RESUMEN
Background and purpose:
Posterior reversible encephalopathy syndrome (PRES) is characterized by vasogenic edema, usually reversible, with the prominent involvement of the parietal and occipital lobes. The exact etiopathogenesis leading to PRES is unknown. Because signs of eclampsia and preeclampsia in neuroimaging often overlap and manifest as PRES, we aimed to evaluate whether demographic, clinical, and laboratory parameters predict PRES in patients with preeclampsia or eclampsia.
213 pre-eclampsia or eclampsia patients with cranial imaging were retrospectively examined. We recorded the patients’ demographic information, systolic blood pressure (SBP), diastolic blood pressure (DBP), mean arterial pressure (MAP), hemogram, biochemical indicators, clinical symptoms, and imaging features.
. Results:Of all patients, 69% (n = 147) had preeclampsia while 31% (n = 66) had eclampsia, and 24.4% (n = 53) were diagnosed with PRES. The mean age of patients who developed PRES was 25.81 ± 6.07 years and thus significantly less than that of patients who did not develop PRES (p = .000). Patients with PRES had significantly higher mean SBP (p = .015), DBP (p = .009), and MAP (p = .003) than patients without PRES, along with significantly higher aspartate aminotransferase (ASAT; p = .001), alanine aminotransferase (ALAT; p = .001) blood urea nitrogen (BUN; p = .001), white blood cell (WBC; p = .003), neutrophil (p = .001), and hemoglobin (Hb; p = .027) levels, but significantly lower albumin (p = .000) levels.
. Conclusion:Age, high blood pressure, and BUN, neutrophil, and WBC levels were predictors of the development of PRES in patients with preeclampsia and eclampsia. Early neuroimaging considering those predictors should be performed to diagnose PRES in patients with preeclampsia and eclampsia.
.Asunto(s)
Eclampsia , Síndrome de Leucoencefalopatía Posterior , Preeclampsia , Humanos , Femenino , Embarazo , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Síndrome de Leucoencefalopatía Posterior/etiología , Síndrome de Leucoencefalopatía Posterior/fisiopatología , Preeclampsia/sangre , Preeclampsia/fisiopatología , Eclampsia/diagnóstico por imagen , Estudios Retrospectivos , Adulto , Factores de Riesgo , Presión SanguíneaRESUMEN
Eclampsia spectrum disorders are a set of serious complications of pregnancy that commonly present after 20 weeks of gestation. There is an association between molar pregnancy, a gestational trophoblastic disease resulting from abnormal fertilisation and gametogenesis, and eclampsia spectrum disorders which can result in manifestation of pre-eclamptic symptomatology earlier than 20 weeks of gestation. We report a case of a gravida 1 para 0 in her mid 20s at 16-weeks gestation presenting with partial hydatidiform mole who developed eclampsia, haemolysis, elevated liver enzymes and low platelets syndrome and posterior reversible encephalopathy syndrome. Ultrasound findings were consistent with molar pregnancy and pathology confirmed partial molar pregnancy with triploid 69, XYY karyotype. This case highlights the early onset potential of eclampsia spectrum disorders in molar pregnancies while suggesting screening such patients for hypertensive disorders.
Asunto(s)
Eclampsia , Síndrome HELLP , Mola Hidatiforme , Neoplasias Uterinas , Humanos , Femenino , Mola Hidatiforme/diagnóstico por imagen , Mola Hidatiforme/diagnóstico , Embarazo , Síndrome HELLP/diagnóstico , Eclampsia/diagnóstico , Adulto , Neoplasias Uterinas/diagnóstico por imagen , Neoplasias Uterinas/diagnóstico , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Síndrome de Leucoencefalopatía Posterior/diagnósticoRESUMEN
BACKGROUND: Guillain-Barré syndrome (GBS) is an autoimmune disorder characterized by demyelination of peripheral nerves. GBS-associated posterior reversible encephalopathy syndrome (PRES) is a rare and potentially life-threatening complication in the pediatric population. We aimed to report and analyze the clinical features, management, and outcomes of three cases of GBS-associated PRES in our setting in the light of the existing literature. METHODS: Medical records of 75 pediatric patients with GBS were reviewed for autonomic changes and GBS-associated PRES. Thirty-one developed dysautonomia while three were identified to have PRES. Clinical, radiological, laboratory, and treatment data were collected and analyzed. RESULTS: All three patients were male and presented with symptoms of acute flaccid paralysis and respiratory distress requiring mechanical ventilation. All three patients experienced various complications, including hypertension, seizures, and hyponatremia, and were subsequently diagnosed with PRES. Multimodal intensive care resulted in patient improvement and discharge in an ambulatory state after an average of 104 days of care. CONCLUSIONS: GBS-associated PRES is a rare and potentially life-threatening complication that can occur in pediatric patients with GBS. Our findings suggest that early recognition, prompt intervention, and multimodal intensive care can improve patient outcomes. Further studies are needed to determine optimal treatment strategies for GBS-associated PRES.
