Reversible Encephalopathy Syndrome Research Articles

IMMU-07. “STROKE MIMICS” ARE NOT BENIGN IN IMMUNOCOMPROMISED CHILDREN

ObjectiveTo determine the clinical variances between strokes and stroke mimics in a pediatric immunocompromised population that consists of children with central nervous system (CNS) and non-CNS malignancies and a history of solid organ transplantation. MethodsWe performed a retrospective cohort analysis of stroke alert activations in patients with high-grade gliomas, low-grade gliomas, atypical teratoid rhabdoid tumors, rare CNS tumors, B-cell acute lymphoblastic leukemia, T-cell acute lymphoblastic leukemia, osteosarcoma, and solid organ transplants at St. Louis Children’s Hospital between February 2013 and September 2019. We categorized final diagnoses as strokes or stroke mimics. We classified diagnoses as a neurologic emergency if the diagnosis necessitated changes in management. ResultsOut of 217 stroke alerts, 31 alerts occurred for 28 patients meeting inclusion criteria. All final diagnoses constituted neurologic emergencies, including: stroke (39%), chemotherapy-related neurotoxicity (29%), tumor progression (19%), and seizures/posterior reversible encephalopathy syndrome (13%). Patients meeting inclusion criteria with strokes and stroke mimics presented similarly, with the exception of altered mental status, which was more prevalent in patients with strokes than stroke mimics (p = 0.03). One child received hyperacute thrombectomy for stroke. Only 58% of children with stroke mimics had complete resolution of their presenting neurologic symptoms. Children with strokes and stroke mimics had similar mortality incidences of 33% and 37%, respectively. ConclusionsAlthough all acute neurologic changes in immunocompromised children are not strokes, stroke mimics in this population are neither benign nor self-limited and carry long-term neurologic morbidity and mortality. This study highlights the utility of an acute stroke evaluation infrastructure and the need for acute and long-term neurology involvement in the care of these patients.

MRI検査で診断したposterior reversible encephalopathy syndrome（PRES）妊産婦8例の検討

MRI検査で診断したposterior reversible encephalopathy syndrome（PRES）妊産婦8例の検討

Analysis of Clinical Symptoms and Brain MRI of Heat Stroke: 2 Case Reports and a Literature Review

Introduction: Heat stroke is defined as high body temperature causing multiple organ failure, psychological change, seizure, and consciousness disturbance, which lead to its high mortality rate. However, the involvement of brain injury is rare, and heat-stroke has only been reported in a few case reports or case series. The purpose of this case study was to evaluate the clinical symptoms and radiological features of heat stroke. Methods: We reviewed our hospital records and previously published reports to find cases of heat stroke. We excluded those with unknown clinical features or radiological findings. Results: We retrieved 2 cases of heat stroke from our hospital, which presented as extensive lesions on brain imaging that led to disseminated intravascular coagulation and death within a few days. In 21 previously reported cases of heat stroke, similar brain lesions were noted. These were classified as infarction/posterior reversible encephalopathy syndrome (PRES)-like lesions. The patients who developed PRES-like lesions and survived often developed cerebellar sequelae. Conclusion: The mechanism of heat stroke is presumed to be multifactorial. Ischemic-like lesions result from hypovolemia and unusual coagulation, whereas PRES-like lesions are caused by direct heat and vasogenic edema due to hypercytokinemia. We need to consider the above mentioned conditions when evaluating heat stroke.

Reversible posterior encephalopathy syndrome after allogeneic hematopoietic stem cell transplantation: report of one case and review of literature

目的 探讨单倍体异基因造血干细胞移植术后可逆性后部脑病综合征（RPES）的临床表现、诊治及预后情况。 方法 回顾性分析1例单倍体异基因造血干细胞移植术后合并RPES患者的临床及影像学资料。 结果 该患者诊断为急性淋巴细胞白血病（前B细胞型，WT1阳性），给予诱导化疗、强化治疗后获得完全缓解（CR），后原发病复发，再次完全缓解（CR2）后行单倍体异基因造血干细胞移植。移植后先后出现移植物抗宿主病、血压增高及神经系统症状，结合影像学检查诊断为RPES，经积极治疗后完全恢复，影像学表现迅速改善。 结论 RPES是造血干细胞移植后少见的严重并发症，可由多种病因产生，临床表现及影像学检查具有鲜明的特征，一般预后较好，早发现、早治疗可有效减少中枢神经系统损害。

免疫グロブリン大量静注療法中にposterior reversible encephalopathy syndrome（PRES）と脳卒中を合併したFisher 症候群の1 例

免疫グロブリン大量静注療法中にposterior reversible encephalopathy syndrome（PRES）と脳卒中を合併したFisher 症候群の1 例

