Childhood Posterior Reversible Encephalopathy Syndrome: Clinicoradiological Characteristics, Managements, and Outcome

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a novel clinicoradiological syndrome characterized by convulsions, headache, altered mentality, and impaired vision, which are usually accompanied by hypertension. As its nomination, PRES is usually diagnosed according to the presence of typical neuroimage showing vasogenic edema predominately involving the posterior brain area. With the widespread utilization of magnetic resonance imaging (MRI), PRES is becoming more perceptible in different medical fields. Compared to adult cases, childhood PRES seems to have a broader clinical and neuroradiological spectrum. PRES can be associated with various underlying comorbidities, medication use, and therapeutic modalities in children with diverse neurological manifestations. Moreover, pediatric patients with PRES have a more significant propensity for atypical MRI findings beyond the typically posterior cerebral areas. The knowledge of typical and atypical presentations in children is essential to avoid misdiagnosing or missing PRES, which is a potentially treatable entity. Early supportive care is the mainstay of treatment, with particular attention to the treatment of hypertension with rigorous attention to all body systems. Prompt identification and symptom-directed management are imperative to achieve a reversible prognosis in childhood PRES. Future studies specially designed for the child population are required to determine potential outcome predictors, and further, to develop novel strategies of neuroprotection in childhood PRES.

Clinico-radiological Profile of Posterior Reversible Encephalopathy Syndrome and Its Associated Risk Factors in PICU: A Single-center Experience from a Tertiary Care Hospital in Bhubaneswar, Odisha

“…Though the majority of patients belong to the middle-aged age group (mean: 39–47 years) with female predominance, the mean age of presentation from multiple pediatric series with different etiology was found to be 11.6 years without any gender difference. 2 It has been increasingly reported in various childhood disorders like hypertensive emergencies, renal disorder, sepsis, lupus nephritis, sickle cell anemia disease, primary immunodeficiency, use of cytotoxic medications, and organ transplantation. Hypertension and renal diseases contribute to PRES in the majority of cases.…”

Posterior Reversible Encephalopathy Syndrome: An Expanding Phenotype

“…It also became clear that hypertension may be absent in about a fifth of cases. 1 , 2 In 1996, Hinchey et al described the disorder, now known as “posterior reversible encephalopathy syndrome” (PRES), in a study of 15 patients. 3 …”

“… 4 Higher prevalence rates are noted in retrospective studies in pediatric intensive care unit (PICU) patients (0.4%) 5 , oncology centers (0.7%), and transplant centers (up to 5.2%). 2 Hypertension appears to be the most common risk factor and is present in ∼80–85%; other common risk factors include renal, hemato-oncological and autoimmune disorders, immunosuppressive/cytotoxic drug use, bone marrow/organ transplantation, and others. 2 …”

“… 2 Hypertension appears to be the most common risk factor and is present in ∼80–85%; other common risk factors include renal, hemato-oncological and autoimmune disorders, immunosuppressive/cytotoxic drug use, bone marrow/organ transplantation, and others. 2 …”

“…Recently, the central role of vascular endothelial growth factor (VEGF), an angiogenic factor secreted by the endothelium that increases vascular permeability, has been highlighted. 1 , 2 …”