Posterior Leukoencephalopathy Syndrome

后部白质脑病综合征
  • 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
    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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  • 文章类型: Case Reports
    后部可逆性脑病综合征是一种由内皮损伤引起的可逆性皮质下血管源性脑水肿引起的各种原因的紧急医学疾病。由血压变化或细胞因子对内皮的直接影响引起。后部可逆性脑病综合征表现为神经系统症状。常见原因包括高血压急症,肾脏疾病,先兆子痫,子痫,和免疫抑制药物。在这个案例报告中,1例因狼疮性肾炎而接受血液透析的17岁女性患者,此前曾接受过已死亡的供体器官移植,并在移植后早期接受了三重免疫抑制,出现后可逆性脑病综合征的神经系统症状.她血压正常,他克莫司水平处于理想水平。他克莫司停止免疫抑制后,放射学病变完全消退,她有所改善。后部可逆性脑病综合征可发生在接受他克莫司作为免疫抑制的一部分的实体器官移植受者中。
    Posterior reversible encephalopathy syndrome is an emergency medical condition with varied causes presenting as reversible subcortical vasogenic brain edema caused by endothelial injury, resulting from changes in blood pressure or direct effects of cytokines on endothelium. Posterior reversible encephalopathy syndrome is manifested by neurologic symptoms. Common causes include hypertensive emergency, renal disease, preeclampsia, eclampsia, and immunosuppressive drugs. In this case report, a 17-year-old female patient on hemodialysis as a result of lupus nephritis who had previously undergone deceased donor organ transplant and was on triple immunosuppression presented with neurological symptoms of posterior reversible encephalopathy syndrome in the early posttransplant period. She was normotensive, and tacrolimus level was in desired level. She improved after cessation of tacrolimus from immunosuppression with complete resolution of radiological lesions. Posterior reversible encephalopathy syndrome can occur in solid-organ transplant recipients who are on tacrolimus as a part of immunosuppression.
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  • 文章类型: Journal Article
    我们系统回顾了可逆性后部脑病综合征(PRES)的病例报告,并调查了日本孕妇PRES的特征以及PRES孕妇可逆性脑血管收缩综合征(RCVS)的临床相关性。使用PubMed/Medline和Ichushi-Web数据库收集文章。本文最终对121篇文献(162例患者)进行了综述。PRES的临床特点,PRES病变的个别部位,水肿类型,并检查了PRES病例中RCVS的临床特征。PRES病变最常见的个体部位是枕叶(83.3%),其次是基底神经节,顶叶,额叶,脑干,小脑,颞叶,丘脑,和call体(47.5、42.6、24.7、16.1、9.3、5.6、4.3和0.0%,分别)。79例PRES的水肿类型主要为血管源性水肿类型(91.1%)。细胞毒性水肿型(3.8%)和混合型(5.1%)的病例很少。在25例RCVS患者中,磁共振血管造影前17例(68.0%)未强烈怀疑RCVS。RCVS与PRES同时观察到13例(约50%),在其他12个PRES发作后的第1天至第14天之间。这些结果表明,基底神经节是孕妇PRES病变的常见部位。RCVS可能发生在PRES发作时或之后,即使没有症状提示RCVS。
    We systematically reviewed case reports of posterior reversible encephalopathy syndrome (PRES), and investigated the characteristics of PRES in pregnant Japanese women and the clinical relevance of reversible cerebral vasoconstriction syndrome (RCVS) in pregnant women with PRES. Articles were collected using the PubMed/Medline and Ichushi-Web databases. This review was ultimately conducted on 121 articles (162 patients). The clinical characteristics of PRES, individual sites of PRES lesions, edema types, and clinical characteristics of RCVS in PRES cases were examined. The most common individual site of PRES lesion was the occipital lobe (83.3%), followed by the basal ganglia, parietal lobe, frontal lobe, brain stem, cerebellum, temporal lobe, thalamus, and splenium corpus callosum (47.5, 42.6, 24.7, 16.1, 9.3, 5.6, 4.3, and 0.0%, respectively). Edema types in 79 cases with PRES were mainly the vasogenic edema type (91.1%), with very few cases of the cytotoxic edema type (3.8%) and mixed type (5.1%). Among 25 PRES cases with RCVS, RCVS was not strongly suspected in 17 (68.0%) before magnetic resonance angiography. RCVS was observed at the same time as PRES in 13 cases (approximately 50%), and between days 1 and 14 after the onset of PRES in the other 12. These results suggest that the basal ganglia is a frequent site of PRES lesions in pregnant women. RCVS may occur at or after the onset of PRES, even if there are no symptoms to suggest RCVS.
