral Hemorrhage/diagnosis , Cyclophosphamide/therapeutic use , Female , Humans , Middle Aged , Posterior Leukoencephalopathy Syndrome/mortality , Pregnancy , Prospective Studies , Treatment Outcome
15.
Acta Neurol Scand ; 130(1): 34-9, 2014 Jul.
Article En | MEDLINE | ID: mdl-24329761

BACKGROUND: Posterior reversible encephalopathy syndrome is a serious and increasingly recognized disorder, but data from observational studies on outcome and mortality in posterior reversible encephalopathy syndrome (PRES) are scarce. We aimed to determine the frequency and associations of in-hospital death and discharge status in a large cohort of patients with PRES. METHOD: We retrospectively reviewed the radiological report databases of our university hospitals between January 1999 and March 2011 for patients with PRES. Patients fulfilling the criteria for PRES after detailed investigation of clinical charts and imaging studies were included. Clinical charts, paraclinical and brain imaging data at onset as well as available data on in-hospital mortality and discharge status were analyzed. RESULTS: A total of 103 patients were included. Five (4.8%) patients died during hospital stay, 27 (26.2%) remained hospitalized after discharge. In univariate analyses, significant differences were found between patients discharged home from hospital and patients referred to rehabilitation or who died in hospital for the following variables: severe edema (P = 0.013), etiology of PRES (P = 0.001), altered mental state at onset (P = 0.003), altered coagulation (P = 0.004), and length of hospital stay >30 days (P < 0.001). CONCLUSION: Features of a severe course of PRES such as severe edema and altered mental state are significantly more frequent in patients who were referred to inpatient rehabilitation or died in hospital. Prospective studies are warranted to establish factors that are associated with unfavorable outcome in PRES.


Hospital Mortality , Patient Discharge/statistics & numerical data , Posterior Leukoencephalopathy Syndrome/mortality , Posterior Leukoencephalopathy Syndrome/pathology , Adult , Aged , Female , Humans , Male , Middle Aged , Posterior Leukoencephalopathy Syndrome/complications , Retrospective Studies , Young Adult
16.
Am J Hematol ; 88(4): 301-5, 2013 Apr.
Article En | MEDLINE | ID: mdl-23460378

Tacrolimus-associated posterior reversible encephalopathy syndrome (PRES) is a potential complication of allogeneic stem cell transplant (SCT). Due to the paucity of information on the management of PRES in SCT patients receiving tacrolimus, more information is needed on trends associated with the incidence of PRES and to characterize its management. A retrospective review was conducted of patients receiving tacrolimus for prevention of graft versus host disease (GVHD) after allogeneic SCT who developed PRES from September 2008 to July 2011. Nineteen patients were identified. Altered mental status, seizures, and visual abnormalities were experienced by 78.9%, 52.6%, and 31.5% of the patients, respectively, at time of PRES onset. Compared with baseline, patients with PRES were likely to have an increase in mean arterial pressure (P < 0.0001) and serum creatinine. Elevated tacrolimus levels and hypomagnesemia were not observed with PRES onset. Tacrolimus was managed in three general strategy groups: not held, held then continued, and switched to another agent. Survival was defined as survival to discharge from PRES hospitalization. When tacrolimus was not held, held then continued, or switched to another agent, 40% (2 of 5), 40% (4/10), and 50% (2/4) survived to discharge, respectively. PRES was associated with high blood pressure and adequate blood pressure control should be part of its management. No management strategy pertaining to tacrolimus usage appeared more beneficial over another.


Hematopoietic Stem Cell Transplantation , Hypertension/drug therapy , Immunosuppressive Agents/adverse effects , Posterior Leukoencephalopathy Syndrome/drug therapy , Tacrolimus/adverse effects , Adult , Aged , Blood Pressure , Disease Management , Female , Graft vs Host Disease/immunology , Graft vs Host Disease/pathology , Graft vs Host Disease/prevention & control , Humans , Hypertension/etiology , Hypertension/immunology , Hypertension/mortality , Leukemia/therapy , Male , Middle Aged , Posterior Leukoencephalopathy Syndrome/etiology , Posterior Leukoencephalopathy Syndrome/immunology , Posterior Leukoencephalopathy Syndrome/mortality , Retrospective Studies , Survival Analysis , Transplantation, Homologous
17.
PLoS One ; 7(9): e44534, 2012.
Article En | MEDLINE | ID: mdl-23024751

