Posterior reversible encephalopathy syndrome-an under recognized manifestation of Chronic Kidney Disease

First Report of the Indian Chronic Kidney Disease (CKD) Registry showed that patient with CKD in India more frequently presented in Stage V. Many patients have uncontrolled hypertension and uremia due to noncompliance or non affordability of renal replacement therapy which can lead to complications such as posterior reversible encephalopathy syndrome (PRES). A 17-year-old female had end stage renal disease (ESRD) due to malignant hypertension. She developed headaches, altered consciousness, visual disturbances and seizures. A non contrast brain computed tomography revealed bilateral symmetrical white matter hypodensity in parietal, temporal, and occipital region suggesting PRES. Intravenous lorazepam was given for acute control of seizure and she required phenytoin, valproic acid, levetiracetam to control seizures. She was started on more frequent hemodialysis. She required five types of antihypertensive drugs to control blood pressure. We report successful renal transplantation (RTx) in a CKD patient with PRES. Constraints in operating an effective maintenance dialysis program leave RTx as the only viable option for ESRD patients in our country to prevent complications like PRES associated with uremia and uncontrolled hypertension     

Posterior reversible encephalopathy syndrome in a child with steroid-resistant nephrotic syndrome: a case report and review of literature

Posterior reversible encephalopathy syndrome (PRES) is a rare and serious syndrome of central nervous system that can develop in both adults and children. It is characterized by acute onset of headache, confusion, seizures or focal neurological deficits along with radiological findings of white matter abnormalities in the parietal and occipital lobes. In the past ten years, this syndrome has been described mainly in adults, rare in children. Here, we report a case of PRES presenting in a 12-year-old girl with steroid-resistant nephrotic syndrome. Her neurological symptom was rapidly recovered after control of hypertension without discontinuation of cyclosporine A.     

Differential serum cytokine profile in patients with systemic lupus erythematosus and posterior reversible encephalopathy syndrome

Systemic lupus erythematosus (SLE) patients are susceptible to the development of posterior reversible encephalopathy syndrome (PRES). The main theory concerning the physiopathology of PRES suggests that there is brain–blood barrier damage, which is associated with endothelial dysfunction, and characterized by vasogenic oedema. However, current evidence regarding its physiopathogenic mechanisms is quite scant. The aim of this study was to analyse the expression of different serum cytokines, as well as vascular endothelial growth factor (VEGF) and soluble CD40 ligand (sCD40L), in patients with PRES/systemic lupus erythematosus (SLE) and to compare them with levels in SLE patients without PRES and in healthy controls. We performed a transversal study in a tertiary care centre in México City. We included 32 subjects (healthy controls, n = 6; remission SLE, n = 6; active SLE, n = 6 and PRES/SLE patients, n = 14). PRES was defined as reversible neurological manifestations (seizures, visual abnormalities, acute confusional state), associated with compatible changes by magnetic resonance imaging (MRI). Serum samples were obtained during the first 36 h after the PRES episode and were analysed by cytometric bead array, Luminex multiplex assay or enzyme‐linked immunosorbent assay (ELISA). Interleukin (IL)‐6 and IL‐10 levels were significantly higher in PRES/SLE patients (P = 0·013 and 0·025, respectively) when compared to the other groups. Furthermore, IL‐6 and IL‐10 levels displayed a positive correlation (r = 0·686, P = 0·007). There were no differences among groups regarding other cytokines, sCD40L or VEGF levels. A differential serum cytokine profile was found in PRES/SLE patients, with increased IL‐6 and IL‐10 levels. Our findings, which are similar to those described in other neurological manifestations of SLE, support the fact that PRES should be considered among the SLE‐associated neuropsychiatric syndromes.     

Postpartum amaurosis in a woman with severe preeclampsia

The maternal and perinatal fetal prognosis of preeclampsia depends on the gestational age of the fetus at onset, the severity of the disease, the quality of care, and the presence of pre-existent medical conditions. One of the uncommon effects of severe preeclampsia on the eye is sudden loss of vision. The present case report is of a woman with severe preeclampsia exacerbated by delivery that coursed with difficult-to-control arterial hypertension and reversible cortical amaurosis without impaired consciousness or seizures.     

Neem oil poisoning: Case report of an adult with toxic encephalopathy

Neem oil has widespread use in Indian subcontinent due to its many bioactive properties. Azadirachtin, an active ingredient, is implicated in causing the effects seen in neem oil poisoning. Neem oil poisoning is rare in adults. This report highlights the toxicity associated with neem oil poisoning in an elderly male. The patient presented with vomiting, seizures, metabolic acidosis, and toxic encephalopathy. The patient recovered completely with symptomatic treatment.     

