Posterior Leukoencephalopathy Syndrome

后部白质脑病综合征
  • 文章类型: Case Reports
    子痫谱系障碍是一组严重的妊娠并发症,通常在妊娠20周后出现。磨牙怀孕之间有联系,一种由受精和配子生成异常引起的妊娠滋养细胞疾病,和子痫谱系障碍,可导致先兆子痫症状的表现早于妊娠20周。我们报告了一例妊娠16周时20多岁时妊娠1para0的病例,出现部分葡萄胎,患有子痫,溶血,肝酶升高和低血小板综合征和后部可逆性脑病综合征。超声检查结果与磨牙妊娠一致,病理证实磨牙部分妊娠具有三倍体69,XYY核型。该病例突出了磨牙妊娠中子痫谱系障碍的早发性潜力,同时建议对此类患者进行高血压疾病筛查。
    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.
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  • 文章类型: Journal Article
    背景:格林-巴利综合征(GBS)是一种以周围神经脱髓鞘为特征的自身免疫性疾病。GBS相关的可逆性后部脑病综合征(PRES)是儿科人群中罕见且可能危及生命的并发症。我们旨在报告和分析临床特征,管理,根据现有文献,在我们的背景下,三例GBS相关PRES的结果。
    方法:回顾了75例GBS患儿的自主神经改变和GBS相关PRES的病历。31人患有自主神经失调,而3人被确定患有PRES。临床,放射学,实验室,收集和分析治疗数据。
    结果:3例患者均为男性,表现为急性弛缓性麻痹和呼吸窘迫,需要机械通气。所有三名患者都经历了各种并发症,包括高血压,癫痫发作,低钠血症,随后被诊断为PRES。经过平均104天的护理,多模式重症监护使患者得到了改善,并在非卧床状态下出院。
    结论:GBS相关PRES是一种罕见且可能危及生命的并发症,可发生在患有GBS的儿科患者中。我们的研究结果表明,早期认识,及时干预,多模式重症监护可以改善患者预后。需要进一步的研究来确定GBS相关PRES的最佳治疗策略。
    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.
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  • 文章类型: Case Reports
    我们报告了加巴喷丁和可乐定治疗酒精戒断综合征期间发生后部可逆性脑病综合征(PRES)的病例。病人出现了严重的高血压,混乱和震颤,最终导致双侧视力丧失和癫痫发作。影像学提示后脑水肿。使用苯二氮卓类药物治疗,抗高血压药,和抗癫痫药物导致解决。一年后,成像显示了发现的分辨率。我们回顾了相关文献,并提出了对PRES子实体的识别,酒精相关的PRES(ARPRES),这可能出现在酒精戒断综合征的背景下,长期饮酒,急性酒精中毒,有或没有高血压。
    We report a case of posterior reversible encephalopathy syndrome (PRES) during treatment for alcohol withdrawal syndrome with gabapentin and clonidine. The patient developed severe hypertension, confusion and tremor, culminating in bilateral vision loss and a seizure. Imaging revealed posterior cerebral edema. Treatment with benzodiazepines, antihypertensives, and anti-seizure medications led to resolution. One year later, imaging showed resolution of the findings. We review the associated literature and propose the recognition of a PRES sub-entity, Alcohol-Related PRES (ARPRES), which can appear in the setting of alcohol withdrawal syndrome, chronic alcohol use, and acute alcohol intoxication, with or without hypertension.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    背景:脑出血(ICH)是孕产妇发病的主要原因,但是它的病理生理学特征很差。我们调查了妊娠相关ICH(P-ICH)的特征,与年龄相似的非妊娠成人的ICH相比。
    结果:我们对2012年1月1日至2021年12月31日入住我们中心的134名18至44岁非创伤性ICH患者进行了回顾性分析。我们比较了3组的ICH特征:P-ICH患者(妊娠或妊娠结束后12个月内);非妊娠妇女;和男性。我们根据改良方案对ICH发病机制进行了分类,SMASH-UP(结构性,药物,淀粉样血管病,系统性,高血压,未确定,后部可逆性脑病综合征/可逆性脑血管收缩综合征),原发性(自发性小血管)ICH与继发性ICH(结构性病变或凝血病相关)的比值比和95%CI,使用非孕妇作为参考。我们还通过SMASH-UP标准比较了具体的ICH发病机制和组间的功能结果。在134名患有非创伤性ICH的年轻人中,25(19%)患有P-ICH,其中60%发生在产后。与未怀孕的女性相比,患有P-ICH的女性患原发性ICH的几率更高(调整后的优势比,4.5[95%CI,1.4-14.7])。原发性ICH的几率在男性和未怀孕的女性之间没有差异。SMASH-UP对ICH的发病机制在组间有显著差异(P<0.001)。虽然P-ICH组的住院死亡率最低(4%),但与未怀孕的女性(13%)和男性(24%)相比,4例P-ICH患者中有1例在出院时卧床并依赖。
    结论:在我们的年轻成人ICH队列中,1/5与妊娠有关。与非妊娠相关的年轻成人ICH相比,P-ICH的发病机制不同,提示独特的病理生理学。
    BACKGROUND: Intracerebral hemorrhage (ICH) is a major cause of maternal morbidity, but its pathophysiology is poorly characterized. We investigated characteristics of pregnancy-associated ICH (P-ICH), compared with ICH in similar aged nonpregnant adults of both sexes.
