Posterior reversible encephalopathy syndrome in a patient with ANCA-associated vasculitis

Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome that manifests with the onset of headache, confusion or decreased level of consciousness, visual changes, and seizures in conjunction with the typical neuroimaging features of posterior cerebral white matter changes, which are usually reversible. This syndrome has been associated with hypertension, eclampsia, renal insufficiency, immunosuppressive drugs, and connective-tissue diseases. To our knowledge, only four cases of PRES associated with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis have been reported in the literature. Here, we present a patient with ANCA-associated vasculitis complicated by PRES.     

Posterior reversible encephalopathic syndrome due to severe hypercalcemia in AIDS

A reversible syndrome of headache, altered mental status, seizures and cerebral visual abnormalities with neuroradiological studies suggesting predominantly posterior white matter abnormalities has been described. This syndrome has been referred to as posterior reversible encephalopathic syndrome (PRES). PRES has been associated with hypertensive encephalopathy, eclampsia and treatment with immunosuppressive or cytotoxic agents. Rare case reports describe association with hypercalcemia. We present a patient with AIDS, Mycobacterium avium intracellulare related hypercalcemia with posterior reversible encephalopathic syndrome.     

Posterior reversible encephalopathy syndrome during systemic lupus erythematosus: four new cases and review of the literature

Posterior reversible encephalopathy syndrome (PRES) associates various neurological manifestations (headaches, seizures, altered mental status, cortical blindness, focal neurological deficits, vomiting) and transient changes on neuroimaging consistent with cerebral edema. Posterior reversible encephalopathy syndrome mainly occurs in the setting of hypertension, eclampsia, renal failure and/or use of immunosuppressive drugs.We report four cases of PRES complicating systemic lupus erythematosus (SLE). In all our cases, renal involvement and hypertension were present. Neurological symptoms were typical. Magnetic resonance imaging showed posterior cerebral edema and in one case hemorrhagic complication. With symptomatic treatment and immunosuppressor withdrawal when they were previously used, symptoms fully resolved within 15 days in all cases, but one who had only partial regression related to cerebral hemorrhage.Including our cases, we reviewed a total of 46 patients with SLE and PRES. Their clinical and radiological presentation was not specific. The peculiar role of SLE itself in the occurrence of PRES was not clear, since hypertension (95%), renal involvement (91%), recent onset of immunosuppressive drugs (54%) and/or recent treatment with high intravenous dose of steroids (43%) were often present. The hypertension and other worsening factors should be treated. Finally, the evolution of this clinical and radiological spectacular syndrome is generally rapidly favorable.     

Posterior reversible encephalopathy syndrome--an underrecognized manifestation of systemic lupus erythematosus

OBJECTIVE: Posterior reversible encephalopathy syndrome (PRES) is a rare, recently described neurologic condition identifiable by clinical presentation and magnetic resonance image (MRI) appearance. It is associated with renal insufficiency, hypertension, and rheumatologic diseases. Patients present with headache, seizures, loss of vision and altered mental function, and a pattern on imaging studies of predominantly transient, posterior cerebral hyperintensities on T2-weighted MRI. There is a high likelihood of presentation of this syndrome to a rheumatologist. METHODS: Three recent cases of systemic lupus erythematosus (SLE) with PRES, along with 9 previously reported cases, are reviewed. RESULTS: All 3 patients presented with seizures and subacute visual changes in association with lupus nephritis. The first presented with hypertension, complete visual field loss, and status epilepticus 2 weeks after starting oral cyclosporine therapy for refractory lupus nephritis. The second patient was normotensive and presented with seizures and visual symptoms while in hospital with SLE-related pancreatitis and nephritis. The third patient had headache and seizures with severe lupus disease activity including nephritis, pancytopenia, and pulmonary hemorrhage. Cranial MRI showed predominantly posterior signal abnormalities on T2-weighted images, which resolved after cessation of cyclosporine in the first case, treatment with IV cyclophosphamide in the second case, and treatment with cyclophosphamide and plasmapheresis in the final case. Literature review showed that PRES is a manifestation of SLE or a consequence of therapy with calcineurin inhibitors or rituximab. The hallmark features are visual loss and seizures. Severe hypertension (> 170/110 mm Hg) and renal failure were present in the majority of previously identified cases of SLE and PRES. Our second case was normotensive but had marked lupus disease activity. PRES can lead to cerebral infarction. CONCLUSION: With increasing availability of MRI, PRES will be identified more frequently. Swift action to identify potential offending agents, controlling hypertension, and treating active disease can lead to reversal of radiologic and neurologic findings.     

