Clinical and imaging features of acute encephalopathy with biphasic seizures and late reduced diffusion in children / 中华儿科杂志

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.

Cortical blindness secondary to posterior reversible encephalopathy syndrome, recovered by successful blood pressure management / Cegueira cortical secundária à síndrome de encefalopatia reversível posterior recuperada pelo controle bem sucedido da pressão arterial

ABSTRACT We report a case of cortical blindness secondary to posterior leukoencephalopathy syndrome, which was successfully treated by blood pressure management. A 66-year-old white man presented at the emergency room complaining of severe frontal headache, disorientation, and progressive blurred vision. The initial physical examination disclosed a blood pressure of 200/176 mmHg. One hour later the patient exhibited mental confusion and bilateral blindness. Computed tomography and magnetic resonance imaging were performed, and a systemic workup was conducted, resulting in a diagnosis of posterior leukoencephalopathy syndrome. This syndrome is a rare manifestation of systemic hypertension that requires proper diagnosis and management to avoid irreversible brain damage. Early recognition of this condition and prompt control of the patient's blood pressure are essential because they may bring about a reversal of the syndrome, which may otherwise result in permanent brain damage.

RESUMO Relatamos um caso de cegueira cortical secundário à síndrome de leucoencefalopatia posterior recuperado pelo controle bem sucedido da pressão arterial. Um homem branco de 66 anos de idade compareceu à emergência com queixa de dor de cabeça frontal severa, desorientação e embaçamento progressivo da visão. O exame físico inicial revelou uma pressão arterial de 200/176 mmHg. Uma hora depois, o paciente apresentou confusão mental e cegueira bilateral. Após a tomografia e a ressonância nuclear magnética, o tratamento sistêmico foram realizados e o diagnóstico de síndrome da leucoencefalopatia posterior foi realizado. A síndrome de leucoencefalopatia posterior é uma manifestação rara de hipertensão sistêmica que requer um diagnóstico e gerenciamento adequados para evitar danos cerebrais irreversíveis. O reconhecimento precoce desta condição e o controle imediato da pressão arterial são essenciais porque podem levar à reversão da síndrome, o que, de outra forma, pode resultar em dano cerebral permanente.

Caso de hemianopsia reversível no pós-parto decorrente de síndrome da encefalopatia reversível posterior (PRES) em gestante com eclâmpsia tardia / Reversible hemianopsia in postpartum due to posterior reversible encephalopathy syndrome in pregnant with late eclampsia

Resumo Objetivos: Descrever um caso de Síndrome da Encefalopatia Reversível Posterior em gestante diagnosticada com eclâmpsia tardia, bem como seu manejo clínico. Descrição do caso: Paciente feminina, 34 anos, em sua terceira gestação, iniciou com aumento dos níveis tensionais durante o trabalho de parto e, após onze dias de puerpério, apresentou quadro de diminuição da acuidade visual à direita, seguida de crise convulsiva e subsequente perda parcial da visão do olho direito. Após a realização de exames de imagem e descartada a possibilidade de acidente vascular encefálico, a paciente foi diagnosticada com Síndrome da Encefalopatia Reversível Posterior (PRES). Instituído o manejo clínico das crises convulsivas e hipertensivas, houve remissão completa dos sintomas e reversão do quadro clínico inicial. Conclusões: Uma vez adequadamente diagnosticada e tratada, a Síndrome da Encefalopatia Reversível Posterior pode apresentar evolução satisfatória, especialmente quando associada a um fator desencadeado agudamente, como a eclâmpsia.

Abstract Objectives: To describe a case of Posterior Reversible Encephalopathy Syndrome diagnosed in pregnant women with late-eclampsia, as well as its clinical management. Case description: A 34 years old patient in her third pregnancy had started with high blood pressure levels during labor; after eleven days postpartum, she presented a decreased right visual acuity; subsequently one episode of seizure followed by partial loss of vision in the right eye. After conducting tests and ruled out stroke, the patient was diagnosed as Posterior Reversible Encephalopathy Syndrome (PRES). Established the clinical management of seizures and hypertensive crisis, there was complete remission of symptoms and reversal of the initial clinical picture. Conclusion: Once properly diagnosed and treated, the Posterior Reversible Encephalopathy Syndrome can present satisfactory progress, especially when associated with an acutely triggered factor, as eclampsia.

Atypical Unilateral Posterior Reversible Encephalopathy Syndrome Mimicking a Middle Cerebral Artery Infarction

Posterior reversible encephalopathy syndrome (PRES) is usually a reversible clinical and radiological entity associated with typical features on brain MR or CT imaging. However, the not-so-uncommon atypical radiological presentations of the condition are also present and they may go unrecognised as they are confused with other conditions. Here, we report a very rare case of atypical, unilateral PRES in a 49-year-old uremic, post-transplant female patient who presented with seizures. Initial MRI showed high-grade occlusion of the left middle cerebral artery (MCA) and lesions suggestive of subacute infarction in the ipsilateral frontotemporoparietal lobe. Patient symptoms had resolved a day after the onset without any specific treatment but early follow-up CT findings suggested hemorrhagic transformation. Follow-up MRI performed 2 years later showed complete disappearence of the lesions and persisting MCA occlusion.

Posterior Reversible Encephalopathy Syndrome.

PRES stands for Posterior Reversible Encephalopathy Syndrome. It is a proposed clinoneuroradiological entity that is characterized by headache, altered mental status, cortical blindness, seizure and other focal neurological signs and diagnostic magnetic resonance imaging picture. A variety of different etiologies have been reported like hypertension, preeclampsia, eclampsia, cyclosporine (and tacrolimus) neurotoxicity, uremia, sepsis, porphyria, SLE and interferon therapy. With early diagnosis and treatment the syndrome is usually fully reversible. A case of reversible encephalopathy is reported here in a 7-day postpartum female patient with marked elevation of blood pressure. Clinician and radiologist must be familiar with this clinically frightening, under diagnosed condition to assure timely diagnosis and treatment so as to prevent persistent defect.

Status epilepticus as a presenting sign of reversible posterior leukoencephalopathy syndrome in a liver transplant recipient

Reversible posterior leukoencephalopathy syndrome [RPLE] is an increasingly recognized disorder, most commonly associated with immunosuppressive and cytotoxic agent, malignant hypertension and toxemia of pregnancy. Neurological symptoms are headache, mental and visual disturbance, and seizures. Brain MRI findings are symmetrical bilateral hyper intense areas of occipital and parietal lobes in T2-weighted magnetic resonance [MR] images. This finding is reversible with prompt successful treatment which may take days to weeks for full reversal. We observed a case of status epilepticus which occurred 32 days after liver transplantation in a patient on regimen consisting of Tacrolimus [FK 506]. He had no history of neurologic disease or metabolic abnormalities. Typical findings of RPLE were present on brain MRI. The recurrence of seizures terminated after a few hours with adding Na-valproate to Phenytoin, Phenobarbital and Midazolam. The radiological finding of brain MRI gradually diminished after several weeks. A physician should be alert of seizures during early post transplant period of liver transplantation. He should bear in mind the diagnosis of RPLE and consider discontinuation of immunosuppressive drugs