Rheumatologic diseases and posterior reversible encephalopathy syndrome: two case reports and review of the literature

Rheumatologic diseases have varied clinical presentations, and posterior reversible encephalopathy syndrome (PRES) can be one of their presentations. The exact etiology of PRES is unknown, but endothelial dysfunction and immunosuppressive medications seem to be the likely cause in rheumatologic diseases. Clinical features include headaches, seizures, altered mental status, cortical blindness, vomiting, and focal neurologic deficits. The diagnosis of PRES can be difficult because several neuropsychiatric illnesses are generally prevalent in rheumatologic diseases; however, a high index of suspicion among physicians along with neuroimaging can help in the accurate diagnosis. Treatment guidelines are lacking, but in a few case series, lowering the blood pressure, controlling the seizures, and removing the immunosuppressive drugs have shown good results. There is need for randomized controlled trials addressing the treatment of PRES in rheumatologic diseases. Medline search was done from year 1950 to March 2011 using ??posterior reversible encephalopathy?? as keyword, and articles relevant to rheumatology were reviewed. We found 48 case reports showing PRES in patients with rheumatologic disease. Most of the patients were female. Age range was from 6 to 59?years. Out of the 48 case reports, 38 patients had systemic lupus erythematosus and most of them had renal disease. Five patients with autoimmune diseases presented with PRES after being started on immunomodulatory drugs. The most frequent symptoms were headache, seizures, and visual changes.     

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.     

Takayasu's arteritis and posterior reversible encephalopathy syndrome: a case-based review

Autoimmune vasculitides can have diverse neurological manifestations, including posterior reversible encephalopathy syndrome (PRES). Takayasu's arteritis (TA) is an uncommon vasculitis rarely associated with PRES. Common clinical features of TA include hypertension, audible arterial bruits, absence of peripheral pulses, claudication of the extremities, reduced blood pressure in one or both arms, and angiographic abnormalities. PRES has been mostly associated with severe hypertension, endothelial injury, and conditions such as renal disease, immunosuppressive medication use, and rheumatologic diseases. Headaches, seizures, and altered mental status are the main clinical features as well as characteristic findings in magnetic resonance imaging. TA frequently presents with hypertension and is associated with endothelial injury, making this entity an ideal setting for the development of PRES. We report the case of a 17-year-old female who presented to the emergency department with severe hypertension, headache, and seizures. Magnetic resonance imaging findings were suggestive of PRES. She had absent pulses in the right upper extremity, abdominal bruits, and angiographic findings included subclavian and renal artery stenoses. The diagnosis of TA was made, and she responded well to treatment. We found ten additional cases of TA and PRES in the literature. All patients were females under the age of 40, had renovascular hypertension, and presented with headaches and seizures. Current literature relevant to this rare association is presented and discussed.     

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 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.     

Recurrent posterior reversible encephalopathy syndrome (PRES)

Posterior reversible encephalopathy syndrome is a proposed cliniconeuroradiological entity characterized by headache, altered mental status, cortical blindness, seizures, and other focal neurological signs, and a diagnostic magnetic resonance imaging picture. A variety of different etiologies have been reported like hypertension, pre-eclampsia/eclampsia, cyclosporin A or tacrolimus neurotoxicity, uraemia and porphyria. With early diagnosis and prompt treatment, the syndrome is usually fully reversible. We report a case of recurrent PRES of unknown aetiology following intensive care unit treatment and only moderately elevated blood pressure. Clinicians as well as radiologists must be familiar with this clinically frightening, underdiagnosed condition to assure timely diagnosis and treatment to prevent persistent deficits.     

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.     

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.     

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.     

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.     

Nephrotic Syndrome and Atypical Posterior Reversible Encephalopathy Syndrome in a Patient with Parkinson's Disease

A 59-year-old man with advanced Parkinson''s disease treated using levodopa-carbidopa intestinal gel (LCIG) presented with leg edema, hypoalbuminemia, and proteinuria at 1 year after the treatment. He subsequently developed a generalized tonic-clonic seizure, and brain magnetic resonance imaging indicated vasogenic edema in the white matter of the left frontal subcortex. He was diagnosed with nephrotic syndrome (NS) and atypical posterior reversible encephalopathy syndrome (PRES). LCIG cessation and corticosteroid treatment improved the NS. To our knowledge, this is the first case report of NS and atypical PRES in patients with Parkinson''s disease. Patients being treated with LCIG should be closely monitored for NS.     