Endoscopic repair of cerebrospinal fluid rhinorrhea

IntroductionNasal liquorrhea indicates a cerebrospinal fluid fistula, an open communication between the intracranial cerebrospinal fluid and the nasal cavity. It can be traumatic and spontaneous. ObjectiveThe aim of this study was to assess the outcome of endoscopic repair of cerebrospinal fluid fistula using fluorescein. MethodsThis retrospective study included 30 patients of both sexes, with a mean age of 48.7 years, treated in the period from 2007 to 2015. All patients underwent lumbar administration of 5% sodium fluorescein solution preoperatively. Fistula was closed using three-layer graft and fibrin glue. ResultsCerebrospinal fluid fistulas were commonly located in the ethmoid (37%) and sphenoid sinus (33%). Most patients presented with traumatic cerebrospinal fluid fistulas (2/3 of patients). The reported success rate for the first repair attempt was 97%. Complications occurred in three patients: one patient presented with acute hydrocephalus, one with reversible encephalopathy syndrome on the fifth postoperative day with bilateral loss of vision, and one patient was diagnosed with hydrocephalus two years after the repair of cerebrospinal fluid fistula. ConclusionEndoscopic diagnosis and repair of cerebrospinal fluid fistulas using fluorescein intrathecally has high success rate and low complication rate.

異なる画像所見,臨床経過を示したposterior reversible encephalopathy syndrome（PRES）合併妊娠の4症例

異なる画像所見,臨床経過を示したposterior reversible encephalopathy syndrome（PRES）合併妊娠の4症例

運動後急性腎不全〔acute renal failure with severe loin pain and patchy renal vasoconstriction after anaerobic exercise（ALPE）〕の認識とposterior reversible encephalopathy syndrome（PRES）の再認識

運動後急性腎不全〔acute renal failure with severe loin pain and patchy renal vasoconstriction after anaerobic exercise（ALPE）〕の認識とposterior reversible encephalopathy syndrome（PRES）の再認識

胸部大動脈瘤術後に両側の視力低下で発症したPosterior Reversible Encephalopathy Syndrome（PRES）の1症例

胸部大動脈瘤術後に両側の視力低下で発症したPosterior Reversible Encephalopathy Syndrome（PRES）の1症例

MRI BRAIN APPEARANCES IN ANAPHYLAXIS: NOVEL OBSERVATIONS TO DIFFERENTIATE FROM GLOBAL HYPOXIC INSULT

Global ‘hypoxic brain injury’ commonly presents typically after hypoperfusion, although true hypoxia may be absent. MRI is used to confirm clinical diagnosis is sought. However, a potentially different pattern of...

Tacrolimus associated to posterior reversible atypical encephalopathy syndrome and brain haemorrhage in renal transplant recipient.

Tacrolimus associated to posterior reversible atypical encephalopathy syndrome and brain haemorrhage in renal transplant recipient.

Clinical analysis of 18 cases with acute tumor lysis syndrome in children with B-cell lymphoma

To investigate risk factors associated with acute tumor lysis syndrome (ATLS) in children with B-cell lymphoma and to explore feasible means for the prophylaxis and treatment. Data from 18 children with ATLS in B-cell lymphoma were collected to assess their tumor burden at diagnosis and before chemotherapy. Evaluation was performed at the 8th day, 3 month, and the end of chemotherapy and follow up. The incidence of ATLS in B-cell lymphoma, and the relationship between the incidence of ATLS and whether the kidney was involved and large tumor burden were analyzed respectively. All patients received hydration, alkalinization and received allopurinol routinely. Urate oxidase and hemodialysis treatment were administered in some cases. Of the 103 children with B-cell lymphoma, 18 were diagnosed as having ATLS (17.5%). All the 18 cases with ATLS were histopathologically confirmed as having Burkitt's lymphoma. All the patients were at stage III or IV and all had large tumor sizes, and 7 were found to have blasts in the bone marrow>25% (38.9%). Lactate dehydrogenase (LDH) levels≥1000 U/L were found in 11 (61.1%) cases. All patients had developed metabolic abnormalities, including hyperuricemia, hyperphosphatemia, hypocalcemia, and uremia. In terms of clinical features and prognosis, all cases had nausea, vomiting, anorexia, oliguria, and anuria at different levels. One had gastrointestinal bleeding, 7 patients experienced seizures. The etiology in five was hypocalcemia and two had reversible posterior encephalopathy syndrome and all responded well to treatment. Nine cases of ATLS responded to supportive care, 4 required hemodialysis, and the other 4 responded to urate oxidase. Ten cases survived and 8 died. The major cause of death was severe complications and treatment was given up in 5 cases and recurrence occurred in 3 cases. ATLS was commonly seen in Burkitt's subtype of B-cell lymphoma. Higher LDH and large tumor sizes and kidney involvement were important risk factors for the development of ATLS in children with B-cell lymphoma. Treatments with hydration, alkalinization and allopurinol were safe and effective. Urate oxidase and hemodialytic treatments should be given timely.