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  • 文章类型: Review
    背景:在2019年冠状病毒病(COVID-19)大流行的过去3年中,COVID-19已被认为会导致各种神经系统并发症,包括罕见的可逆性后部脑病综合征(PRES)。在先前报道的与COVID-19相关的PRES病例中,大多数患者患有严重的COVID-19感染和已知的PRES诱发因素,比如不受控制的高血压,肾功能不全,使用免疫抑制剂。尚不清楚这些危险因素或严重急性呼吸综合征冠状病毒2(SARS-CoV-2)的感染是否有助于这些患者的PRES的发展。在这里,我们报告了与COVID-19相关的PRES的特殊病例,没有任何已知的PRES危险因素,表明SARS-CoV-2在与COVID-19相关的PRES发病机理中的直接作用。
    方法:一名18岁女性患者因腹痛被送往急诊科就诊。初步调查显示没有异常,除了使用口咽拭子的新型冠状病毒核酸检测结果呈阳性。然而,患者随后出现强直阵挛性癫痫发作,头痛,第二天呕吐。进行了广泛的调查,包括脑部MRI和腰椎穿刺.脑MRI显示双侧枕骨低信号T1加权和高信号T2加权病变,额叶,和顶骨皮质无增强作用。血液和脑脊液分析结果为阴性。病人没有高血压,肾功能不全,自身免疫性疾病,或使用免疫抑制剂或细胞毒性药物。
    方法:根据PRES的临床特征和典型MRI表现诊断PRES。
    方法:对患者进行对症治疗,如抗惊厥药。
    结果:患者在1周内完全康复。最初的MRI异常在11天后进行的第二次MR检查中也完全消失。支持PRES的诊断。患者随访6个月,保持正常状态。
    结论:当前病例没有典型的PRES危险因素,这表明尽管COVID-19患者PRES的原因可能是多因素的,SARS-CoV-2的感染可能在COVID-19相关PRES的发病中起直接作用。
    BACKGROUND: During the past 3 years of the corona virus disease 2019 (COVID-19) pandemic, COVID-19 has been recognized to cause various neurological complications, including rare posterior reversible encephalopathy syndrome (PRES). In previously reported cases of PRES associated with COVID-19, the majority of patients had severe COVID-19 infection and known predisposing factors for PRES, such as uncontrolled hypertension, renal dysfunction, and use of immunosuppressants. It remains unclear whether these risk factors or infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) contributes to the development of PRES in these patients. Here we report a special case of PRES associated with COVID-19 without any known risk factors for PRES, indicating the SARS-CoV-2\'s direct role in the pathogenesis of PRES associated with COVID-19.
    METHODS: An 18-year-old female patient presented to the emergency department with abdominal pain. Preliminary investigations showed no abnormalities, except for positive results in novel coronavirus nucleic acid tests using oropharyngeal swabs. However, the patient subsequently developed tonic-clonic seizures, headaches, and vomiting on the second day. Extensive investigations have been performed, including brain MRI and lumbar puncture. Brain MRI showed hypointense T1-weighted and hyperintense T2-weighted lesions in the bilateral occipital, frontal, and parietal cortices without enhancement effect. Blood and cerebrospinal fluid analyses yielded negative results. The patient had no hypertension, renal insufficiency, autoimmune disease, or the use of immunosuppressants or cytotoxic drugs.
    METHODS: PRES was diagnosed based on the clinical features and typical MRI findings of PRES.
    METHODS: Symptomatic treatments such as anticonvulsants were administered to the patients.
    RESULTS: The patient fully recovered within 1 week. The initial MRI abnormalities also disappeared completely on a second MR examination performed 11 days later, supporting the diagnosis of PRES. The patient was followed up for 6 months and remained in a normal state.
    CONCLUSIONS: The current case had no classical risk factors for PRES, indicating that although the cause of PRES in COVID-19 patients may be multifactorial, the infection of SARS-CoV-2 may play a direct role in the pathogenesis of PRES associated with COVID-19.
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  • 文章类型: Review
    后部可逆性脑病综合征(PRES)是一种临床罕见的疾病,在系统性红斑狼疮(SLE)患者中已见到。其主要表现为癫痫发作,头痛和其他神经症状。虽然情况是可逆的,如果不及时治疗,可能有脑出血的风险。我们在这里报道了一个年轻的SLE患者在接受免疫抑制剂后发展为PRES的病例,霉酚酸酯。神经症状,标志,或者患者病情的变化不能用狼疮解释,应该提醒医生该药物引起PRES的可能性,并应迅速停止。
    Posterior reversible encephalopathy syndrome (PRES) is a rare clinical disease, which has been seen in patients with systemic lupus erythematosus (SLE). Its main manifestations are seizure, headache and other neurological symptoms. While the condition is reversible, if not treated in time, there can be risks of cerebral haemorrhage. We report here the case of a young patient with SLE who developed PRES after receiving the immunosuppressant, mycophenolate mofetil. Neurological symptoms, signs, or changes in a patient\'s condition that cannot be explained by lupus, should alert physicians to the possibility of the drug causing PRES, and prompt discontinuation should ensue.
