PRES: posterior or potentially reversible encephalopathy syndrome?

Abstract Posterior reversible encephalopathy syndrome (PRES) is an acronym that identifies a new clinico-neuroradiologic entity occurring in association with different conditions. We report a patient with eclamptic encephalopathy whose clinico-radiological picture normalised after prompt treatment. We suggest defining this condition as potentially RES, to emphasise that reversibility is not spontaneous but is usually related to an adequate treatment, and that the posterior localisation of the lesions, even if constant, may not represent the most relevant finding in some patients.     

可逆性后部白质脑病综合征1例报告并文献复习

目的：探讨可逆性后部白质脑病的常见病因、发病机制、临床表现、诊断及治疗措施。方法对1例产褥期可逆性后部白质脑病综合征患者的诊疗经过进行分析。结果患者有中枢神经系统症状、体征,结合相关检查结果,采用脱水、营养神经、对症支持治疗,预后良好。结论可逆性后部白质脑病综合征是一种由多种原因引起的以神经系统异常为主要表现的临床综合征。常见的病因有恶性高血压、妊娠子痫、各类严重肾脏疾病、免疫抑制剂及细胞毒药物、自身免疫病的治疗等。急性或亚急性起病,临床表现多种多样,常见的有头痛、痫性发作、视觉障碍、意识障碍及精神异常等。临床诊断主要依据其典型的影像学改变,大多数影像学改变为可逆性,预后良好。     

Predictors of poor outcome in patients with posterior reversible encephalopathy syndrome

Purpose: Posterior reversible encephalopathy syndrome (PRES) is an acute neurotoxic syndrome that, although characteristically reversible, can be fatal or result in long-term disability in a subset of patients. Our aim was to identify factors associated with poor discharge outcome in PRES patients. Materials and methods: We retrospectively reviewed the clinical and radiological records of all patients with PRES admitted at our tertiary care medical center from 2007 to 2014. They were divided based their modified Rankin Score at discharge and compared for their baseline variables, clinical, laboratory and imaging features. Poor outcome was defined by a modified Rankin scale 2–6 and was subdivided based on the primary mechanism that led to poor outcome. Results: Out of 100 PRES subjects, 36% had poor discharge outcomes. Factors associated with poor outcomes on univariate analysis were history of diabetes mellitus, coma, high Charlson comorbidity index, post-transplantation, autoimmune condition, lack of systolic or diastolic hypertension, elevated blood urea nitrogen and involvement of the corpus callosum. On multivariate analysis, only prior diabetes mellitus odd ratio (OR) = 6.8 (95% CI 1.1–42.1, p = 0.04), corpus callosum involvement (OR = 11.7; 95% CI 2.4–57.4, p = 0.00) were associated with poor outcome. Poor outcome also correlated with increased length of hospital stay (OR = 7.9; 95% CI 1.3–49.7, p = 0.03). Conclusion: Large prospective studies incorporating serial blood glucose values and advanced imaging studies are required to validate these findings.     

HELLP syndrome with reversible posterior leukoencephalopathy

We report the case of a 30-year-old primipara who developed a triplet pregnancy after having received artificial insemination. At the end of the eighth month of pregnancy she had a generalized tonic-clonic seizure and at the same time blood chemistry was indicative of HELLP syndrome. Brain MRI and EEG were altered immediately after the episode but returned to normal during a 6-month follow-up period. Received: 15 December 2002 / Accepted in revised form: 14 April 2003 Correspondence to: E. Marano     

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

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

A case of autoimmune thyroid disease presenting posterior reversible encephalopathy syndrome

A 40-year-old woman was admitted to our hospital with disturbance of consciousness and seizure. We diagnosed encephalopathy associated with autoimmune thyroid disease (EAATD). Fluid-attenuated inversion recovery and diffusion-weighted MRI demonstrated hyperintense lesions in the left occipitotemporal lobe on admission, but these findings disappeared on day 11 without neurological deficits, compatible with posterior reversible encephalopathy syndrome (PRES). We report here this case of autoimmune thyroid disease presenting as PRES.     

