Posterior Reversible Encephalopathy Syndrome in Childhood

Acute headaches with remittent vomiting and seizures represent an emergency in neuropediatric patients. Severe reversible posterior encephalopathy is a rare diagnosis in childhood. The literature provides case reports to the course of adults. The term has been used first in 1996 by Hinchey on the occasion of a series of 15 cases. Those patients exhibited a reversible syndrome with headache, neuropsychological distinctive features, visual abnormalities (blurry gaze, visual hallucinations, visual neglect, hemianopia, and cortical blindness) and seizures. The posterior reversible encephalopathy syndrome (PRES) is known under merely different designations and a misnomer, because it is not reversible in all of the cases, not always limited to the posterior regions of the brain and the gray matter often is affected too. The causes are versatile. Triggers primarily are chemotherapeutic agents and immunosuppressive drugs, renal diseases as well as accelerated blood pressure with hypertensive crises. These are responsible for the development of a vasogenic edema. Magnetic resonance imaging of the brain is the favored modality of investigation. Cerebral edema involving the posterior regions of the cerebral hemispheres, particularly the posterior parietal and occipital lobes, is seen as increased T2 and fluid attenuated inversion recovery signal. Apparent diffusion coefficient maps show increased signal, and diffusion weighted imaging images normal or decreased signal intensity of lesions. These features can differentiate between vasogenic cerebral edema in PRES. Prognostic crucial is a rapid start of an efficient therapy (decline of the hypertension and stop of the responsible medication). We report the case of a 14-year-old boy with PRES within the frame work of an unknown hypertension before admission to the hospital and a girl aged 16 years with unknown origin who also presented with PRES. Furthermore, we want to give an overview of the literature with a special attention to the publications to PRES in children.