一氧化碳中毒引发迟发性脑病机制的研究进展

机体在短时间内吸入了高浓度的一氧化碳毒性气体即可导致急性一氧化碳中毒,它是冬季较为常见的中毒性疾病之一,尤其在我国的北方地区发病率较高.此病在临床上的致残致死率极高,是急诊科常见的急危重症.患者多在经历一定时间的“假愈期”后发生一氧化碳中毒迟发性脑病,并且无法完全治愈,从而给社会和家庭造成严重的经济负担.但直到现在,一氧化碳中毒迟发性脑病的具体发病机制仍未完全阐明,并存在着多种学说.在此对一氧化碳中毒迟发性脑病发病机制的研究进行探讨,以期为其临床治疗提供新的思路及理论基础.     

Posterior Reversible Encephalopathy Syndrome: An Expanding Phenotype

How to cite this article: Udani V. Posterior Reversible Encephalopathy Syndrome: An Expanding Phenotype. Indian J Crit Care Med 2020;24(12):1163–1164.     

Recurrent posterior reversible encephalopathy syndrome in a sickle cell patient

Posterior reversible encephalopathy syndrome (PRES) in sickle cell patients has been rarely reported previously in the setting of severe crisis or uncontrolled hypertension. Here, we report a rare and unusual case of recurrent PRES in a young adult sickle cell patient without any obvious precipitating factors.     

Posterior reversible encephalopathy syndrome in the setting of COPD: Proposed pathogenesis

Posterior reversible encephalopathy syndrome (PRES) has been associated with many conditions – particularly inflammatory, neoplastic and following organ failure. We submit that Chronic Obstructive Pulmonary Disease (COPD) is a significant predisposing factor for a number of these cases. Increased levels of circulating TNF-alpha, IL-1 and endothelin-1 (ET-1) are herein proposed as key mediators of this association. This theory builds on the central role of endothelial dysfunction in the pathogenesis of PRES.     

Posterior reversible encephalopathy syndrome: A neuropsychiatric manifestation of systemic lupus erythematosus

Posterior Reversible Encephalopathy Syndrome (PRES) is an acute neurological syndrome clinically characterized by seizures, altered mental status, headache, and visual disturbances. It is caused by a variety of abnormalities in the endothelial function that ultimately result in vasogenic edema in the circulation of the central nervous system. This is reflected by the neuroimaging findings, that most often show reversible parieto-occipital edema. An important proportion of patients with PRES present with Systemic Lupus Erythematosus (SLE), and its complications, as their sole risk factors. This review describes the relationship between these two clinical entities and explains the pathophysiological models that have been proposed to describe the development of PRES. We explain how SLE can cause alterations in every pathway implicated in the development of PRES. Given the relatively high frequency and the distinct clinical course, PRES in the setting of SLE might be best described as a distinct neuropsychiatric syndrome associated with SLE.     

长托宁治疗有机磷杀虫剂中毒减少反跳等并发症的临床分析

目的探讨长托宁在治疗有机磷杀虫剂中毒时对减少猝死等并发症发生的临床价值。方法65例有机磷杀虫剂中毒患者用阿托品救治,68例有机磷杀虫剂中毒患者用长托宁救治,观察比较其并发症发生率的差异。结果长托宁组患者中毒症状恢复时间、胆碱酯酶活性恢复时间明显短于阿托品组,心律失常的发生率明显低于阿托品组,长托宁组"反跳、中间综合征"发生率低于阿托品组。结论抢救有机磷农药中毒时,使用长托宁可以大大减少并发症的发生率。     

Serum creatine phosphokinase as predictor of intermediate syndrome in organophosphorus poisoning

Background:

Organophosphorus (OP) compounds are commonly used pesticides. In OP poisoning, intermediate syndrome (IMS) manifests between the end of the acute cholinergic crisis and delayed neuropathy. Respiratory paralysis in IMS, if identified early can reduce the need for ventilator support, morbidity, and mortality. Serum creatine phosphokinase (CPK) is elevated in IMS. The objectives of our study were to measure serum CPK level, correlate CPK levels with severity of poisoning and estimate atropine dose used.

Materials and Methods:

A prospective, observational study was conducted for 1-year. Patients diagnosed with OP poisoning were included. Demographic characteristics, type of poison, time since poisoning, Peradeniya Organophosphorus Poisoning (POP) score, serum pseudocholinesterase, CPK levels, atropine dose, and outcome of treatment were documented.

Results:

Seventy-five patients were recruited of which 57% and 43% were males and females, respectively, with a mean age of 31.48 ± 11.76 years. The most common OP compound was chlorpyriphos followed by triazophos and methylparathion. The time required to reach hospital was 181.26 ± 89.53 min. About 73.3% and 26.7% of patients had mild and moderate poisoning, respectively, as per POP scale. Pseudocholinesterase level was 364 (205–2168) IU. The amount of atropine used was 190.66 ± 78.83 mg. Serial serum CPK values were 279.72 ± 350.21 IU, 389.78 ± 376.33 IU and 163.13 ± 155.15 IU at admission, 48 h, and 96 h after admission, respectively. A weak positive correlation between serum CPK levels and severity of poisoning (r = 0.352) was observed. All patients recovered completely within 10.69 ± 5.57 days. Three patients developed IMS, and their serial CPK levels were 1837.33 ± 243.19 IU/L, 1935 ± 97.41 IU/L, and 714.66 ± 394.82 IU/L; and recovered in 17 ± 5.6 days.

