Atypical Posterior Reversible Encephalopathy Syndrome in a Postpartum Woman With Moyamoya Disease: A Case Report and Literature Review

Background: Moyamoya disease is a rare cerebrovascular occlusive disease, which is characterized by stenosis and gradual occlusion of the internal carotid arteries, causing the progression of characteristic collateral vessels. To date, most studies investigating moyamoya disease have focused on medical implications, and the potential implications for neurocognitive and/or neuropsychiatric functioning were inconclusive.Case Presentation: we present a case of a 26-year-old Chinese postpartum woman who presented to the emergency department with a 19-h history of cognitive decline, vomiting, and convulsions. Blood pressure, heart rate, and respiration rate were 200/120 mmHg, 115 beats/minute, and 30 breaths/minute, respectively, on arrival. The Glasgow Coma Scale, modified RANKIN scale (mRS), and National Institute of Health stroke scale (NIHSS) scores were 3, 5, and 18, respectively. Moyamoya disease was diagnosed using cerebral angiography and digital subtraction angiography. The cognitive functions of orientation, use of language, ability to calculate, and memory significantly improved after 11 days of treatment (Glasgow Coma Scale: 15; mRS: 0; NIHSS: 0).Conclusions:This patient was diagnosed with reversible posterior leukoencephalopathy syndrome related to moyamoya disease. This case highlights that atypical posterior reversible encephalopathy syndrome can occur in patients with moyamoya disease, and should be considered for the differential diagnosis of cerebral infarcts and hemorrhage in a postpartum female.

A case series of posterior reversible encephalopathy syndrome

Introduction: The posterior reversible encephalopathy (PRE) is a clinico-radiologic syndrom characterized by seizures, consciousness’s disorder, visual loss, and headache associated with cerebral posterior abnormalities on imaging. It raises often a problem of differential diagnosis with cerebral infarction. Case reports: We report the case of five patients, the first followed for systemic lupus erythematosus, the second is hypertensive poorly follow-up, the 3 other patients were in postpartum after cesarean delivery. All our patients had generalized tonic-clonic seizures associated with an hypertensive peak without edema syndrome. Laboratory tests were unremarkable. The EEG showed epileptic abnormalities and brain MRI was in favor of a reversible posterior leukoencephalopathy. Evolution under treatment was favorable without recurrence with a decline of 20 months. Conclusion: Rapid regression clinical and radiolologic abnormalities suggest cerebral vosogenic oedema as mechanism of this disease and confirm its mildness. Nevertheless, PRE may not be reversible without an early control of causes.

Reversible Posterior Leukoencephalopathy Syndrome (RPLS) in Late Onset Postpartum Eclampsia- A Retrospective Study

Background: Reversible posterior leukoencephalopathy syndrome (RPLS) or Posterior reversible encephalopathy syndrome (PRES) is a unique clinicoradiologic entity characterized by acute onset headache, seizures, blindness and altered mental state associated with reversible vasogenic edema of the brain. It is a major complication of eclampsia, but data on clinicoradiological features of PRES in late-onset postpartum eclampsia are scarce. The objective is to analyze the clinicoradiologic features and outcome in a cohort of late postpartum eclampsia patients with PRES. Subjects and Methods: Eighteen patients of late postpartum eclampsia with clinical as well as neuroimaging features consistent with PRES were included in this retrospective study. All had undergone magnetic resonance imaging (MRI) with diffusion-weighted imaging (DWI) and apparent diffusion coefficient(ADC) mapping. Data on age, antepartum history, peak systolic and diastolic blood pressures, clinical features, time to neuroimaging, location of lesions on brain imaging and presence of associated ischemia or hemorrhage in MRI were collected and analyzed. Results: The mean age was 25.72 3.23 years. The average duration between labor and clinical symptoms was 8.5 days. 13/18 patients (72.2%) had elevated blood pressure at admission. Mean systolic and diastolic blood pressures were 143.88 (120-180) and 93.88 (80-100) mm Hg respectively. Headache was the presenting feature in 16 patients. The parieto-occipital regions were the most frequently involved followed by the cerebellum (7/18) and frontal lobe (4/18). Atypical regions were not involved. Clinical recovery had been noted in all. Conclusion: PRES associated with late postpartum eclampsia, an entity of limited awareness can present without antecedent preeclampsia. Radiological changes are the key to early diagnosis.

