4.Clinical characteristics analysis of children with reversible posterior leukoen-cephalopathy syndrome during the treatment of hematological tumor.
Peng Hui WU ; Yao XIE ; Wei Hong ZHAO ; Ying HUA ; Qing SUN ; Shuo LI ; Ye WU ; Xin Tian LU
Journal of Peking University(Health Sciences) 2018;50(4):662-665
OBJECTIVE:
To analyze the clinical characteristics of patients with hematological tumor or disease before and after reversible posterior leukoen-cephalopathy syndrome (RPLS).
METHODS:
Five patients were both from Peking University First Hospital Pediatric Hematology-oncology Department in the period from March 2012 to March 2017. The gender, age, BMI, underlying diseases, with or without renal damage, hypertension family history, clinical manifestations of convulsions, hemoglobin, and blood pressure, serum sodium levels before and after convulsion, and other data of the children with RPLS were retrospectively analyzed. In the meantime, we followed up the five patients for 6 months to 66 months, kept a watchful eye on their original condition and the recovery of symptoms and signs of the nervous system. The relevant literature was reviewed.
RESULTS:
All of the subjects were females in school-age or pre-school age. The underlying diseases were malignant tumor associated with renal involvement or on one side of nephrectomy in 4 of these subjects, while the other one was refractory autoimmune hemolytic anemia. All of the subjects suffered from mild or moderate anemia. The day before RPLS occurred they received chemotherapy made up with cyclophosphamide, vincristine, and actinomycin-D, or the therapy with cyclosporin A and glucocorticoid. The clinical manifestations were afebrile convulsion after getting up in the mooring or in the afternoon. We observed elevation of blood pressure and cutting down of serum sodium compared with themselves. All of the cases recovered soon after management with diazepam, furosemide and amlodipine besylate. Four of them had a good outcomes and did not remain any sequela, while only one girl became childish in emotion and behavior, and then returned gradually to normal two years later. However, by long-term follow-up, the elevation of blood pressure was mainly reviewed in literature.
CONCLUSION
The patients attacked by RPLS, with hematology or oncology cases, could have the underlying disease of renal damage and anemia. Blood pressure elevation and serum sodium falling down at the same time may play an important role during the occurrence of RPLS. Remaining stable of blood pressure and electrolyte level together will possibly reduce or mitigate RPLS.
Blood Pressure
;
Child
;
Child, Preschool
;
Female
;
Hematologic Neoplasms/complications*
;
Humans
;
Hypertension/etiology*
;
Posterior Leukoencephalopathy Syndrome/therapy*
;
Retrospective Studies
;
Seizures/etiology*
5.Posterior reversible encephalopathy syndrome in systemic lupus erythematosus: a case report.
Xiao Hui ZHANG ; Xue Rong DENG ; Fan LI ; Ying ZHU ; Zhuo Li ZHANG
Journal of Peking University(Health Sciences) 2018;50(6):1102-1107
This case report concerns a 22-year-old woman who had been diagnosed with systemic lupus erythematosus (SLE). She had intermittent fever, butterfly erythema, photosensitivity, oral ulcers, and multiple arthralgia in the past seven years, but she did not adhere to regular treatments. The edema of the lower extremities and face aggravated in the recent two weeks, so she was admitted to our Department of Rheumatology and Clinical Immunology. Meanwhile, we found she had severe hypertension, the maximal blood pressure was 170/120 mmHg. The patient had high SLE disease activity (the disease activity index score was as high as 23) with blood involvement, acute renal insufficiency, multiple serous effusion and rash. After one week treatments of intravenous methylprednisolone 80 mg daily and other drugs, her conditions made some extent improvement. However, she suffered sudden epileptic attacks. No positive neuropathological signs were found, and the blood pressure was up to 190/130 mmHg before the onset of the seizures. Her cerebrospinal fluid (CSF) pressure was 330 mmH2O, the CSF protein level was normal value, and the white blood cell count was 0 cell/mm3, with no signs of infection. Cranial MRI showed vasogenic edema at bilateral parietal, occipito-parietal regions, and centrum ovale. We prescribed drugs of decreasing intracranial pressure, intravenous drugs of decreasing blood pressure and midazolam for sedation, without corticosteroid impulse therapy. She recovered consciousness in the next day, without epilepsy recurrence. We eventually diagnosed it as posterior reversible encephalopathy syndrome (PRES), according to the history, laboratory results, imaging featuresand clinical outcome. PRES is a disorder of reversible subcortical vasogenic brain edema in patients with acute neurological symptoms (eg, seizures, encephalopathy, headache, and visual disturbances). PRES is mainly caused by blood pressure changes or endothelial injury, which lead to breakdown of the blood-brain barrier and subsequent brain edema. Most patients have a favourable prognosis. SLE complicated with PRES is not rare, especially in patients with disease activity, hypertension, lupus nephritis and/or renal insufficiency, and use of cytotoxic drugs, early recognition and appropriate treatment remain important. Brainstem involvement, intracranial hemorrhage, renal insufficiency and high disease activity of lupus are risk factors for poor prognosis.
