posterior reversible encephalopathy syndrome

后部可逆性脑病综合征
  • 文章类型: Case Reports
    急性间歇性卟啉症是一种罕见的常染色体显性代谢紊乱。它会影响自主神经,外围,和中枢神经系统。本研究报告28岁中国女性可逆性后部脑病综合征,可逆性脑血管收缩综合征和心肌缺血,在急性间歇性卟啉症患者中很少报道。
    Acute intermittent porphyria is a rare autosomal dominant metabolic disorder. It can affect the autonomic, peripheral, and central nervous system. The present study reports on the case of 28-year-old Chinese female patient with posterior reversible encephalopathy syndrome, reversible cerebral vasoconstriction syndrome and myocardial ischemia which have been very rarely reported in patients with acute intermittent porphyria.
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  • 文章类型: Case Reports
    可逆性后部脑病综合征(PRES)是一种以头痛等症状为特征的医学疾病,改变了意识,视觉异常,癫痫发作,和相关的放射性血管源性水肿。条件,正如它的名字所暗示的,一般是可逆的。PRES通常影响45岁左右的人群,在老年人群中很少见。
    在本报告中,作者介绍了一例83岁女性患者的病例,她因癫痫发作和视力障碍的病史到急诊科就诊.经检查,她的格拉斯哥昏迷评分(GCS)为14,并且进行了双边回合,reactive,正常的瞳孔。进行了普通计算机断层扫描(CT)头,显示枕区多个出血区与血管源性水肿相关。磁共振成像提示PRES.该个体在作者研究所接受了保守的管理,并逐渐康复。
    PRES通常出现在中年人身上,在老年人群中很少发生。早期放射学和临床评估对于及时诊断这种疾病至关重要。此外,确定诱发因素,如高血压,子痫,电解质失衡,肾功能衰竭,自身免疫性疾病,以及使用细胞毒性药物对患者进行最佳管理。
    PRES通常是可逆的,正如它的名字所暗示的。根据放射学和临床相关性保持高度怀疑,加上对基本条件的有效管理,可能有助于其潜在的可逆性。
    UNASSIGNED: Posterior reversible encephalopathy syndrome (PRES) is a medical condition characterized by symptoms such as headache, altered consciousness, visual abnormalities, seizures, and associated radiological vasogenic edema. The condition, as its name suggests, is generally reversible. PRES typically affects individuals around the age of 45 and is rarely seen in the elderly population.
    UNASSIGNED: In this report, the authors present the case of an 83-year-old woman who presented to the emergency department with a history of seizures and visual disturbances. Upon examination, her Glasgow Coma Scale (GCS) was 14, and she had bilateral round,reactive, regular pupils. A plain computed tomography (CT) head was performed, which revealed multiple hemorrhagic areas in the occipital region associated with vasogenic edema. magnetic resonance imaging was done which was suggestive of PRES. The individual underwent conservative management in the authors\' institute and gradually recovered.
    UNASSIGNED: PRES typically presents in middle-aged individuals, with infrequent occurrences in the elderly population. Early radiological and clinical assessments are crucial for the prompt diagnosis of this condition. Additionally, it is essential to identify predisposing factors such as hypertension, eclampsia, electrolyte imbalances, renal failure, autoimmune diseases, and the use of cytotoxic drugs for optimum management of the patient.
    UNASSIGNED: PRES is generally reversible, as its name implies. Maintaining a high level of suspicion based on radiological and clinical correlations, coupled with the effective management of underlying conditions, can contribute to its potential reversibility.
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  • 文章类型: Case Reports
    可逆性后部脑病综合征(PRES)主要表现为神经系统症状,在男性中更为常见,经常发生在肿瘤患者中。它也可能与肾脏疾病有关,如链球菌性肾小球肾炎,小儿高血压的常见原因。管理包括血压和癫痫治疗。在某些情况下,它可能导致不可逆和严重的并发症。早期治疗对于预防至关重要。
    在过去的六个月里,我们记录了两名患者的病例,年龄分别为15岁和10岁,均患有PRES和肾脏疾病。这些病人因全身不适而入院,头痛,恶心,呕吐,视觉障碍,和血压升高。随后,两名患者都经历了癫痫发作。只有第一位患者需要转移到儿科重症监护病房(PICU)。在两种情况下,脑磁共振成像(MRI)扫描均显示出明显的PRES病变。经过全面调查,两例病例均在急性链球菌性肾小球肾炎后诊断为PRES.