    RESULTS: We performed a retrospective analysis of 134 adults aged 18 to 44 years admitted to our center with nontraumatic ICH from January 1, 2012, to December 31, 2021. We compared ICH characteristics among 3 groups: those with P-ICH (pregnant or within 12 months of end of pregnancy); nonpregnant women; and men. We categorized ICH pathogenesis according to a modified scheme, SMASH-UP (structural, medications, amyloid angiopathy, systemic, hypertension, undetermined, posterior reversible encephalopathy syndrome/reversible cerebral vasoconstriction syndrome), and calculated odds ratios and 95% CIs for primary (spontaneous small-vessel) ICH versus secondary ICH (structural lesions or coagulopathy related), using nonpregnant women as the reference. We also compared specific ICH pathogenesis by SMASH-UP criteria and functional outcomes between groups. Of 134 young adults with nontraumatic ICH, 25 (19%) had P-ICH, of which 60% occurred postpartum. Those with P-ICH had higher odds of primary ICH compared with nonpregnant women (adjusted odds ratio, 4.5 [95% CI, 1.4-14.7]). The odds of primary ICH did not differ between men and nonpregnant women. SMASH-UP pathogenesis for ICH differed significantly between groups (P<0.001). While the in-hospital mortality rate was lowest in the P-ICH group (4%) compared with nonpregnant women (13%) and men (24%), 1 in 4 patients with P-ICH were bedbound and dependent at the time of discharge.
    CONCLUSIONS: In our cohort of young adults with ICH, 1 in 5 was pregnancy related. P-ICH differed in pathogenesis compared with non-pregnancy-related ICH in young adults, suggesting unique pathophysiology.
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  • 文章类型: Journal Article
    在这项回顾性药物警戒研究中,我们收集了药物诱发的可逆性后部脑病综合征(PRES)的数据.我们的目标是通过分析食品和药物管理局不良事件报告系统(FAERS)数据库来确定PRES中的主要可疑药物。
    我们确定并分析了2004年至2021年FAERS数据库中列出的PRES报告。使用报告赔率比和95%置信区间,我们评估了与PRES相关的每种药物的安全性信号.
    我们回顾了与PRES对应的11,077份不良事件报告。主要的可疑药物类别是抗肿瘤药物,免疫抑制剂,和糖皮质激素。女性发生PRES的可能性比男性高24.77%。药物诱导的PRES通常发生在癌症患者中,那些经历过器官/干细胞移植的人,和那些有自身免疫性疾病的人。
    我们的研究结果表明,最常怀疑引起PRES的药物是抗肿瘤药物,免疫抑制剂,和糖皮质激素。需要进一步的研究来阐明PRES背后的病理生理改变。同时,处方者和患者应了解与药物治疗相关的PRES的潜在风险,个别药物的产品特征摘要应更新以包含此信息。
    UNASSIGNED: In this retrospective pharmacovigilance study, we gathered data on drug-induced posterior reversible encephalopathy syndrome (PRES). Our goal was to identify the primary suspect drugs in PRES by analyzing the Food and Drug Administration Adverse Events Reporting System (FAERS) database.
    UNASSIGNED: We identified and analyzed reports of PRES listed in the FAERS database between 2004 and 2021. Using the reporting odds ratio and 95% confidence interval, we evaluated the safety signals for each of the drugs associated with PRES.
    UNASSIGNED: We reviewed 11,077 reports of adverse events corresponding to PRES. The primary suspect drug categories were antineoplastics, immunosuppressants, and glucocorticoids. PRES was 24.77% more likely to occur in females than in males. Drug-induced PRES usually occurs in individuals with cancer, those who have undergone an organ/stem cell transplant, and those with autoimmune conditions.
    UNASSIGNED: Our results show that the drugs most commonly suspected to cause PRES were antineoplastics, immunosuppressants, and glucocorticoids. Future studies are needed to illuminate the pathophysiological alterations that underlie PRES. In the meantime, prescribers and patients should be made aware of the potential risks of PRES associated with pharmaceutical therapy, and the summaries of product characteristics for individual drugs should be updated to include this information.