Female
;
Humans
;
Lupus Erythematosus, Systemic/complications*
;
Lupus Nephritis
;
Magnetic Resonance Imaging
;
Posterior Leukoencephalopathy Syndrome/etiology*
;
Seizures
;
Young Adult
6.Posterior reversible encephalopathy syndrome: an acute manifestation of systemic lupus erythematous.
Dexter Yak Seng CHAN ; Yin Sheng ONG
Singapore medical journal 2013;54(9):e193-5
Stroke mimickers are common, and they represent a diagnostic dilemma for clinicians. Many, like posterior reversible encephalopathy syndrome (PRES), are easily reversible. The manifestation of PRES is characterised by headaches, convulsions, altered mental functioning and blindness. In most cases, computed tomography of the brain will show hypodense lesions in the parieto-occpitial lobe, which only further confounds the physician. Although this syndrome is uncommon, prompt and accurate recognition allows early treatment, which has been shown to produce favourable outcomes. Herein, we report the case of a 54-year-old woman, who presented with PRES, as an acute manifestation of systemic lupus erythematous (SLE) and lupus nephritis. The patient was initially thought to be experiencing an ischaemic stroke, but the diagnosis was later changed. On management of her underlying condition, her symptoms resolved. PRES should be recognised as an acute emergency manifestation of SLE. It should not be mistaken for an ischaemic stroke as inappropriate treatment could have adverse outcomes.
Acute Disease
;
Biopsy
;
Diagnosis, Differential
;
Female
;
Humans
;
Lupus Erythematosus, Systemic
;
complications
;
diagnosis
;
Middle Aged
;
Posterior Leukoencephalopathy Syndrome
;
diagnosis
;
etiology
;
Tomography, X-Ray Computed
7.Cerebrovascular complications during pregnancy and postpartum
Journal of Neurocritical Care 2019;12(1):20-29
Cerebrovascular complications that more likely to occur during pregnancy or postpartum include cerebral venous thrombosis, ischemic and hemorrhagic stroke, posterior reversible encephalopathy syndrome, and postpartum angiopathy, which although rare, can lead to serious outcomes for both mother and fetus. Pregnancy is a very unique condition, especially, in terms of treatment and, as such, neurointensivists must always consider its potential effects on the fetus. The purpose of this review is to help understand the physiological changes during pregnancy and postpartum, and to inform treatment decisions regarding pregnancy-related cerebrovascular complications.
Critical Care
;
Female
;
Fetus
;
Humans
;
Hypertension, Pregnancy-Induced
;
Mothers
;
Posterior Leukoencephalopathy Syndrome
;
Postpartum Period
;
Pregnancy Complications
;
Pregnancy
;
Stroke
;
Venous Thromboembolism
;
Venous Thrombosis
8.Clinical and imaging features of acute encephalopathy with biphasic seizures and late reduced diffusion in children.