Ictal asystole in a patient with posterior reversible encephalopathy syndrome (PRES) and seizures

We describe a case of ictal asystole in a patient with posterior reversible encephalopathy syndrome (PRES), which has never been described previously. Ictal asystole is rare and has possible serious medical consequences, including syncope, and may be a potential mechanism for sudden unexpected death in epilepsy (SUDEP). Awareness that PRES may be observed with recurrent ictal asystole may aid in the recognition and treatment of seizures in this condition and the prevention of asystole‐associated complications.     

Spontaneous posterior fossa hemorrhage associated with low-molecular weight heparin in an adolescent recently diagnosed with posterior reversible encephalopathy syndrome: case report and review of the literature

Background Posterior reversible encephalopathy syndrome (PRES) constitutes a well-described clinical entity, classically entailing characteristic edematous lesions on imaging studies in a patient with altered mental status, visual disturbances, headache, and seizures. PRES is reversible in the vast majority of cases, although progression to ischemia and hemorrhage has been documented.Methods We report a case of a 16-year-old male with chronic renal failure who developed PRES during a hypertensive crisis. The hypertension was successfully managed and PRES-associated symptomatology showed complete regression. However, approximately 2 months later, the patient returned with a spontaneous posterior fossa hemorrhage. Two weeks before this second admission, treatment with low-molecular weight heparin had been initiated. The patient finally succumbed, despite surgical evacuation. The pertinent literature is reviewed.Conclusion We suggest that the administration of low-molecular weight heparin in the setting of recent PRES might increase the risk for the development of intracranial hemorrhage.     

Unusual case of intra-arterial doxorubicin chemoembolization-associated posterior reversible encephalopathy syndrome

A 63-year-old white female with a history of metastatic breast cancer to the liver developed acute-onset nausea, vomiting, mental status change, and generalized seizures following transarterial chemoembolization using doxorubicin. The patient was hospitalized with the above symptoms immediately following transarterial chemoembolization using drug eluting bead doxorubicin into the right hepatic artery. The patient developed intractable nausea, vomiting, and abdominal pain and had a generalized tonic-clonic seizure lasting for 40 seconds, approximately 24 hours after the procedure. The patient was confused and lethargic for 2 days with progressive improvement in her mental status. Her neurological examination showed encephalopathy with disorientation to time, place or person, and she also had a glassy look. Cranial nerves were normal other than lack of response to threat stimulus bilaterally; motor and sensory examination was unremarkable. Initial blood pressure was 130/90 mm Hg and routine chemistry and complete blood count on admission were within normal limits. The cerebrospinal fluid analysis showed clear and colorless fluid with glucose of 56 mg/dL, protein of 42 mg/dL, white blood cells of 2/μL, and red blood cells of 10/μL and did not show any evidence of infectious or toxic etiology on encephalopathy. Continuous electro encephalography showed diffuse slowing but no epileptiform discharges. The magnetic resonance imaging (MRI) revealed increased signal intensity in the bilateral parieto-occipital area, right more than the left, on fluid-attenuated inversion recovery, apparent diffusion coefficient, and T2-weighted imaging, with no increased signal on diffusion weighted image consistent with vasogenic edema. The patient's symptoms and MRI findings were consistent with diagnosis of posterior reversible encephalopathy syndrome. Resolution of the MRI changes is noted on the follow-up imaging 8 weeks later. Posterior reversible encephalopathy syndrome in this case is most likely related to intra-arterial doxorubicin infusion because of the temporal association between administration, symptom onset, and MRI changes.     