Posterior reversible encephalopathy syndrome (PRES) in a patient with moyamoya disease: A case report

Introduction:Moyamoya disease (MMD) and posterior reversible encephalopathy syndrome (PRES) share similar pathophysiological characteristics of endothelial dysfunction and impaired cerebral autoregulation. However, there have never been any published studies to demonstrate the relationship between these 2 rare diseases.Patient concerns:A 26-year-old Asian man presented with a throbbing headache, blurred vision, and extremely high blood pressure. We initially suspected acute cerebral infarction based on the cerebral computed tomography, underlying MMD, and prior ischemic stroke. However, the neurological symptoms deteriorated progressively.Diagnosis:Cerebral magnetic resonance imaging indicated the presence of vasogenic edema rather than cerebral infarction.Interventions and outcomes:An appropriate blood pressure management prevents the patient from disastrous outcomes successfully. Cerebral magnetic resonance imaging at 2 months post treatment disclosed the complete resolution of cerebral edema. The patient''s recovery from clinical symptoms and the neuroimaging changes supported the PRES diagnosis.Conclusion:This report suggests that patients with MMD may be susceptible to PRES. It highlights the importance of considering PRES as a differential diagnosis while providing care to MMD patients with concurrent acute neurological symptoms and a prompt intervention contributes to a favorable clinical prognosis.     

Primary Hyperparathyroidism Presenting as Posterior Reversible Encephalopathy Syndrome: A Report of Two Cases

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological entity characterized by subcortical vasogenic edema presenting with acute neurological symptoms. Common precipitating causes include renal failure, pre-eclampsia/eclampsia, post-organ transplant, and cytotoxic drugs. Hypercalcemia is a rare cause of PRES; most cases occur in the setting of severe hypercalcemia secondary to malignancy or iatrogenic vitamin D/calcium overdose. Primary hyperparathyroidism (PHPT), as a cause of PRES, is an oddity. We report two cases of adolescent PHPT presenting with generalized tonic-clonic seizures and altered sensorium. On evaluation, both had hypertension, severe hypercalcemia (serum calcium 14.1 mg/dL and 14.5 mg/dL, respectively) and elevated parathyroid hormone levels. Magnetic resonance imaging (MRI) revealed T2/fluid-attenuated inversion recovery hyperintensities located predominantly in the parieto-occipital regions, suggestive of PRES. Identification and excision of parathyroid adenoma led to the restoration of normocalcemia. Neurological symptoms and MRI changes improved subsequently. An extensive literature search revealed only four cases of PHPTassociated PRES; none of them being in the pediatric/adolescent age group. The predominant clinical manifestations were seizures and altered sensorium. All had severe hypercalcemia; three had hypertension at presentation, while one was normotensive. Parathyroid adenomectomy led to normalization of serum calcium and resolution of neurological symptoms and radiological changes. Thus, severe hypercalcemia, although rare in PHPT, can lead to hypercalcemic crisis precipitating acute hypertension that can result in cerebral endothelial dysfunction with the breakdown of the blood-brain barrier, culminating in PRES. We therefore recommend that serum calcium levels should be checked in all patients with PRES and that PHPT be regarded as a differential diagnosis in those with underlying hypercalcemia.     