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  • 文章类型: Meta-Analysis
    背景:可逆性后部脑病综合征(PRES)和可逆性脑血管收缩综合征(RCVS)可引起缺血性卒中和颅内出血。我们研究的目的是评估上述结果的频率。
    方法:我们进行了PROSPERO注册(CRD42022355704)的系统评价和荟萃分析,访问PubMed,直到2022年11月7日。纳入标准为:(1)原始出版物,(2)成年患者(≥18岁),(3)招募PRES和/或RCVS患者,(4)英语和(5)结果信息。结果是(1)缺血性卒中和(2)颅内出血的频率,分为蛛网膜下腔出血(SAH)和脑实质内出血(IPH)。使用了Cochrane偏差风险工具。
    结果:我们确定了848项研究,包括48项相关研究。摘要和全文。我们发现了11项关于RCVS(未选择的患者)的研究,报告2746例患者。在分析的患者中,15.9%(95%CI9.6%-23.4%)有缺血性卒中,22.1%(95%CI10%-39.6%)有颅内出血。另外20.3%(95%CI11.2%-31.2%)具有SAH,并且6.7%(95%CI3.6%-10.7%)具有IPH。此外,我们发现了28项关于PRES的研究(未选择的患者),报告1385名患者。在分析的患者中,11.2%(95%CI7.9%-15%)患有缺血性卒中,16.1%(95%CI12.3%-20.3%)患有颅内出血。Further,7%(95%CI4.7%-9.9%)患有SAH,9.7%(95%CI5.4%-15%)患有IPH。
    结论:颅内出血和缺血性卒中是PRES和RCVS的常见结局。个别研究中报告的频率差异很大。
    BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) may cause ischaemic stroke and intracranial haemorrhage. The aim of our study was to assess the frequency of the afore-mentioned outcomes.
    METHODS: We performed a PROSPERO-registered (CRD42022355704) systematic review and meta-analysis accessing PubMed until 7 November 2022. The inclusion criteria were: (1) original publication, (2) adult patients (≥18 years), (3) enrolling patients with PRES and/or RCVS, (4) English language and (5) outcome information. Outcomes were frequency of (1) ischaemic stroke and (2) intracranial haemorrhage, divided into subarachnoid haemorrhage (SAH) and intraparenchymal haemorrhage (IPH). The Cochrane Risk of Bias tool was used.
    RESULTS: We identified 848 studies and included 48 relevant studies after reviewing titles, abstracts and full text. We found 11 studies on RCVS (unselected patients), reporting on 2746 patients. Among the patients analysed, 15.9% (95% CI 9.6%-23.4%) had ischaemic stroke and 22.1% (95% CI 10%-39.6%) had intracranial haemorrhage. A further 20.3% (95% CI 11.2%-31.2%) had SAH and 6.7% (95% CI 3.6%-10.7%) had IPH. Furthermore, we found 28 studies on PRES (unselected patients), reporting on 1385 patients. Among the patients analysed, 11.2% (95% CI 7.9%-15%) had ischaemic stroke and 16.1% (95% CI 12.3%-20.3%) had intracranial haemorrhage. Further, 7% (95% CI 4.7%-9.9%) had SAH and 9.7% (95% CI 5.4%-15%) had IPH.
    CONCLUSIONS: Intracranial haemorrhage and ischaemic stroke are common outcomes in PRES and RCVS. The frequency reported in the individual studies varied considerably.
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  • 文章类型: Journal Article
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  • 文章类型: Letter
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  • 文章类型: Case Reports
    背景:霍奇金淋巴瘤,淋巴系统癌症,通过化疗治疗,放射治疗,和造血干细胞移植。后部可逆性脑病综合征(PRES)是一种罕见的神经毒性作用,与几种药物和全身性疾病有关。该案例研究强调了强化化疗方案的潜在风险,并假设Willis变异环对PRES半球病变异质性的影响。
    方法:一名42岁女性被诊断为IIA期结节性硬化性霍奇金淋巴瘤和慢性血小板减少症,在最初诊断6年和单倍体移植后4年后出现。她接受了计划中的异环磷酰胺化疗,卡铂,和依托泊苷。
    方法:她的精神状态发生了改变。头颈部的计算机断层扫描扫描和血管造影显示,发现与PRES和左胎型大脑后动脉一致,左脑前动脉的再生障碍性A1段。一小时后,她被发现昏迷,并伴有无核疝的临床后遗症。
    方法:随后的事件导致紧急插管,服用23.4%的高渗盐水。重复的计算机断层扫描显示右侧实质内出血,液体水平高达4.7厘米,双侧蛛网膜下腔出血,右耳疝,向左中线偏移15毫米。她紧急接受了右半开颅减压术。
    结果:脑部磁共振成像显示双侧细胞毒性水肿累及顶枕叶。尽管采取了干预措施,病人的神经状况恶化,导致在第8天宣布脑死亡。
    结论:这个案例强调了认识到严重神经系统并发症的重要性,包括PRES,与霍奇金淋巴瘤的化疗治疗有关。在这种情况下,PRES也可能因凝血病如血小板减少而加剧。Willis变异环可能会影响脑血流,自动调节,和其他血液动力学因素,导致对放射学病变负担和最差临床结局的易感性增加。
    BACKGROUND: 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.
    METHODS: 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.
    METHODS: 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.
    METHODS: 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.
    RESULTS: 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.
    CONCLUSIONS: 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.
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