    患者在服用抗高血压和抗惊厥药物后表现出改善,以及对潜在肾脏疾病的治疗。
    UNASSIGNED: Posterior reversible encephalopathy syndrome (PRES) primarily shows neurological symptoms and is more frequent in males, often occurring in oncological patients. It can also be associated with renal conditions like post-streptococcal glomerulonephritis, a common cause of pediatric hypertension. Management involves blood pressure and seizure treatment. In some cases, it may lead to irreversible and severe complications. Early treatment is essential for prevention.
    UNASSIGNED: In the past six months, we have documented the cases of two patients, aged 15 and 10, both of whom presented with PRES and renal disease. These patients were admitted because of general malaise, headaches, nausea, vomiting, visual disturbances, and elevated blood pressure. Subsequently, both patients experienced epileptic episodes. Only the first patient required transfer to the Pediatric Intensive Care Unit (PICU). Cerebral magnetic resonance imaging (MRI) scans revealed distinct PRES lesions in both cases. Following comprehensive investigations, both cases were diagnosed with PRES in the context of acute post-streptococcal glomerulonephritis.
    UNASSIGNED: The patients showed improvement following the administration of antihypertensive and anticonvulsant medications, along with treatment for the underlying renal condition.
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  • 文章类型: Case Reports
    后部可逆性脑病综合征(PRES)是一种神经放射学综合征,临床表现为意识受损,头痛,视觉障碍,和癫痫发作,和放射学脑水肿。很少记录由输血引起的PRES病例。我们报告此病例是为了提高治疗医生对快速输血可能并发症的认识。一名29岁的男子出现多发性创伤,并处于出血性休克。他被多次输血。稍后,他被发现患有四肢瘫痪,左手手指活动很少。他的计算机断层扫描显示多个脑区脑水肿。我们认为这种情况下的病因是快速输血引起血红蛋白急性升高,从而导致PRES。输血对血流量的影响,血液粘度,内皮功能障碍导致血脑屏障功能障碍,这可能会导致PRES。
    Posterior reversible encephalopathy syndrome (PRES) is a neuroradiological syndrome, clinically present by impaired consciousness, headache, visual disturbances, and seizures, and radiologically brain edema. Cases of PRES induced by blood transfusion are rarely documented. We report this case to increase the awareness of treating physicians for the possible complications of rapid blood transfusion. A 29-year-old man presented with polytrauma and was in hemorrhagic shock. He was transfused with multiple transfusions. Later, he was found to have quadriplegia with minimal movement of fingers in the left hand. His computed tomography showed cerebral edema in multiple cerebral regions. We propose that the etiology in this case is that rapid blood transfusion induced acute rise in hemoglobin which led to PRES. The influences of blood transfusion on blood flow, blood viscosity, and endothelial dysfunction lead to blood-brain barrier dysfunction, which can result in PRES.
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  • 文章类型: Letter
    我们报告了一例成年患者的后部可逆性脑病综合征,该患者符合已证实的早期莱姆神经性贝类病的标准。
    We report a case of posterior reversible encephalopathy syndrome in an adult patient fulfilling criteria for proven early Lyme neuroborreliosis.
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  • 文章类型: Case Reports
    背景:后部可逆性脑病综合征(PRES)是一种输血相关并发症,通常预后良好,无死亡报告。PRES的病理评估也很少。
    方法:一名88岁的骨髓增生异常综合征(MDS)女性患者因压缩性骨折和慢性心力衰竭伴慢性贫血而到我院就诊。尽管她的血红蛋白水平从4.6g/dL提高到8.0g/dL,但经过6个单位的红细胞输注和利尿剂治疗后,胸腔积液明显减少,观察到认知功能和言语减少逐渐下降.PRES通过头部磁共振成像诊断。尽管接受了重症支持治疗,患者在第20天陷入昏迷,第22天去世。尽管与输血相关的PRES及其对生存的影响之间的病理生理联系尚未完全了解,尸检结果证实了PRES的诊断,并显示了早期影像学研究中未发现的多发性脑出血。
    结论:此案例突出了警惕监测和管理PRES的重要性,特别是在高危人群中,如患有多种合并症的老年患者或血小板减少症患者。需要进一步的研究来阐明血液疾病患者PRES的机制。
    BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) is known as a transfusion-related complication with typically favorable prognosis and no report fatalities. Pathological evaluation of PRES is also scarce.