Posterior reversible encephalopathy syndrome: magnetic resonance imaging and diffusion-weighted imaging in 12 cases

Posterior reversible encephalopathy syndrome (PRES) is a potentially devastating neurologic syndrome, but timely treatment may lead to complete reversal of the disease course. We reviewed 12 cases of PRES and describe the clinical history and imaging findings, including conventional magnetic resonance imaging (MRI), diffusion-weighted imaging (DWI), and calculated apparent diffusion coefficient (ADC) maps, used to establish the diagnosis of PRES. Three male and nine female patients aged between 11 and 70 years (mean, 37 years) with clinical and imaging findings consistent with PRES were enrolled in the study. All patients had undergone conventional MRI and 10 had undergone additional DWI studies. Ten patients had follow-up MRI studies. DWI was performed using a 1.5T system with a single-shot spin-echo echoplanar pulse sequence. Initial and follow-up neuroimaging and clinical history were reviewed. Lesions were almost always present over the posterior circulation, mainly the parieto-occipital region, affecting primarily the white matter. The anterior circulation region, brainstem, cerebellum, deep cerebral white matter, and thalamus were also involved in five cases. Conventional MRI revealed hyperintensity on T2-weighted and fluid-attenuated inversion recovery images. DWI showed isointensity and increased signal intensity on ADC values in all cases, indicating vasogenic edema. Clinical and MRI follow-up showed that the symptoms and radiologic abnormalities could be reversed after appropriate treatment of the causes of PRES in most patients (9 of 10). In one patient, the ADC value was lower on follow-up images, indicating cytotoxic edema with ischemic infarct. DWI was a useful complement to MRI in the diagnosis of PRES.     

Actual versus ideal body weight for acute kidney injury diagnosis and classification in critically Ill patients

Background

Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiological entity characterized clinically by headache, altered mental status, seizures, visual disturbances, and other focal neurological signs, and radiographically by reversible changes on imaging. A variety of different etiologies have been reported, but the underlying mechanism is thought to be failed cerebral autoregulation. To the best of our knowledge, we report the third known case of PRES in an adult receiving intermittent peritoneal dialysis (PD).

Case presentation

A 23-year-old male receiving PD was brought to hospital after experiencing a generalized seizure. On presentation he was confused and hypertensive. An MRI brain was obtained and showed multiple regions of cortical and subcortical increased T2 signal, predominantly involving the posterior and paramedian parietal and occipital lobes with relative symmetry, reported as being consistent with PRES. A repeat MRI brain obtained three months later showed resolution of the previous findings.

Conclusion

Due to having a large number of endothelium-disrupting risk factors, including hypertension, uremia, and medications known to disrupt the cerebrovascular endothelium, we suggest that those with end-stage renal disease (ESRD) receiving PD are at high risk of developing PRES. Furthermore, we surmise that PRES is likely more prevalent in the ESRD population but is under recognized. Physicians treating those with ESRD must have a high index of suspicion of PRES in patients presenting with neurological disturbances to assure timely diagnosis and treatment.     

Tumor lysis syndrome as a risk factor for posterior reversible encephalopathy syndrome in children with hematological malignancies

Posterior reversible encephalopathy syndrome (PRES) is a neurological disorder characterized by seizures, altered mental status and visual disorders, along with characteristic radiological findings. It is strongly related to hypertension induced by steroids and other immunosuppressive agents. There are an increasing number of reports regarding PRES arising during the course of chemotherapy for hematological malignancies. To clarify the risk factors for this phenomenon, we retrospectively analyzed pediatric patients undergoing treatment for hematological malignancies. Of 161 patients, six patients (3.7 %) developed PRES with characteristic clinical and radiographic findings. Univariate analysis revealed that tumor lysis syndrome (TLS) was a significant risk factor for the onset of PRES. TLS is a significant risk factor for the development of PRES in pediatric patients receiving chemotherapy for hematological malignancies.     