Mei Jiao ZHANG ; Li LIN ; Wen Hui WANG ; Wen Hui LI ; Cui Jie WEI ; Han XIE ; Qing Ping ZHANG ; Ye WU ; Hui XIONG ; Shui Zhen ZHOU ; Bin YANG ; Xin Hua BAO
Chinese Journal of Pediatrics 2023;61(11):989-994
Objective: To explore the clinical and imaging features of acute encephalopathy with biphasic seizures and late reduced diffusion(AESD) in children. Methods: For the case series study, 21 children with AESD from Peking University First Hospital, Provincial Children's Hospital Affiliated to Anhui Medical University, Children's Hospital of Fudan University, and Shanxi Children's Hospital who were diagnosed and treated from October 2021 to July 2023 were selected. Clinical data were collected to summarize their clinical information, imaging, and laboratory tests, as well as treatment and prognostic characteristics. Descriptive statistical analysis was applicated. Results: Of the 21 cases with AESD, 11 were males and 10 were females, with the age of onset of 2 years and 6 months (1 year and 7 months, 3 years and 6 months). Of the 21 cases, 18 were typical cases with biphasic seizures. All typical cases had early seizures within 24 hours before or after fever onset. Among them, 16 cases had generalized seizures, 2 cases had focal seizures, and 7 cases reached the status epilepticus. Of the 21 cases, 3 atypical cases had late seizures in biphasic only. The late seizures in the 21 cases occurred on days 3 to 9. The types of late seizures included focal seizures in 12 cases, generalized seizures in 6 cases, and both focal and generalized seizures in 3 cases. Diffusion-weighted imaging (DWI) test on days 3 to 11 showed reduced diffusion of subcortical white matter which was named "bright tree sign" in all cases. The diffuse cerebral atrophy predominantly presented in the front-parietal-temporal lobes was found in 19 cases between day 12 and 3 months after the onset of the disease. Among 21 cases, 20 had been misdiagnosed as autoimmune encephalitis, central nervous system infection, febrile convulsions, posterior reversible encephalopathy syndrome, acute disseminated encephalomyelitis, and hemiconvulsion-hemiplegia-epilepsy syndrome. All the cases received high-dose gammaglobulin and methylprednisolone pulse therapy with poor therapeutic effect. By July 2023, 18 cases were under follow-up. Among them, 17 cases were left with varying degrees of neurologic sequelae, including 11 cases with post-encephalopathic epilepsy; 1 recovered completely. Conclusions: AESD is characterized by biphasic seizures clinically and "bright tree sign" on DWI images. Symptomatic and supportive treatments are recommended. The immunotherapy is ineffective. The prognosis of AESD is poor, with a high incidence of neurological sequelae and a low mortality.
Male
;
Female
;
Child
;
Humans
;
Infant
;
Child, Preschool
;
Posterior Leukoencephalopathy Syndrome/complications*
;
Seizures/etiology*
;
Brain Diseases/diagnostic imaging*
;
Status Epilepticus
;
Seizures, Febrile/diagnostic imaging*
9.A case of Guillain-Barre syndrome complicated with posterior reversible encephalopathy syndrome.
Ying YANG ; Jian-hua FENG ; Yu-wen DAI
Chinese Journal of Pediatrics 2013;51(6):477-478
Biomarkers
;
blood
;
Brain
;
diagnostic imaging
;
pathology
;
Brain Edema
;
etiology
;
pathology
;
Child
;
Diagnosis, Differential
;
Electroencephalography
;
Guillain-Barre Syndrome
;
complications
;
diagnosis
;
therapy
;
Humans
;
Magnetic Resonance Imaging
;
Male
;
Posterior Leukoencephalopathy Syndrome
;
complications
;
diagnosis
;
therapy
;
Radiography
10.Reversible posterior leukoencephalopathy syndrome in children with nephrotic syndrome: a case report.
Sheng-da LIU ; Qing-min SHEN ; Chun-feng LV
Chinese Medical Sciences Journal 2014;29(1):55-57
REVERSIBLE posterior leukoencephalopathy syndrome (RPLS) is a rare neurological syndrome characterized by headache, altered mental status, seizures, and visual disturbance, associated with reversible white matter changes.1 It has been commonly reported in patients with severe hypertension and pre-eclampsia. Here we report a case with nephrotic syndrome complicated by RPLS.
Brain
;
diagnostic imaging
;
drug effects
;
pathology
;
Child
;
Female
;
Humans
;
Magnetic Resonance Imaging
;
Nephrotic Syndrome
;
complications
;
diagnosis
;
drug therapy
;
Posterior Leukoencephalopathy Syndrome
;
complications
;
diagnosis
;
drug therapy
;
Tomography, X-Ray Computed
;
Treatment Outcome