Conclusion:

Increased serum CPK levels at 48 h after poisoning was observed in all the patients, but three patients had more than 1500 IU/L, who manifested with IMS. Early diagnosis of IMS by serial estimation of CPK may help in timely intervention and reduce further life-threatening complications.     

血清白细胞介素-6、白细胞介素-17和基质金属蛋白酶9水平与一氧化碳中毒迟发性脑病的关系

目的 探讨白细胞介素(IL)-6)、IL-17和基质金属蛋白酶9(MMP9)与一氧化碳中毒迟发性脑病(DEACMP)的关系.方法 随机抽取58例体检健康人群为对照组,抽取71例DEACMP患者为实验组,采用酶联免疫吸附试验(ELISA)检测DEACMP患者血清中IL-6、IL-17和MMP9的表达水平.结果 和对照组相比,轻重度DEACMP患者血清中IL-6和MMP9表达水平升高(F=22.47、F=36.51,P＜0.05),其中在重度DEACMP患者血清中升高最显著;IL-17表达水平未有显著变化,差异无统计学意义(F=19.62,P＞0.05).Pearson相关分析显示,DEACMP患者血清IL-6和MMP9浓度呈正相关(r=0.58,P＜0.05).结论 DEACMP患者血清中IL-6和MMP9的表达水平与一氧化碳中毒的程度和预后密切相关.     

丁苯酞及针刺联合高压氧治疗急性一氧化碳中毒后迟发性脑病的疗效观察

目的探讨丁苯酞及针刺联合高压氧治疗急性一氧化碳中毒后迟发性脑病的临床疗效。方法将符合标准的120例患者随机分为研究组和对照组,每组60例。研究组在高压氧等常规治疗的基础上加用丁苯酞及针刺治疗,对照组给予高压氧等常规治疗。观察和比较两组治疗效果及治疗前后MMSE评分、脑电图变化。结果研究组简易智能精神量表评分及脑电图与对照组比较差异有统计学意义（P〈0．05）,研究组能明显改善MMSE评分且脑电图异常率比对照组低;研究组在痊愈率和总有效率方面显著优于对照组,差异有统计学意义（P〈0．05）。结论丁苯酞及针刺联合高压氧治疗急性一氧化碳中毒后迟发性脑病可以提高患者的治疗效果和生存质量,有良好的临床应用价值。     

MECP2 mutation in non-fatal, non-progressive encephalopathy in a male

To study the clinical overlap between Rett (RTT) and Angelman syndromes (AS), we screened the MECP2 gene in a cohort of 78 patients diagnosed as possible AS but who showed a normal methylation pattern at the UBE3A locus. MECP2 missense (R106W, G428S), nonsense (R255X, R270X), and frameshift mutations (803 delG) were identified in 6/78 patients including 4/6 female cases consistent with RTT, one female case with progressive encephalopathy of neonatal onset, and one isolated male case with non-fatal, non-progressive encephalopathy of neonatal onset. This study shows that MECP2 mutations can account for a broad spectrum of clinical presentations and raises the difficult issue of the screening of the MECP2 gene in severe encephalopathy in both males and females.


Keywords: MECP2 gene; Rett syndrome; Angelman syndrome; encephalopathy     

Posterior fossa midline cryptococcoma in a patient with idiopathic CD4 lymphocytopenia

Idiopathic CD4 lymphocytopenia (ICL) is a rare disorder which is often diagnosed as HIV-negative AIDS in the light of poor immunity and AIDS-defining illnesses. We present a case of a 50-year-old male who presented with a midline posterior fossa tumour with ICL diagnosed as cerebellar cryptococcoma.     

Behenoyl cytarabine-associated reversible encephalopathy in a patient with acute myelogenous leukemia

We report a case of reversible encephalopathy syndrome in a 16-year-old girl with acute myelogenous leukemia (AML), who is undergoing during consolidation chemotherapy composed of BH-AC (N4-behenoyl-1-beta-D-arabinofuranosyl cytosine) and idarubicin. On the 6th day of chemotherapy, she was in a drowsy state following generalized tonic clonic seizure lasting 20 minutes. MR images revealed extensive cortical and subcortical white matter brain edema. Alertness returned over the 24 hr following by the discontinuation of BH-AC and intravenous administration of diphenylhydantoin, although she complained of intermittent headaches and visual disturbance. She gradually recovered from these symptoms during subsequent 7 days. Previously noted abnormal signal intensities have nearly disappreared on follow-up MRI obtained on the 22nd day after the first seizure. She was discharged without any neurologic sequela. This case suggests that BH-AC, a derivative of cytosine arabinoside (1-beta-D-arabinofuranosylcytosine) could be a cause of reversible encephalopathy syndrome.