Posterior reversible encephalopathy syndrome (PRES) and chronic kidney disease

Posterior reversible encephalopathy (PRES) is a recently described syndrome, defined by clinical and neuroimaging features. Chronic kidney disease patients may be especially vulnerable to this syndrome because they are frequently exposed to several of its possible causes, including uremia and hypertension. In its most severe form, PRES can manifest clinically as seizures, coma or death. However, if properly diagnosed and treated, this syndrome can be completely reversible. Therefore, neuroimaging methods, especially brain magnetic resonance is fundamental for its diagnosis because it shows brain edema in characteristic pattern, and excludes causes of seizures or coma. An important example is the case of a young hypertensive chronic kidney disease patient on peritoneal dialysis, brought to the emergency room comatous with generalized tonic-clonic seizures; the cerebral magnetic resonance imaging features were impressive. Anti-hypertensive therapy and hemodialysis allowed complete recovery. The reversibility of this syndrome depends on timely diagnosis and therapy and therefore it should be kept in mind in the differential diagnosis of seizures. or coma in chronic kidney disease patients.     

A case report of reversible posterior encephalopathy syndrome with intracranial hemorrhage in a child

Introduction:The objective is to analyze the clinical diagnosis and treatment of children with rescindable posterior encephalopathy syndrome (PRES) and intracranial hemorrhage (ICH) to improve the pediatrician''s understanding of PRES combined with ICH in children.Patient concerns and Diagnosis:After liver transplantation, the patient developed symptoms of epilepsy and coma. Meanwhile, massive necrosis of acute cerebral infarction and small hemorrhage was observed in the left cerebellar hemisphere and left occipital lobe, respectively. The above symptoms were initially diagnosed as PRES.Interventions and outcomes:After adjusting the anti-rejection drug regimen, it was found that the child''s neurological symptoms were relieved, and the limb motor function gradually recovered during follow-up. Imaging examination showed significant improvement on abnormal signals in brain.Conclusion:In general, children with PRES may further develop ICH and contribute to a poor prognosis. Early diagnosis, detection of risk factors and timely adjustment of medication regimen are the keys to prevent irreversible brain damage.     

系统性红斑狼疮合并脑后部可逆性脑病综合征的临床分析

目的 脑后部可逆性脑病综合征(PRES)是一种有特征性临床和影像学改变的神经精神综合征.通过分析系统性红斑狼疮(SLE)合并PRES患者的临床表现和复习文献,探讨其发病特点.方法 分析4例确诊为SLE合并PRES患者的临床特点,收集其临床表现、发病诱因和影像学检查资料.结果 4例和文献报道共48例SLE合并PRES患者,发生高血压为42例(88%),血肌酐升高为30例(63%),39例使用激素、免疫抑制剂或细胞毒药物(81%).头痛46例(96%),视觉障碍28例(58%),意识障碍20例(42%),癫痫发作43例(90%),CT及磁共振成像(MRI)显示多发病灶主要位于双侧枕叶、顶叶、额叶、颞叶和小脑.降压和及时处理诱因症状可缓解.结论 PRES是一种临床-影像综合征,常与高血压、肾功能衰竭、免疫抑制剂等药物相关,SLE合并PRES若能快速诊断并给予正确治疗可使症状缓解,病变可逆.     

Delayed Gemcitabine-Induced Posterior Reversible Encephalopathy Syndrome

IntroductionPosterior reversible encephalopathy syndrome (PRES) is a rare clinical-radiographic syndrome that has been expanding rapidly in the world of clinical medical oncology and hematology. In this article, we provide a unique patient case of delayed gemcitabine-induced PRES.Brief Case ReportA 60-year-old African American female with significant past medical history of ER+/PR+/HER2- invasive ductal carcinoma of the left breast is seen in the medical oncology clinic with vague, mild complaints of lightheadedness. She had progressed on multiple lines of chemotherapy and was ultimately switched to gemcitabine. One month after her third dose of gemcitabine, she developed acute vision loss and soon developed generalized tonic-clonic seizure. Extensive workup was unrevealing other than PRES and she slowly improved with supportive care and withdrawal of the medication.DiscussionMultiple case reports have described PRES in the context of combination chemotherapy with gemcitabine and a platinum agent in the treatment of gastrointestinal malignancies. With growing evidence, this case is consistent with the hypothesis that gemcitabine as monotherapy has a direct association with PRES. This case highlights a unique aspect in that PRES can occur at a delayed time interval, much further than the expected hours to days after the previous treatment.     