Síndrome da Encefalopatia Posterior Reversível (PRES) no ciclo gravídico puerperal: revisão bibliográfica

Objetivo: Elencar as principais características fisiopatológicas, clínicas, diagnósticas e manejo terapêutico da Síndrome da Encefalopatia Posterior Reversível (PRES) em gestantes e puérperas. Métodos: Esta revisão da literatura usou a base de dados de pesquisa CAPES, com os descritores: “PRES”, “Posterior Reversible Encephalopathy Syndrome”, “Posterior Leukoencephalopathy Syndrome” e “Pregnancy”, entre os anos de 2003 a 2019, resultando em 50 artigos que preencheram os critérios de inclusão. Resultados: Verificou-se que, os mecanismos fisiopatológicos que buscam explicar a ocorrência geral dessa síndrome, ainda não são claros. Os principais sinais clínicos identificados foram convulsões associadas a cefaleia e alterações visuais. O exame padrão-ouro é a ressonância magnética, preferencialmente em T1, T2, ADC e DWI, estando a restrição de difusão relacionada a um pior prognóstico. Feito o diagnóstico, é necessário o manejo terapêutico com estabilização da paciente, controles pressórico, controle de convulsões e afastar a causa base que desencadeou o processo da PRES. Considerações finais: A PRES é uma síndrome de início agudo, sendo importante o reconhecimento das suas características clínicas e diagnósticas. Por isso quando pensar em PRES? Sugerimos um recurso mnemônico para lembrar como diagnóstico diferencial. P: Pré-eclâmpsia e eclâmpsia; R: Restrição de difusão na Ressonância Magnética; E: Edema cerebral; S: Sintomas visuais.

PRES Syndrome

The aim of this review, as well as the case report, is to become familiar with the syndrome, although it is not very common, but may still be encountered by an ophthalmologist during clinical practice. It is also interesting to point out how the clinical unit can be independent and unchangeable in medicine and, on the other hand, in the context of the reversible posterior leukoencephalopathy syndrome (PRES syndrome), the name can be changed. As such, cortex blindness arises after complete destruction of the visual cortex of both occipital lobes, often as a result of vascular circulatory disorders. PRES syndrome is characterized by magnetic resonance imaging or computed tomography, where bilateral irregular hypodensive arteries are present in the occipital lobes that cause transient cortex blindness within the syndrome, which in its name carries the word reversible. Case report: A patient who was hospitalized at the Pneumology Department in which PRES syndrome and transient cortex blindness were diagnosed.

Infection-provoked reversible posterior leukoencephalopathy syndrome in an adult with nephrotic syndrome: a case report

Background Reversible posterior leukoencephalopathy syndrome (RPLS) is a rare and heterogeneous clinico-neuroradiological syndrome characterized by headache, altered mental status, seizures, and visual disturbances. Hypertension and immunosuppression are two of the main factors that predispose an individual to RPLS. However, RPLS can develop when no major risk factors are present. RPLS has been reported in pediatric nephrotic patients, but rarely in adults. Case presentation A 42-year-old Asian woman with nephrotic syndrome presented with seizures, headaches, and nausea. Her blood pressure was controlled, and no immunosuppressants had been prescribed. All symptoms and tests indicated RPLS following infection with pneumonia, which was successfully treated by immediate administration antibiotic and anti-epileptic medications. Seizures did not recur during a 2-year follow-up period. Conclusions When patients with nephrotic syndrome have an infection, RPLS symptoms should be investigated thoroughly. With early diagnosis and appropriate treatment of RPLS, morbidity and mortality can be prevented.