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  • 文章类型: Review
    背景:遗传性肾性低尿酸血症(RHUC)是一种异质性疾病,其特征是尿酸(UA)重吸收缺陷导致低尿酸血症和UA排泄分数增加。RHUC是运动性急性肾损伤(EIAKI)的重要原因,肾结石和后部可逆性脑病综合征(PRES)。我们在这里介绍了一例RHUC患者的不寻常病例,该患者患有复发性EIAKI,并且在SLC2A9基因中有两个杂合突变。
    方法:一名43岁的男子因双侧腰痛入院,剧烈运动后3天恶心和失眠。实验室结果显示血尿素氮(BUN)(15mmol/l)和血清肌酐(Scr)(450μmol/l)水平升高,UA水平极低,为0.54mg/dl,他的尿酸盐排泄分数(FE-UA)为108%。该患者在大约20年前踢足球后发生了急性肾损伤,在常规体检中,他的UA小于0.50mg/dl。鉴于明显的低尿酸血症和高FE-UA,怀疑有RHUC的诊断,这导致我们对SLC22A12和SLC2A9基因进行突变筛选。DNA测序显示SLC22A12基因无突变,而是SLC2A9基因中的两个杂合突变。
    结论:这是一例由于SLC2A9突变导致的RHUC2患者的罕见报道。EIAKI的这种独特症状以及UA的血清浓度降低或正常,作为RHUC的早期线索,值得更多关注。
    Hereditary renal hypouricemia (RHUC) is a heterogenous disorder characterized by defective uric acid (UA) reabsorption resulting in hypouricemia and increased fractional excretion of UA. RHUC is an important cause of exercise-induced acute kidney injury (EIAKI), nephrolithiasis and posterior reversible encephalopathy syndrome (PRES). We present here an unusual case of a patient with RHUC who presented with recurrent EIAKI and had two heterozygous mutations in the SLC2A9 gene.
    A 43-year old man was admitted to our clinic because of bilateral loin pain, nausea and sleeplessness for 3 days after strenuous exercise. The laboratory results revealed increased levels of blood urea nitrogen (BUN) (15 mmol/l) and serum creatinine (Scr) (450 μmol/l), while the UA level was extremely low at 0.54 mg/dl, and his fractional excretion of urate (FE-UA) was 108%. The patient had an episode of acute kidney injury after playing soccer approximately 20 years ago, and on routine physical examination, his UA was less than 0.50 mg/dl. In view of the marked hypouricemia and high FE-UA, a diagnosis of RHUC was suspected, which led us to perform mutational screening of the SLC22A12 and SLC2A9 genes. DNA sequencing revealed no mutation in SLC22A12 gene, but two heterozygous mutations in the SLC2A9 gene.
    This is a rare report of a patient with RHUC2 due to the mutation of SLC2A9. And this unique symptom of EIAKI and decreased or normal serum concentrations of UA warrant more attention as an early cue of RHUC.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    后部可逆性脑病综合征(PRES)是一种罕见的短暂性神经放射学现象,可引起血管源性脑水肿,可由某些药物引起。如分子特异性靶试剂。Lenvatinib属于酪氨酸激酶抑制剂,并于2015年被批准用于放射性碘(I-131)治疗难治性进行性局部晚期或转移性甲状腺癌。在这里,我们介绍一个65岁的女人,在接受lenvatinib治疗放射性碘难治性转移性甲状腺乳头状癌的同时,在初始评估时发展为PRES,无高血压。退出上述治疗后,她的临床和放射学检查结果有所改善,后来有可能重新掺入较低剂量的药物,正如在全球医学文献中发现的其他三例病例报告中所描述的那样。对该实体的识别对于及时中止药物和避免更大的合并症至关重要。这是第一篇在西班牙裔人群中使用lenvatinib报告这种不良事件的论文。
    Posterior reversible encephalopathy syndrome (PRES) is an uncommon transient neuroradiological phenomenon that develops vasogenic cerebral edema and could be caused by some pharmacological agents, such as molecular-specific target agents. Lenvatinib belongs to the tyrosine kinase inhibitors and was approved in 2015 for progressive locally advanced or metastatic thyroid cancer refractory to radioactive iodine (I-131) treatment. Herein, we present the case of a 65-year-old woman who, while receiving treatment with lenvatinib for radioiodine-refractory metastatic papillary thyroid carcinoma, developed PRES without hypertension at the initial evaluation. Her clinical and radiological findings improved after withdrawing from the mentioned therapy, and later it was possible to re-incorporate lower doses of the medication, as described in the other three case reports found in the worldwide medical literature. The recognition of this entity is essential to timely suspend the drug and avoid greater comorbidity. This is the first paper reporting this kind of adverse event using lenvatinib in a Hispanic population.
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