Posterior reversible encephalopathy syndrome in alcoholic hepatitis: Hepatic encephalopathy a common theme

Posterior reversible encephalopathy syndrome(PRES) is a neuro-radiologic diagnosis that has become more widely recognized and reported over the past few decades. As such, there are a number of known risk factors that contribute to the development of this syndrome, including volatile blood pressures, renal failure, cytotoxic drugs, autoimmune disorders, pre-eclampsia, and eclampsia. This report documents the first reported case of PRES in a patient with severe alcoholic hepatitis with hepatic encephalopathy and delves into a molecular pathophysiology of the syndrome.     

A survey on hematology‐oncology pediatric AIEOP centres: The challenge of posterior reversible encephalopathy syndrome

Objectives

Posterior reversible encephalopathy syndrome (PRES) is one of the most common neurological complications in hematology‐oncology pediatric patients. Despite an increasingly recognized occurrence, no clear consensus exists regarding how best to manage the syndrome, because most cases of PRES have reported in single‐case reports or small series. Aim of this paper is to identify incidence, clinical features, management, and outcome of PRES in a large series of hematology‐oncology pediatric patients.

Methods

The cases of PRES occurred in twelve centers of the Italian Association of Pediatric Hematology and Oncology were reported.

Results

One hundred and twenty‐four cases of PRES in 112 pediatric patients were recorded with an incidence of 2.1% and 4.7%, respectively, in acute lymphoblastic leukemia in first complete remission and hematopoietic stem cell transplantation (HSCT). The majority of cases occurred after a cycle of chemotherapy rather than after stem cell transplant. PRES after chemotherapy significantly differs from that after HSCT for diagnosis, time of presentation, risk factors, management, and outcome.

Conclusions

This study demonstrates that PRES is a common neurological complication and occurring preferentially in course of induction treatment of some hematologic malignancies, as ALL and after HSCT. It also highlights great clinical differences in the management and outcome in patients with PRES occurring after chemotherapy or after HSCT.     

Diálisis peritoneal: ¿un factor de riesgo o de protección para la encefalopatía posterior reversible (PRES)? Revisión de la literatura

Posterior reversible encephalopathy syndrome is a clinical and radiological entity with acute or subacute neurological presentation associated with brain lesions that primarily affect the white matter of the posterior regions. It is often associated with the rapid onset of severe hypertension and/or with kidney failure (acute and chronic), but it has also been reported as a neurological complication in several medical conditions. In recent years, there has been an increase in the number of cases and related publications due to the advance of diagnostic imaging techniques. The characteristic radiological finding includes hyperintense lesions in T2- and FLAIR-weighted magnetic resonance imaging, which are often bilateral and located in the posterior cerebral regions and correspond to areas of vasogenic oedema.Little is known about the pathophysiology of posterior reversible encephalopathy syndrome. The most accepted theory, especially in cases with associated hypertension, is the loss of cerebral self-regulation which leads to the onset of vasogenic oedema. The main feature of this syndrome is the reversibility of both symptoms and cerebral lesions with an early and appropriate diagnosis.Despite the frequent association with kidney failure and severe hypertension, there are few cases reported in patients on peritoneal dialysis. This article presents a review of PRES in peritoneal dialysis patients in the published literature.     

