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*

OBJECTIVES: Reversible posterior leukoencephalopathy syndrome (RPLS) is a rare clinical imaging syndrome. The causes of RPLS are complex and diverse, the pathogenesis is not yet clear. The onset is urgent and the onset age span is large, ranging from children to the elderly. The clinical symptoms of RPLS have no significant specificity, which can be manifested as headache, blurred vision, disturbance of consciousness or seizures. Clinicians have little knowledge on the disease, which may lead to misdiagnosis or missed diagnosis. This study aims to analyze and summarize the MRI changes and clinical characteristics regarding RPLS patients, so as to provide basis for rapid diagnosis and timely intervention for this disease. METHODS: The clinical data and complete imaging data of 77 patients with RPLS diagnosed in Xiangya Hospital of Central South University from January 2012 to March 2021 were retrospectively collected. The main image data include T1 weighted imaging (T1WI), T2 weighted imaging (T2WI), T2 liquid attenuation inversion recovery (T2-FLAIR), diffusion weighted imaging (DWI) (b value=1 000×10-6 mm2/s), and apparent diffusion coefficient (ADC). The case group included 63 patients who underwent DWI examination, and 71 normal controls matched in age and sex. The characteristics of patients' magnetic resonance signals and the ADC value of 19 regions of interest (ROI) were analyzed. The differences in bilateral ADC value in the case group, the difference of ADC value between the case group and the normal control group, and the difference of ADC value in the case group before and after treatment were compared. RESULTS: Compared with the normal control group, the ADC value of the right frontal lobe, bilateral parietal lobe, bilateral thalamus, bilateral head of caudate nucleus, left lenticular nucleus, right internal capsule, bilateral temporal lobe and pons in the case group were significantly higher (all P<0.01). There was no significant difference in ADC value of bilateral sides of the case group and before and after treatment in the case group (all P>0.01). The lesions of RPLS were widely distributed and multiple, usually high signal in the posterior parieto temporo occipital lobe or pons of the brain, and involved the cortex and subcortical white matter. Most of them were bilateral, but not completely symmetrical. CONCLUSIONS The imaging manifestations of RPLS and the occurrence and development of clinical symptoms are basically synchronous. The imaging manifestations are specific. Magnetic resonance imaging can show the range of involvement of RPLS. ADC value can provide information on the severity of the disease and predict the prognosis. There are few reversible diseases. It is very important to fully understand and timely diagnose the disease.
Child ; Humans ; Aged ; Posterior Leukoencephalopathy Syndrome/etiology* ; Retrospective Studies ; Magnetic Resonance Imaging/methods* ; Brain/pathology* ; Diffusion Magnetic Resonance Imaging

Posterior reversible encephalopathy syndrome (PRES) is a newly described adverse effect possibly associated with gonadotropin-releasing hormone (GnRH) agonist therapy. We report a case of PRES after 2 doses of depot GnRH agonists in a 44-year-old woman with a huge myoma uteri and iron-deficiency anemia. Brain magnetic resonance imaging showed high signal lesions in both occipital lobes on fluid-attenuated inversion-recovery (FLAIR) images, compatible with PRES. After treatment with anticonvulsant, she recovered both radiographically and clinically. The association between PRES and GnRH agonist use is still enigmatic, and thus should be further clarified.
Adult ; Anemia, Iron-Deficiency ; Brain ; Brain Diseases ; Female ; Gonadotropin-Releasing Hormone* ; Humans ; Leuprolide ; Magnetic Resonance Imaging ; Myoma ; Occipital Lobe ; Posterior Leukoencephalopathy Syndrome* ; Uterus

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

Patients with severe active lupus nephritis (LN) require immunosuppressive therapy to induce remission. However, the development of profound hypogammaglobulinemia causing cytomegalovirus (CMV) disease is a rare occurrence during standard immunotherapy. A 27-year-old woman who presented with active LN along with moderate renal impairment was treated with of mycophenolate mofetil (MMF) and methylprednisolone. MMF was soon switched with low-dose intravenous (IV) cyclophosphamide (CYC) owing to the development of posterior reversible encephalopathy syndrome and deterioration of renal function requiring hemodialysis. After two cycles of IV CYC, she developed CMV colitis and pneumonia. Although her serum immunoglobulin (Ig) concentrations before receiving immunosuppressive treatment were normal, they were profoundly reduced at CMV disease onset and continued to maintain low level for 30 months. Severe hypogammaglobulinemia can occur during standard therapy for LN, especially in patients with impaired renal function, pointing out the importance of close monitoring of Ig levels and CMV infection.
Adult ; Agammaglobulinemia ; Colitis ; Cyclophosphamide ; Cytomegalovirus Infections ; Cytomegalovirus ; Female ; Humans ; Immunoglobulins ; Immunotherapy ; Lupus Nephritis ; Methylprednisolone ; Pneumonia ; Posterior Leukoencephalopathy Syndrome ; Renal Dialysis

Posterior reversible encephalopathy syndrome (PRES) is characterized by neurotoxic symptoms and neuroimaging finding of reversible cerebral edema in association with various conditions including hypertension, eclampsia, and autoimmune diseases. The author experienced a 47-year-old woman with systemic lupus erythematosus and systemic sclerosis overlap syndrome who developed PRES. The patient presented with alteration of consciousness in association with hypertension and increased autoimmune activity. Magnetic resonance imaging revealed vasogenic edema in the bilateral cerebral cortex, subcortical white matter, basal ganglia, brainstem, and cerebellum.
Autoimmune Diseases ; Basal Ganglia ; Brain Edema ; Brain Stem ; Cerebellum ; Cerebral Cortex ; Consciousness ; Eclampsia ; Edema ; Female ; Humans ; Hypertension ; Lupus Erythematosus, Systemic ; Magnetic Resonance Imaging ; Middle Aged ; Neuroimaging ; Posterior Leukoencephalopathy Syndrome ; Pregnancy ; Scleroderma, Systemic ; White Matter

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*

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

We report a unique case of intestinal duplication detected on posterior reversible encephalopathy syndrome (PRES) in a 13-year-old girl. She was admitted to the pediatric Emergency Department because of generalized seizures. Radiological assessment revealed a large, well-defined, thick-walled cystic lesion in the mid abdomen, suggestive of duplication cyst associated to a PRES. Exploration confirmed the diagnosis of ileal duplication cyst, and the mass was resected. The postoperative course was uneventful. Both hypertension and neurological dysfunction resolved after the mass resection. A followup brain magnetic resonance imaging was performed 9 months later and showed complete resolution of the cerebellar changes. Although extrinsic compression of the retroperitoneal structures has not been reported in the literature as a complication of duplication cyst, we strongly believe that this is the most logical and plausible hypothesis that would explain the pathogenesis of PRES in our patient.
Abdomen ; Adolescent ; Brain ; Diagnosis ; Emergency Service, Hospital ; Female ; Follow-Up Studies ; Humans ; Hypertension ; Ileum ; Logic ; Magnetic Resonance Imaging ; Metoclopramide ; Posterior Leukoencephalopathy Syndrome* ; Seizures

No abstract available.
Amnesia, Transient Global* ; Posterior Leukoencephalopathy Syndrome*