Asunto(s)
Síndrome de Guillain-Barré , Síndrome de Leucoencefalopatía Posterior , Humanos , Síndrome de Guillain-Barré/terapia , Síndrome de Guillain-Barré/complicaciones , Síndrome de Guillain-Barré/fisiopatología , Masculino , Síndrome de Leucoencefalopatía Posterior/etiología , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Síndrome de Leucoencefalopatía Posterior/terapia , Síndrome de Leucoencefalopatía Posterior/fisiopatología , Niño , Adolescente , PreescolarRESUMEN
Posterior reversible encephalopathy syndrome (PRES) is an acute neurological condition associated with different etiologies, including antibiotic therapy. To date, most data regarding antibiotic-related PRES are limited to case reports and small case series. Here, we report a novel case description and provide a systematic review of the clinico-radiological characteristics and prognosis of available cases of PRES associated with antibiotic therapy. We performed a systematic literature search in PubMed and Scopus from inception to 10 January 2024, following PRISMA guidelines and a predefined protocol. The database search yielded 12 subjects (including our case). We described the case of a 55-year-old female patient with PRES occurring one day after administration of metronidazole and showing elevated serum neurofilament light chain protein levels and favorable outcome. In our systematic review, antibiotic-associated PRES was more frequent in female patients (83.3%). Metronidazole and fluoroquinolones were the most reported antibiotics (33.3% each). Clinical and radiological features were comparable to those of PRES due to other causes. Regarding the prognosis, about one third of the cases were admitted to the intensive care unit, but almost all subjects (90.0%) had a complete or almost complete clinical and radiological recovery after prompt cessation of the causative drug. Antibiotic-associated PRES appears to share most of the characteristics of classic PRES. Given the overall good prognosis of the disease, it is important to promptly diagnose antibiotic-associated PRES and discontinue the causative drug.
Asunto(s)
Antibacterianos , Síndrome de Leucoencefalopatía Posterior , Humanos , Síndrome de Leucoencefalopatía Posterior/inducido químicamente , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Femenino , Persona de Mediana Edad , Antibacterianos/efectos adversos , Antibacterianos/uso terapéutico , Metronidazol/efectos adversosAsunto(s)
Glomerulonefritis , Infecciones Estreptocócicas , Humanos , Infecciones Estreptocócicas/complicaciones , Glomerulonefritis/complicaciones , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Síndrome de Leucoencefalopatía Posterior/etiología , Niño , Masculino , Femenino , Vasoespasmo Intracraneal/diagnóstico por imagen , Vasoespasmo Intracraneal/etiología , Vasoespasmo Intracraneal/complicaciones , Imagen por Resonancia MagnéticaRESUMEN
BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is characterized by cerebral blood flow dysregulation and the blood-brain barrier (BBB) disruption. While renal insufficiency has been considered a factor in BBB fragility, the relationship between renal insufficiency and the PRES lesions volume remains unclear. METHODS: This observational study was performed retrospectively. PRES patients were categorized into two groups with renal insufficiency, defined as an estimated glomerular filtration rate (eGFR) of less than 60 mL/min/1.73m2 on the day of symptom occurrence. Lesion volume was measured using fluid-attenuated inversion recovery (FLAIR) imaging, and the brain was divided into nine regions. The volume of the parietal-occipital-temporal lobe was considered typical, while the other six regions were labeled as atypical. RESULTS: The study included 200 patients, of whom 94 (47%) had renal insufficiency. Patients with renal insufficiency had a larger lesion volume (144.7 ± 125.2 cc) compared to those without renal insufficiency (110.5 ± 93.2 cc; p = 0.032); particularly in the atypical lesions volume (49.2 ± 65.0 vs. 29.2 ± 44.3 cc; p = 0.013). However, there was no difference in the reversibility of the lesions (35.2 ± 67.5 vs. 18.8 ± 33.4 cc; p = 0.129). Multiple regression analysis revealed that decreases in eGFR (ß = -0.34, 95% CI -0.62-0.05, p = 0.020) were positively associated with total lesion volume. CONCLUSION: Our findings suggest that PRES patients with renal insufficiency experience more severe lesion volumes, likely due to the atypical brain regions involvement. The lesions involving atypical regions may have a similar pathophysiology to typical lesions, as the PRES lesions reversibility was found to be similar between individuals with and without renal insufficiency.