Tacrolimus-associated posterior reversible encephalopathy syndrome after allogeneic haematopoietic stem cell transplantation

Neurotoxicity is a significant complication of the use of tacrolimus. From April 1998 to December 2001, we identified 10 patients (six women, four men) who developed 11 episodes of tacrolimus-associated posterior reversible encephalopathy syndrome (PRES) after allogeneic haematopoietic stem cell transplantation for haematological malignancies. The diagnosis was made by characteristic clinical findings (mental status changes, seizures, neurological deficits) with the exclusion of other causes and characteristic imaging findings. The median age was 35.5 years (range 19-57 years). Seven patients received a matched-unrelated donor transplant and three received a cord blood transplant. The overall incidence of PRES was 1.6%, while the incidence in matched-unrelated, mismatched-related and cord blood transplants was 3.5%, 4.9% and 7.1% respectively. Mental status changes, cognitive deficits, seizures and lethargy were the most common clinical findings. Eight of 10 patients had characteristic findings of hyperintensity of the white matter on T2-weighted images and FLAIR (fluid-attenuated inversion recovery) sequence on magnetic resonance imaging of the brain. Serum tacrolimus levels were within the therapeutic range in most patients. Tacrolimus treatment was continued (n = 4) or temporarily withheld (n = 7) for 1-14 d. One patient was changed to cyclosporine. In most patients, subsequent treatment with tacrolimus was well tolerated without recurrence of neurotoxicity.     

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.     

Posteriores reversibles Enzephalopathiesyndrom (PRES)

Background

Posterior reversible encephalopathy syndrome (PRES) is an acute neurological disease. Although there are some case reports of patients with a poor clinical outcome, studies about PRES and intensive care medicine are rare.

Patients and methods

The medical records of our department were screened for the diagnosis of PRES. Data of 26 patients were analyzed retrospectively.

Results

Of the 26 PRES patients, 65.4% were treated in the intensive care unit (n=13) or stroke unit (n=4) for a mean duration of 3.9 days (range: 1–9 days). During admission, the correct diagnose was suspected for only 1 patient. Generalized seizures occurred in 88.5%. Only 1 patient needed artificial ventilation (>12 h).

Conclusion

Patients with PRES are frequently treated in the intensive care unit/stroke unit because of the clinical occurrence of the syndrome as a severe neurological disease. On the other hand, there are many life-threatening differential diagnoses. Epileptic seizures are the most frequent symptoms. Especially the combination of disturbed vision and epileptic seizures make the diagnosis of PRES more likely.     

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: a rare neurological manifestation in Von Hippel-Lindau disease

We report on a 34-year-old woman, who was recently diagnosed with Von Hippel-Lindau disease (VHL), genetically confirmed. At this moment, she presented with an acute history of arterial hypertension, headache, cortical blindness and epilepsy. On the basis of clinical and magnetic resonance imaging (MRI) criteria the diagnosis of a posterior reversible encephalopathy syndrome (PRES) was made. A iodine 123-Labeled metaiodobenzylguanidine (MIBG) scan revealed the presence of bilateral adrenal pheochromocytomas.     

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.     

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.     

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.     

Coincidence of Apical Ballooning Syndrome (Tako-Tsubo/Stress Cardiomyopathy) and Posterior Reversible Encephalopathy Syndrome: Potential Common Substrate and Pathophysiology?

BackgroundApical ballooning syndrome (ABS) and posterior reversible encephalopathy syndrome (PRES) are recently described, seemingly unrelated, reversible conditions. The precise pathophysiology of these syndromes remains unknown. The aim of this study was to describe the clinical characteristics and outcomes of a unique series of patients with both ABS and PRES.Methods and ResultsIn a retrospective study of 224 consecutive patients diagnosed with ABS between 2002 and 2010, 6 (2.7%) were also diagnosed with PRES. All were female with a mean age of 63.7 ± 12.5 years. All patients had preceding medical comorbidities and physical stress triggers that precipitated ABS and PRES. Mean peak troponin T levels and left ventricular ejection fraction at presentation were 0.47 ± 0.48 mg/dL and 31.5 ± 8.2%, respectively. Characteristic left ventricular wall motion abnormalities (regional wall motion score index 2.22 ± 0.37) were noted in all patients, and magnetic resonance imaging of the brain was significant for vasogenic edema, predominantly in the posterior circulation. All patients recovered left ventricular (ejection fraction at follow-up 60.2 ± 6.0%) and neurologic function with supportive management. Two patients had recurrence of ABS and 1 of PRES during follow-up.ConclusionsABS and PRES can occur simultaneously during an acute illness. Patients with ABS who develop neurologic dysfunction should be evaluated for PRES and vice versa. Because transient sympathetic overactivity and microvascular dysfunction have been observed in both reversible syndromes, we speculate that they may represent the shared pathophysiologic mechanism.