子痫前期或子痫致可逆性后部脑病综合征的临床及影像学分析

目的 探讨子痫前期或子痫致可逆性后部脑病综合征( posterior reversible encephalopathy syndrome,PRES)的临床及影像学特点.方法 选择子痫前期或子痫致PRES患者21例,采用回顾性分析方法对临床症状及脑磁共振成像(magnetic resonance imaging,MRI)资料进行分析.结果 主要临床症状:21例患者中癫痫发作18例,头痛16例,意识状态改变15例,视觉障碍12例;PRES病灶分布:顶、枕叶20例,额叶14例,颞叶11例,基底节区11例,胼胝体压部4例,小脑半球3例,脑干1例;PRES病灶分布模式:经典型7例,全脑型7例,额上沟型6例,部分或不对称型1例.结论 子痫前期或子痫致PRES临床症状典型,除顶、枕叶外,额叶、颞叶、基底节区等部位累及常见,多见3种主要病灶分布模式,准确识别其影像学表现对正确诊断具有重要意义.     

Posterior reversible encephalopathy syndrome following hemodynamic treatment of aneurysmal subarachnoid hemorrhage-induced vasospasm

Posterior reversible encephalopathy syndrome (PRES) is an uncommon but significant complication of hemodynamic therapy after aneurysmal subarachnoid hemorrhage (aSAH)-induced vasospasm. We performed a PubMed literature search for the period January 1999 to January 2015 using the search terms “posterior reversible encephalopathy syndrome”, “subarachnoid hemorrhage”, “vasospasm”, and “hypertensive encephalopathy”, and identified nine cases of PRES after aSAH-induced vasospasm in the literature. We also present a 63-year-old man with aSAH complicated by vasospasm treated with hemodynamic augmentation who subsequently developed PRES. Imaging following development of PRES symptoms shows vasogenic edema in the white matter of the parietal and occipital lobes. Age, sex, history of hypertension, and baseline blood pressure were variable among patients in the literature review. In all cases, patients improved both from a radiological and clinical perspective following blood pressure reduction. To summarize, PRES is a rare complication of hemodynamic therapy for vasospasm following aSAH. The literature at the time of writing demonstrates no common pattern with regard to patient demographics, medical history, or mode of treatment for symptomatic vasospasm. Given its sporadic and unpredictable nature, considering PRES in the differential diagnosis is important when addressing neurological decline following hemodynamic treatment of vasospasm related to aSAH.     

可逆性后部脑病综合征四例的临床和影像学特征分析

目的探讨可逆性后部脑病综合征（RPES）的临床特征和MRI表现。方法回顾性分析4例经临床诊断RPES的临床和MRI资料。结果4例患者均有血压升高，神经症状有剧烈头痛（2例）、烦躁不安（3例）、视觉模糊（2例）、近期记忆力减退（1例）、癫痫发作（2例）；MRI上病灶主要对称性分布于顶叶后部及枕叶（4例），表现为T2加权像及压水像高信号，T1加权像低信号，2例主要累及皮质，1例表现皮质及皮质下白质同时受累，1例主要累及白质。4例复查时病灶明显消散。结论RPES的MRI表现较为有特征性，病灶多对称性分布于大脑后部，累及皮质或皮质下白质，为可逆性病灶；结合临床血压升高和某些后部脑结构受累所致神经症状等特点有助于该综合征的早期鉴别诊断。     

伴有胼胝体压部可逆性病灶的临床症状轻微的脑炎/脑病1例报道并文献复习

目的探讨伴有胼胝体压部可逆性病灶的临床症状轻微的脑炎/脑病的临床特点、治疗及预后、发病机制。方法报道1例伴有胼胝体压部可逆性病灶的临床症状轻微的脑炎患者的诊治过程,总结其临床特征并复习相关文献。结果患者表现为发热、头痛、呕吐等非特异性脑膜炎症状,合并出现抗利尿激素分泌不当导致的低钠血症,头颅MRI检查发现胼胝体压部有可逆性的DWI高信号的孤立病灶,经抗病毒及对症治疗,临床痊愈。结论伴有胼胝体压部可逆性病灶的临床症状轻微的脑炎/脑病以头颅MRI上发现胼胝体压部可逆性病灶为特点,临床症状轻微,容易合并低钠血症,预后良好,病因及发病机制尚不清楚。     