Accelerated hypertension with features of reversible posterior encephalopathy syndrome

Accelerated hypertension with features of reversible posterior encephalopathy syndrome

A phase II study of VEGF trap (aflibercept) in patients with radioactive iodine-refractory, positron emission tomography (PET) positive thyroid carcinoma.

5587 Background: Historically, systemic therapy for RAI-refractory thyroid cancer has been understudied, but activity has recently been seen with multi-targeted tyrosine kinase inhibitors (TKIs) that act partly as angiogenesis inhibitors. VEGF Trap (VT) is a recombinantly- produced fusion protein consisting solely of human sequences for vascular endothelial growth factor receptor (VEGFR) extracellular domains and human immunoglobulin G1. Methods: The study was a single institution, phase II, Simon 2-stage design ([21->41 patients (pts)] based on major response rate/6 month progression-free survival as the primary endpoints. Eligible pts have progressive, RAI- refractory/fluorodeoxyglucose (18-F)-avid, recurrent/metastatic, non-medullary, non-anaplastic thyroid cancer; RECIST measurable disease; and adequate organ/marrow function. VT 4 mg/kg IV was administered every 14 days. This abstract provides results from the completed first stage of accrual. Results: 21 pts have been enrolled between 8/22/08 and 1/20/09: female-62%; median age-65 years; histology-papillary (14)/follicular (2)/Hürthle (5). Grade 4-5 adverse events at least possibly related to drug documented to date: grade 5 - none: grade 4 (one of each) – proteinuria; thrombotic microangiopathy; lymphopenia; confusion/posterior reversible encephalopathy syndrome; myocardial infarction. Evaluation of best response: complete or partial (0); stable disease (18); progression (3). 10/18 pts with stable disease remained on study for > 6 months, 3/18 pts for > 12 months; the median duration of stable disease on treatment is 178 days. Conclusions: Despite major response rates being reported with TKIs that are angiogenesis inhibitors, no responses have been seen with VT to date. However, durable stable disease is common and toxicity is manageable. Accrual to the second stage of the study continues. (Study funded by grant N01 CM 62206.) Author Disclosure Employment or Leadership Position Consultant or Advisory Role Stock Ownership Honoraria Research Funding Expert Testimony Other Remuneration sanofi-aventis

Reversible Leukoencephalopathy Syndrome

Reversible encephalopathy syndrome is a multietiological condition, the pathophysiology of which lies in the breach of the blood-brain barrier due to increased local cerebral perfusion pressure. Patients present clinically in nonspecific ways; acute computed tomography is often unrevealing, and as such, there should be a low threshold for early magnetic resonance imaging even in the acute setting because misdiagnosis may lead to mismanagement. Although initially described to present with changes depicted on imaging in the "posterior aspects" of the brain, more recent literature has focused attention on the fact that signal changes may occur anywhere in the brain. We present a case demonstrating such prolific changes throughout the brain stressing on the need for early appropriate use of radiodiagnostics.

The posterior reversible encephalopathy syndrome

The posterior/potentially reversible encephalopathy syndrome is a unique syndrome encountered commonly in hypertensive encephalopathy. A 13-year-old boy presented with of intermittent high grade fever, throbbing headache and non-projective vomiting for 5 days. The patient had a blood pressure of 120/80 mmHg but fundoscopy documented grade 3 hypertensive retinopathy. The patient improved symptomatically following conservative management. However, on the 5(th) post-admission day headache reappeared, and blood pressure measured at that time was 240/120 mmHg. Neuroimaging suggested white matter abnormalities. Search for the etiology of secondary hypertension led to the diagnosis of pheochromocytoma. Repeated MRI after successful surgical excision of the tumor patient showed reversal of white matter abnormalities. Reversible leucoencephalopathy due to pheochromocytoma have not been documented in literature previously.

シクロスポリン（ＣｙＡ）内服中に起こったｐｏｓｔｅｒｉｏｒ ｒｅｖｅｒｓｉｂｌｅ ｅｎｃｅｐｈａｌｏｐａｔｈｙ ｓｙｎｄｒｏｍｅ（ＰＲＥＳ）の１例

近年シクロスポリン (CyA) は，難治性ネフローゼ症候群へ広く用いられているが，一方でさまざまな副作用が報告されており，Posterior reversibel encephalopathy syndrome (PRES) は，CyAの注目すべき副作用のうちの一つである。 今回，我々はCyAの投与によりPRESを発症したネフローゼ症候群の一男児例を経験した。症例は9歳5ヵ月の頻回再発型ネフローゼ症候群の男児で，6回目の再発に際し経口ステロイドにCyAを併用した。CyA投与後11日目に頭痛，嘔吐，痙攣発作を来たし，当科に入院したが臨床症状は発症当日のうちに無治療で消失した。CyAによる神経障害を考え，同薬剤を中止した。入院翌日に施行した頭部MRIで右頭頂葉から後頭葉に異常所見を認めたが21日後には消失し，PRESと診断した。本症例では，拡張期の高血圧と血清マグネシウムの低下が確認され，PRES発症への関与が考えられた。