Asunto(s)
Imagen por Resonancia Magnética , Síndrome de Leucoencefalopatía Posterior , Insuficiencia Renal , Humanos , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Síndrome de Leucoencefalopatía Posterior/patología , Síndrome de Leucoencefalopatía Posterior/fisiopatología , Síndrome de Leucoencefalopatía Posterior/complicaciones , Femenino , Masculino , Insuficiencia Renal/patología , Insuficiencia Renal/fisiopatología , Estudios Retrospectivos , Persona de Mediana Edad , Adulto , Encéfalo/patología , Encéfalo/diagnóstico por imagen , Anciano , Tasa de Filtración Glomerular/fisiologíaRESUMEN
OBJECTIVES: Posterior reversible encephalopathy syndrome (PRES) is a clinic radiological disorder characterized by headache, epileptic seizure, encephalopathy, visual impairment, and focal neurological deficits. Gestational hypertension, which is a significant risk factor for PRES, may cause significant morbidity and mortality among pregnant women. DESIGN: Twenty-four patients with PRES caused by eclampsia who were admitted to our hospital in the last 5 years were included in this study. PARTICIPANTS/MATERIALS, SETTING, METHODS: Blood pressure at admission, the number of regions with vasogenic edema in the brain, and recurrent seizures were noted. Patients were divided into three groups: mild, moderate, and severe. RESULTS: Using Kruskal-Wallis and Pearson χ2 tests, there was no statistical significance between the groups in terms of cranial involvement (p = 0.471). However, binary logistic regression analysis showed that seizure recurrence increased in correlation with blood pressure (p = 0.04). LIMITATIONS: PRES is a rare syndrome associated with several etiologies. In our study, only patients with PRES due to eclampsia were included. Therefore, the number of included patients was limited (24 participants). CONCLUSION: PRES may occur in eclamptic patients with mild, moderate, or severe blood pressure values. Evaluation by magnetic resonance imaging is needed to confirm the diagnosis. Early and rapid treatment is essential for reducing morbidity and mortality among pregnant women.
Asunto(s)
Eclampsia , Imagen por Resonancia Magnética , Síndrome de Leucoencefalopatía Posterior , Recurrencia , Convulsiones , Humanos , Femenino , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Síndrome de Leucoencefalopatía Posterior/fisiopatología , Síndrome de Leucoencefalopatía Posterior/etiología , Síndrome de Leucoencefalopatía Posterior/complicaciones , Embarazo , Eclampsia/fisiopatología , Adulto , Convulsiones/etiología , Convulsiones/fisiopatología , Presión Sanguínea/fisiología , Adulto Joven , Factores de RiesgoAsunto(s)
Eclampsia , Síndrome de Leucoencefalopatía Posterior , Humanos , Femenino , Embarazo , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Síndrome de Leucoencefalopatía Posterior/diagnóstico , Síndrome de Leucoencefalopatía Posterior/etiología , Eclampsia/diagnóstico , Adulto , Imagen por Resonancia MagnéticaRESUMEN
RATIONALE: Hodgkin lymphoma, a lymphatic system cancer, is treated by chemotherapy, radiation therapy, and hematopoietic stem cell transplantation. Posterior reversible encephalopathy syndrome (PRES) is a rare neurotoxic effect associated with several drugs and systemic conditions. This case study emphasizes the potential risks of intensive chemotherapy regimens and postulates the impact of the circle of Willis variants on the heterogeneity of hemispheric lesions in PRES. PATIENT CONCERNS: A 42-year-old woman diagnosed with stage IIA nodular sclerosing Hodgkin lymphoma and chronic thrombocytopenia presented after 6 years of initial diagnosis and 4 years post-haploidentical transplant. She underwent planned chemotherapy with ifosfamide, carboplatin, and etoposide. DIAGNOSES: She developed an alteration in her mental status. A computerized tomography scan and angiogram of the head and neck revealed findings consistent with PRES and a left fetal-type posterior cerebral artery with an aplastic A1 segment of the left anterior cerebral artery. One hour later she was found comatose with clinical sequelae of an uncal herniation. INTERVENTIONS: Subsequent events led to emergent intubation, and administration of 23.4% hypertonic saline. A repeat computerized tomography scan showed a right intraparenchymal hemorrhage with fluid-fluid levels measuring up to 4.7 cm, bilateral subarachnoid hemorrhage, right uncal herniation, and 15 mm of