可复性后部脑病综合征的影像学诊断

目的探讨可复性后部脑病综合征（PRES）的影像学表现.方法回顾性分析了12例PRES病人的临床和影像学资料,其中9例为子痫/先兆子痫,2例为高血压脑病,1例为环孢菌素A（CSA）的神经毒性.12例均行MRI检查,其中7例同时行钆喷替酸葡甲胺（Gd-DTPA）增强扫描,4例行磁共振血管造影（3D-TOF MRA）检查,1例行弥散加权成像（DWI）.7例行CT平扫检查,2例行脑血管造影（DSA）检查.结果MRI显示病灶基本上呈双侧对称性分布,多数病灶位于顶、枕叶脑实质内,T1WI呈等或略低信号,T2WI呈高信号,FLAIR像显示皮层和皮层下白质明显高信号影,较T1WI、T2WI更加清楚.注射Gd-DTPA后多无明显异常对比增强.1例DWI显示双侧顶、枕叶及额叶皮层内弥散受限呈高信号,ADC图显示邻近的皮层下白质呈高信号.4例CT显示双侧顶、枕叶及额叶对称性斑片状低密度影,3例CT未见异常.经对症处理后复查示所有病灶几乎完全吸收消失.结论PRES的影像学表现具有特征性.MRI应作为诊断本病的首选手段.     

A unique case of intraventricular hemorrhage associated with posterior reversible encephalopathy syndrome in an adolescent

Intraventricular hemorrhage is a rare finding in patients with the posterior reversible encephalopathy syndrome and generally carries a poor prognosis. We report a unique case of an 18-year-old girl with glomerulonephritis who developed posterior reversible encephalopathy syndrome without hypertension but with a primary intraventricular hemorrhage and subarachnoid blood without demonstrable parenchymal blood. The normotensive presentation of posterior reversible encephalopathy syndrome and intraventricular hemorrhage in association with systemic vasculitis is rare. Our patient had a good initial outcome and was discharged with resolution of her symptoms and signs of raised intracranial pressure.     

Presentation of reversible posterior leukoencephalopathy syndrome in patients on calcineurin inhibitors

Background

Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinico-radiologic diagnosis associated with numerous medical conditions including hypertension, immunosuppressant medications, and eclampsia. It is characterized by headache, altered mental status, seizures, visual disturbance, and neuroimaging consistent with posterior-predominant vasogenic edema.The objective of this study was to characterize the clinical spectrum and outcomes in a large series of RPLS patients, and to compare the presentation of patients taking calcineurin inhibitors (CNIs) to that of other RPLS patients.

Methods

We reviewed records of patients seen by the neurology and transplant services over an 18-year period. Comorbid conditions, medications, blood pressure, laboratory testing, clinical outcomes, and radiographic findings were collected.

Results

84 episodes of RPLS were identified in 79 patients. Etiologies included CNIs (43%), hypertension (29%), renal disease (12%), preeclampsia/eclampsia (7%), and chemotherapy (5%). Patients on CNIs had lower blood pressures (p = 0.002) and a lower prevalence of headache (p = 0.02) compared to RPLS patients with other etiologies. Clinical recovery occurred in 65% of episodes, and radiographic resolution occurred in 67%.

Conclusions

Patients with CNI-induced RPLS have lower blood pressure than other RPLS patients, but otherwise present similarly. RPLS typically occurs within days to weeks of CNI initiation in patients without elevated medication levels. Clinical and radiographic recovery occurred in the majority of patients in this series, but one-third suffered residual neurologic deficits or death. These findings highlight the importance of prompt recognition and treatment of RPLS triggers to prevent permanent sequelae.