    METHODS: An 88-year-old female with myelodysplastic syndromes (MDS) attended our hospital because of a compression fracture and chronic heart failure with chronic anemia. While her hemoglobin levels improved from 4.6 to 8.0 g/dL and the pleural effusions substantially decreased following six units of red blood cell transfusion and diuretic therapy, a gradual decline in cognitive function and speech reduction was noted. PRES was diagnosed by magnetic resonance imaging of the head. Despite treatment of intensive supportive care, the patient fell into a coma by the 20th day and passed away on the 22nd day. Although the pathophysiological link between blood-transfusion-related PRES and its impact on survival is not fully understood, autopsy findings confirmed the diagnosis of PRES and revealed multiple cerebral hemorrhages that were not detected in earlier imaging studies.
    CONCLUSIONS: This case highlights the importance of vigilant monitoring and management of PRES, especially in high-risk populations such as elderly patients with multiple comorbidities or those with thrombocytopenia. Further studies are needed to elucidate the mechanisms of PRES in patients with hematologic diseases.
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  • 文章类型: Case Reports
    后部可逆性脑病综合征(PRES)是与各种医疗状况相关的日益公认的临床实体。它通常被认为是不受控的,严重的高血压.然而,最近,它在输血的背景下被描述过,尤其是那些患有慢性贫血的人,即使没有严重的高血压。我们描述了一名患者在大量输血后12天到急诊科就诊,慢性贫血伴头痛,视力丧失,表现性失语症和精神状态的变化,只有轻微的血压升高,最终被诊断为PRES和难治性非惊厥性癫痫持续状态。急诊医生通常是第一个为血红蛋白低的人开始输血的人。因此,谨慎进行慢性贫血患者的输血。对于急诊医生来说,对于那些在纠正慢性贫血后出现神经系统疾病的患者,保持PRES的差异也很重要,即使没有严重的高血压。
    Posterior reversible encephalopathy syndrome (PRES) is an increasingly recognized clinical entity associated with a variety of medical conditions. It is commonly considered in the presentation of uncontrolled, severe hypertension. However, more recently, it has been described in the setting of blood transfusion, particularly in those with chronic anemia, even in the absence of severe hypertension. We describe a patient who presented to the emergency department 12 days after large blood transfusion for severe, chronic anemia with headache, vision loss, expressive aphasia and a change in mental status, with only mild blood pressure elevation, who was ultimately diagnosed with PRES and refractory non-convulsive status epilepticus. Emergency physicians are often the first to initiate blood transfusion for those with a low hemoglobin. Therefore, it is prudent to proceed with caution in transfusing those with chronic anemia. It is also important for the emergency physician to keep PRES on the differential for those presenting with a neurologic complaint after correction of their chronic anemia, even in the absence of severe hypertension.
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  • 文章类型: Case Reports
    一名4岁男孩,患有核因子-κB必需调节剂缺乏综合征,在造血干细胞移植后出现脑病。MRI显示后大脑皮层T2/FLAIR高信号,涉及后部可逆性脑病综合征。在停用可疑的药理药物(环孢菌素)后,临床改善得到了赞赏。2个月后进行的18F-FDGPET/CT筛查移植后淋巴增殖性疾病显示,后大脑皮层中FDG的摄取显着降低,涉及顶叶和枕叶。我们描述,据我们所知,首例患有临床解决的可逆性后部脑病综合征的儿童发生深度脑代谢不足。
    A 4-year-old boy with Nuclear factor-kappa B Essential Modulator deficiency syndrome presented with encephalopathy post haematopoietic stem cell transplantation. MRI demonstrated T2/FLAIR-hyperintensities in the posterior cerebral cortex concerning for posterior reversible encephalopathy syndrome. Clinical improvement was appreciated following withdrawal of the suspected offending pharmacological agent (Cyclosporine). An 18F-FDG PET/CT performed 2 months later to screen for post-transplant lymphoproliferative disease demonstrated markedly reduced FDG uptake in the posterior cerebral cortex, involving the parietal and occipital lobes. We describe, to the best of our knowledge, the first case of profound cerebral hypometabolism in a child with clinically resolved posterior reversible encephalopathy syndrome.