leftward midline shift. She emergently underwent a right decompressive hemi-craniectomy. OUTCOMES: An magnetic resonance imaging of the brain demonstrated bilateral cytotoxic edema involving the parieto-occipital lobes. Despite interventions, the patient's neurological condition deteriorated, leading to a declaration of brain death on the 8th day. LESSONS: This case underscores the importance of recognizing the severe neurological complications, including PRES, associated with chemotherapeutic treatments in Hodgkin lymphoma. PRES may also be exacerbated by coagulopathies such as thrombocytopenia in this case. The circle of Willis variants may influence cerebral blood flow, autoregulation, and other factors of hemodynamics, leading to increased susceptibility to both radiographic lesion burden and the worst clinical outcomes.
Asunto(s)
Encefalopatías , Enfermedad de Hodgkin , Síndrome de Leucoencefalopatía Posterior , Trombocitopenia , Humanos , Femenino , Adulto , Síndrome de Leucoencefalopatía Posterior/inducido químicamente , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Enfermedad de Hodgkin/complicaciones , Círculo Arterial Cerebral , Encefalopatías/complicaciones , Hemorragia/complicaciones , Trombocitopenia/complicaciones , Circulación Cerebrovascular , HomeostasisAsunto(s)
Síndrome de Leucoencefalopatía Posterior , Humanos , Síndrome de Leucoencefalopatía Posterior/inducido químicamente , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Femenino , Esclerosis Múltiple/tratamiento farmacológico , Esclerosis Múltiple/complicaciones , Esclerosis Múltiple/diagnóstico por imagen , Interferón beta-1a/efectos adversos , Adulto , Imagen por Resonancia Magnética , Factores Inmunológicos/efectos adversosRESUMEN
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.
Asunto(s)
Síndrome de Leucoencefalopatía Posterior , Adulto , Femenino , Humanos , Embarazo , Encéfalo/diagnóstico por imagen , Encéfalo/irrigación sanguínea , Relevancia Clínica , Japón/epidemiología , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Vasoconstricción/fisiología , Vasoespasmo Intracraneal/diagnóstico por imagen , Informes de Casos como AsuntoRESUMEN
Posterior reversible encephalopathy syndrome (PRES) is a pathology seen not only in precelampsia with severe symptoms and eclampsia but in a varicty of diseases/ conditions. With the availability of neuroimaging, it is possible to know the exact underlying Central nervous system (CNS) pathology in preeclampsia with severe symptoms and eclampsia and thus therapy can be targeted. Preeclampsia with severe symptoms and eclampsia remains to be an important cause of maternal morbidity and mortality in both the developing and developed world. The objective of this study was to evaluate the association of Posterior reversible encephalopathy syndrome (PRES) by MRI (Magnetic resonance imaging) with preeclampsia with severe symptoms and eclampsia in south east part of Bangladesh. This cross-sectional observational study was performed among women suffering from preeclampsia with severe symptoms and eclampsia who attended at Obstetrics & Gynaecology department of Chittagong Medical College Hospital (CMCH), Bangladesh from January 2021 to June 2021. According to inclusion/exclusion criteria 50 samples were taken by convenient sampling for this study. A detail history was taken and complete general physical and gynecological examination was performed. Required data was collected through preset questionnaire. Neuroimaging reports were reviewed by both neurologist and radiologist. Data was analyzed by using windows based computer software device, SPSS 25.0. Results obtained from this study will be used to make a statement regarding aggressive management for cerebral vasospasm in severe preeclampsia and eclamptia related PRES. PRES has been reported to be reversible but late recognition or incorrect treatment can cause irreversible brain damage. Institution of early treatment leads to resolution of symptoms without any neurologic deficit and thus reduces maternal morbidity and mortality. PRES is a cliniconeuroradiologic entity. This study can aware doctors regarding prompt diagnosis of PRES in peripartum period among patient suffering from preeclampsia with severe symptoms and eclampsia by imaging aside clinical findings. A conclusive decision can be made to improve the outcome in this potentially life threatening but reversible condition.