OBJECTIVE: Few outcome data are available about posterior reversible encephalopathy syndrome (PRES). We studied 90-day functional outcomes and their determinants in patients with severe PRES. DESIGN: 70 patients with severe PRES admitted to 24 ICUs in 2001-2010 were included in a retrospective cohort study. The main outcome measure was a Glasgow Outcome Scale (GOS) of 5 (good recovery) on day 90. MAIN RESULTS: Consciousness impairment was the most common clinical sign, occurring in 66 (94%) patients. Clinical seizures occurred in 57 (81%) patients. Median mean arterial pressure was 122 (105-143) mmHg on scene. Cerebral imaging abnormalities were bilateral (93%) and predominated in the parietal (93%) and occipital (86%) white matter. Median number of brain areas involved was 4 (3-5). Imaging abnormalities resolved in 43 (88%) patients. Ischaemic and/or haemorrhagic complications occurred in 7 (14%) patients. The most common causes were drug toxicity (44%) and hypertensive encephalopathy (41%). On day 90, 11 (16%) patients had died, 26 (37%) had marked functional impairments (GOS, 2 to 4), and 33 (56%) had a good recovery (GOS, 5). Factors independently associated with GOS<5 were highest glycaemia on day 1 (OR, 1.22; 95%CI, 1.02-1.45, p = 0.03) and time to causative-factor control (OR, 3.3; 95%CI, 1.04-10.46, p = 0.04), whereas GOS = 5 was associated with toxaemia of pregnancy (preeclampsia/eclampsia) (OR, 0.06; 95%CI, 0.01-0.38, p = 0.003). CONCLUSIONS: By day 90 after admission for severe PRES, 44% of survivors had severe functional impairments. Highest glycaemia on day 1 and time to causative-factor control were strong early predictors of outcomes, suggesting areas for improvement.


Posterior Leukoencephalopathy Syndrome/diagnosis , Posterior Leukoencephalopathy Syndrome/rehabilitation , Adult , Brain/pathology , Cohort Studies , Female , Humans , Lod Score , Magnetic Resonance Imaging , Male , Middle Aged , Neuroimaging , Posterior Leukoencephalopathy Syndrome/mortality , Posterior Leukoencephalopathy Syndrome/therapy , Pre-Eclampsia , Pregnancy , Prognosis , Retrospective Studies , Severity of Illness Index , Tomography, Emission-Computed , Treatment Outcome
18.
Rev Neurol (Paris) ; 167(11): 812-9, 2011 Nov.
Article Fr | MEDLINE | ID: mdl-21737111

INTRODUCTION: The occurrence of posterior reversible encephalopathy in eclampsia is a rare but known event. We propose to describe the clinical and radiological features and the outcome. METHODS: A retrospective study was conducted from January 2005 to April 2010 including all cases of posterior reversible encephalopathy syndrome (PRES) occurring on eclampsia in patients hospitalized in the obstetrical intensive care unit, University Hospital of Casablanca. RESULTS: Thirteen cases of PRES on eclampsia were collected, the average age was 29 years (18-42). Systolic pressure and diastolic blood pressure at admission were higher than 150 mmHg and 100 respectively in 10 cases. The signs found were: a regressive blindness in five patients and focal signs in four. The complications were thrombocytopenia in 10 patients, abnormal liver function in eight, Hellp syndrome in nine, and acute renal failure in two. The brain regions most commonly affected were the parietal and occipital areas (13 patients), followed by temporal regions, frontal, and basal ganglia (eight patients each). Five patients required assisted ventilation (AV) over 24 hours. Death complicated the outcome in four of our patients, but no deaths were directly attributable to PRES itself, and all four patients had Hellp syndrome and required AV greater than 48 hours. In the other patients, total regression of neurological signs was noted. CONCLUSION: This study emphasizes the severity of the Posterior 'reversible' encephalopathy syndrome on eclampsia.


Eclampsia/etiology , Posterior Leukoencephalopathy Syndrome/complications , Adolescent , Adult , Cohort Studies , Disease Progression , Eclampsia/diagnostic imaging , Eclampsia/epidemiology , Eclampsia/mortality , Female , Humans , Magnetic Resonance Imaging , Morocco , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Posterior Leukoencephalopathy Syndrome/epidemiology , Posterior Leukoencephalopathy Syndrome/mortality , Pregnancy , Radiography , Retrospective Studies , Severity of Illness Index , Young Adult
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