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  • 文章类型: Editorial
    在这篇社论中,我们讨论了一个令人信服的案例,重点是后部可逆性脑病综合征(PRES),这是接受肝移植并接受他克莫司治疗的患者的并发症。他克莫司(FK506),来自筑鹿链霉菌,是一种有效的免疫抑制大环内酯。它通过结合FK结合蛋白抑制T细胞转录,并能增强糖皮质激素和孕酮的作用。他克莫司可有效预防移植患者的同种异体移植排斥反应,但具有不利影响,例如他克莫司相关的PRES。PRES表现出各种神经症状以及血压升高,在神经影像学上主要表现为血管源性水肿。当计算机断层扫描检测到初始病变时,磁共振成像,尤其是流体衰减反转恢复序列,对诊断皮质和皮质下水肿具有优势。我们的讨论集中在实体器官移植受者中PRES的发生率,范围在0.5到5+ACU-之间,随着不同的介绍,从癫痫到视觉障碍。一名66岁男性肝移植后的病例强调了与他克莫司相关的PRES相关的诊断和管理挑战。在顶叶和枕叶放射学上明显,PRES强调需要提高医疗保健提供者的警惕。这篇社论强调了及早认识的重要性,准确诊断,和PRES的有效管理,以优化肝移植患者的预后。该病例进一步探讨了他克莫司免疫抑制疗效与其潜在神经系统风险之间的平衡,强调在这一患者人群中仔细监测和干预策略的必要性。
    In this editorial, we talk about a compelling case focusing on posterior reversible encephalopathy syndrome (PRES) as a complication in patients undergoing liver transplantation and treated with Tacrolimus. Tacrolimus (FK 506), derived from Streptomyces tsukubaensis, is a potent immunosuppressive macrolide. It inhibits T-cell transcription by binding to FK-binding protein, and is able to amplify glucocorticoid and progesterone effects. Tacrolimus effectively prevents allograft rejection in transplant patients but has adverse effects such as Tacrolimus-related PRES. PRES presents with various neurological symptoms alongside elevated blood pressure, and is primarily characterized by vasogenic edema on neuroimaging. While computed tomography detects initial lesions, magnetic resonance imaging, especially the Fluid-Attenuated Inversion Recovery sequence, is superior for diagnosing cortical and subcortical edema. Our discussion centers on the incidence of PRES in solid organ transplant recipients, which ranges between 0.5 to 5 +ACU-, with varying presentations, from seizures to visual disturbances. The case of a 66-year-old male status post liver transplantation highlights the diagnostic and management challenges associated with Tacrolimus-related PRES. Radiographically evident in the parietal and occipital lobes, PRES underlines the need for heightened vigilance among healthcare providers. This editorial emphasizes the importance of early recognition, accurate diagnosis, and effective management of PRES to optimize outcomes in liver transplant patients. The case further explores the balance between the efficacy of immunosuppression with Tacrolimus and its potential neurological risks, underlining the necessity for careful monitoring and intervention strategies in this patient population.
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  • 文章类型: Case Reports
    后部可逆性脑病综合征(PRES)是一种罕见的神经系统疾病,可能与高血压有关,自动监管失败,心脏移植(HT)后使用钙调磷酸酶抑制剂。在这篇文章中,我们提出了一系列PRES的案例,讨论其潜在原因和管理策略。在我们医院的126名HT接受者中,其中4人被诊断为PRES。这些患者中有3人在HT后7天内出现PRES。在PRES发作之前,所有患者都经历了持续性高血压,并保持严格的血压(BP)控制。四名患者中有三名康复,没有PRES复发,而一名患者在意识改变后死于败血症。在PRES发作之前,所有患者均观察到高血压,大多数患者在血压控制下症状改善。虽然大多数PRES病例在保守治疗下是可逆的,包括抗癫痫药的管理,一例不可逆病例导致院内死亡.因此,PRES可能会产生严重的结果,并且并非总是良性的。
    Posterior reversible encephalopathy syndrome (PRES) is a rare neurological disease that may be associated with hypertension, autoregulatory failure, and the use of calcineurin inhibitors following heart transplantation (HT). In this article, we present a case series of PRES, discussing its potential causes and management strategies. Among the 126 HT recipients at our hospital, four were diagnosed with PRES. Three of these patients developed PRES within 7 days after HT. Prior to the onset of PRES, all patients experienced sustained hypertension, and strict blood pressure (BP) control was maintained. Three of the four patients recovered without PRES recurrence, while one patient died of sepsis after an episode of altered consciousness. Hypertension was observed in all patients prior to the onset of PRES, and the majority experienced symptom improvement with BP control. While most cases of PRES were reversible with conservative treatment, including the administration of antiepileptics, one irreversible case resulted in in-hospital mortality. Thus, PRES can have serious outcomes and is not invariably benign.
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