Reversible cerebral vasoconstriction syndrome

Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical‐radiological syndrome characterised by severe thunderclap headaches with or without other neurological symptoms and multifocal constriction of cerebral arteries that usually resolves spontaneously within 3 months. Most patients recover completely, but up to 10% have a permanent neurological disability and some even die. Previously RCVS has been described in many clinical contexts and under different names with the term RCVS first being suggested in 2007 to unify the group. The condition may be spontaneous, but in up to 60% of cases it is secondary to another cause, including vasoactive substances (medications and illicit drugs), blood products and the post‐partum state. It is believed to have a similar pathophysiological mechanism to the posterior reversible encephalopathy syndrome (PRES), and both can occur in similar clinical contexts and are frequently associated. Treatment options include calcium channel antagonists. RCVS occurs in a broad range of clinical situations making it an increasingly recognised condition about which doctors in various specialties need to be aware.     

A Young Patient with Microscopic Polyangiitis Requiring Hemodialysis with Complications of Repeated Episodes of Posterior Reversible Encephalopathy Syndrome Probably Due to Different Etiologies

A young woman with microscopic polyangiitis (MPA) requiring hemodialysis showed repeated posterior reversible encephalopathy syndrome (PRES) with spatiotemporal multiple lesions over a period of two months. The first PRES episode with confusion and the second PRES episode with vertigo and nausea were caused by MPA, hypertension and renal failure. These symptoms were improved by the reinforcement of MPA treatment and blood pressure management. The third PRES episode with nausea, headache, seizure and visual changes was induced by rituximab infusion and hypertension. The PRES was improved with blood pressure and convulsant management. These conditions are challenging to diagnose and treat.     

Systemic lupus erythematosus complicated with posterior reversible encephalopathy syndrome and intracranial vasculopathy

Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic condition characterized by reversible vasogenic edema on neuroimaging. It is associated with various neurological manifestations, including headaches, vomiting, seizures, visual loss, altered mental status and focal neurological deficits. PRES mainly occurs in the setting of eclampsia, hypertension, uremia, malignancy, transplantation, autoimmune diseases and/or use of immunosuppressive drugs. This syndrome has been described in patients with systemic lupus erythematosus (SLE). PRES is a potentially reversible clinical-radiological entity; however, it can be complicated with vasculopathy, infarction or hemorrhage. Vasculopathy has been demonstrated to be a common finding in patients with SLE. We report the case of a woman with lupus nephritis and PRES whose diffuse vasculopathy was present on initial neuroimaging. Subsequent brain computed tomography scan demonstrated interval development of intraparenchymal hemorrhage and subarachnoid hemorrhage. To our knowledge, this unique brain image pattern has not been reported in SLE patients.     

Posterior reversible encephalopathy syndrome: another manifestation of CNS SLE?

A variety of neuropsychiatric findings may complicate systemic lupus erythematosus (SLE) and pose diagnostic and therapeutic dilemmas. We describe the clinical and radiographic features of posterior reversible encephalopathy syndrome (PRES) and distinguish PRES from other conditions seen in SLE. Patient charts and magnetic resonance imaging (MRI) findings of four patients with SLE on immunosuppressive therapy with acute or subacute neurologic changes initially suggesting cerebritis or stroke were reviewed. The English language literature was reviewed using the Medline databases from 1996-2006 for other reports of PRES with SLE. Literature review yielded 26 other SLE cases reported with PRES. SLE patients with PRES were more commonly on immunosuppressive drugs, had episodes of relative hypertension, and had renal involvement. Characteristic findings are seen on MRI, which differentiate PRES from other CNS complications of SLE. Clinical and radiographic resolution of abnormalities within 1-4 weeks is typically seen. PRES has been increasingly recognized. Reversible changes are found on brain MRI accompanied by sometimes dramatic signs and symptoms. The therapeutic implications for separating PRES from stroke or cerebritis are important. We propose that PRES should be considered in the differential diagnosis in SLE patients with new-onset neurologic signs and symptoms.