Asunto(s)
Eclampsia , Síndrome de Leucoencefalopatía Posterior , Preeclampsia , Embarazo , Femenino , Humanos , Eclampsia/terapia , Síndrome de Leucoencefalopatía Posterior/diagnóstico , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Bangladesh/epidemiología , Estudios TransversalesRESUMEN
BACKGROUND: To investigate the relationship between immunosuppressive treatments and posterior reversible encephalopathy syndrome (PRES) in transplant patients. METHODS: We presented a retrospective study of 4 cases of PRES in transplant patients. Patient records were reviewed to identify potential risk factors, clinical presentations, radiological findings, and immunosuppressive treatments used. RESULTS: Our analysis revealed a potential association between immunosuppressive treatments and the development of PRES in transplant patients. Specifically, we found that adjusting or switching immunosuppressive treatments can improve outcomes and prevent the recurrence of PRES. CONCLUSION: Our findings highlight the importance of recognizing PRES as a potential complication of immunosuppressive treatments in transplant patients. Early detection and management, including a review of immunosuppressive treatments, may improve patient outcomes and prevent further complications.
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Inhibidores de la Calcineurina , Síndrome de Leucoencefalopatía Posterior , Humanos , Inhibidores de la Calcineurina/efectos adversos , Inmunosupresores/efectos adversos , Síndrome de Leucoencefalopatía Posterior/inducido químicamente , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Estudios Retrospectivos , SirolimusRESUMEN
INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is a rare and complex disorder with variable clinical presentation and a typical magnetic resonance imaging (MRI) pattern of vasogenic edema with typical and atypical locations. It is often triggered by other diseases and drugs and the most prototypical association is with persistently elevated arterial pressure values. Among the potential cerebrovascular complications, intracranial bleeding has been described, but ischemic stroke is uncommonly reported. METHODS: We are presenting a case of a male patient with prolonged and sustained arterial hypertension acutely presenting with lacunar ischemic stroke involving the right corona radiata and composite MRI findings with the association of chronic small vessel disease (SVD) markers, acute symptomatic lacunar stroke, and atypical, central variant, posterior fossa dominant PRES. In the MRI follow-up, the white matter hyperintensities in T2-fluid attenuated inversion recovery (FLAIR sequences) due to PRES. DISCUSSION: The pathophysiology of PRES is not yet fully known, but the association with markedly increased values of arterial pressure is typical. In this context, ischemic stroke has not been considered in the clinical and neuroradiological manifestations of PRES and it has been only occasionally reported in the literature. In this case, the main hypothesis is that sustained hypertension may have triggered both manifestations, PRES, and ischemic stroke and the last one allowed to diagnose the first one. CONCLUSIONS: Atypical variants of PRES are not so rare and it may also occur in typical triggering situations. The association with ischemic stroke is even rarer and it may add some clues to the pathomechanisms of PRES.
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Hipertensión , Accidente Cerebrovascular Isquémico , Síndrome de Leucoencefalopatía Posterior , Accidente Vascular Cerebral Lacunar , Sustancia Blanca , Humanos , Masculino , Síndrome de Leucoencefalopatía Posterior/complicaciones , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Accidente Cerebrovascular Isquémico/complicaciones , Hipertensión/complicaciones , Imagen por Resonancia Magnética/métodos , Accidente Vascular Cerebral Lacunar/complicaciones , Accidente Vascular Cerebral Lacunar/diagnóstico por imagenAsunto(s)
Porfiria Intermitente Aguda , Síndrome de Leucoencefalopatía Posterior , Femenino , Humanos , Porfiria Intermitente Aguda/complicaciones , Porfiria Intermitente Aguda/diagnóstico , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Síndrome de Leucoencefalopatía Posterior/etiología , Dolor Abdominal/etiología , Imagen por Resonancia MagnéticaRESUMEN
PURPOSE: Identify MR features predictive of poor outcomes in non-oncologic pediatric PRES. METHOD: A six-year search of all non-oncologic pediatric patients with clinical and MR features of PRES was performed. Modified Rankin scores were used to classify clinical outcomes into good versus poor, then clinical and MR features were compared among groups. Univariate and multivariate analysis was performed to identify MR predictors of poor outcomes for various imaging features, and p-values < 0.05 were considered statistically significant. RESULTS: One hundred and forty-one patients (mean age 10.1 ± 3.0 years, male to female ratio 1:1.1) were included. Clinically, nephrotic syndrome (p = 0.03), focal deficits (p = 0.04), longer hospitalization (p < 0.001), and mechanical ventilation (p < 0.001) were significantly associated with poor outcomes. Univariate analysis revealed that deep grey matter nuclei (OR = 5.29, 95 % CI: 1.6-18.0) and cerebellar edema patterns (OR = 3.49, 95 % CI: 1.3-9.5), cytotoxic edema (OR = 63.6, 95 % CI:16.5-244.2), hemorrhage (OR = 16.58, 95 % CI: 4.3-64.2), and severe PRES patterns (OR = 11.0, 95 % CI: 3.5-34.7) on MR were all significantly associated with poor outcomes (p-values = 0.008 and 0.014, <0.001, <0.001, and < 0.001, respectively). This remained true for cytotoxic edema (OR = 84.26, 95 % CI: 17.3-410.9, p-value < 0.001) and hemorrhage (OR = 44.56, 95 % CI: 6.9-289.7, p-value < 0.001) on multivariate analysis. CONCLUSION: Diffusion restriction and hemorrhage on initial MR scans were the two independent predictors of poor outcomes in non-oncologic pediatric patients.
Asunto(s)
Edema Encefálico , Síndrome de Leucoencefalopatía Posterior , Humanos , Niño , Masculino , Femenino , Adolescente , Síndrome de Leucoencefalopatía Posterior/diagnóstico por imagen , Síndrome de Leucoencefalopatía Posterior/complicaciones , Imagen por Resonancia Magnética , Hemorragia/complicaciones , Edema , Estudios RetrospectivosRESUMEN
INTRODUCTION: Sporadic Creutzfeldt-Jakob disease (sCJD) is a fatal neurodegenerative condition caused by prion proteins. Cortical and subcortical diffusion-weighted imaging restriction on magnetic resonance imaging (MRI) is associated with sCJD. Posterior reversible encephalopathy syndrome (PRES) results from impaired vessel autoregulation due to an identifiable trigger, which is associated with subcortical fluid-attenuated inversion recovery changes on MRI. We report a case of sCJD initially presenting with PRES. CASE REPORT: A 70-year-old woman presented to an outside hospital with progressive confusion and difficulty in managing activities of daily living. Initial examination revealed stuporous mental state and stimulus-induced myoclonus. MRI revealed bilateral subcortical occipital lobe T2-fluid-attenuated inversion recovery hyperintensities without contrast enhancement suggestive of PRES. Electroencephalogram (EEG) revealed frequent generalized periodic discharges meeting criteria for nonconvulsive status epilepticus. Clinical examination and EEG did not improve despite escalating antiseizure medications. Initial lumbar puncture was unremarkable. She was transferred to our hospital with a presumptive diagnosis of PRES, although there was no clear trigger. Continuous EEG revealed ongoing generalized periodic discharges with myoclonic activity meeting criteria for myoclonic seizures that were refractory to multiple antiseizure medications. Repeat MRI showed resolution of PRES but revealed subtle diffuse cortical diffusion-weighted imaging restriction. Repeat lumbar puncture was performed and 14-3-3 and real-time quaking-induced conversion returned positive, confirming sCJD. CONCLUSIONS: This case reports highlights that sCJD can present with neuroimaging consistent with PRES. The diagnosis of sCJD should be considered in patients with PRES who continue to show neurological decline despite optimal management and radiographic improvement of PRES on MRI. Further research is needed to identify a pathophysiological relationship between these clinical phenotypes.