Posterior reversible encephalopathy syndrome

C Lamy, C Oppenheim, J L Mas
Handbook of Clinical Neurology 2014, 121: 1687-701
Posterior reversible encephalopathy syndrome (PRES) is a recently proposed cliniconeuroradiologic entity with several well-known causes, such as hypertensive encephalopathy, eclampsia, and the use of cytotoxic and immunosuppressive drugs, as well as some causes more recently described. PRES is characterized by neuroimaging findings of reversible vasogenic subcortical edema without infarction. The pathogenesis is incompletely understood. Two opposing hypotheses are commonly cited, but the issue is controversial: (1) the current more popular theory suggests that severe hypertension exceeds the limits of autoregulation, leading to breakthrough brain edema; (2) the earlier original theory suggests that hypertension leads to cerebral autoregulatory vasoconstriction, ischemia, and subsequent brain edema. The clinical syndrome of PRES typically involves headache, encephalopathy, visual symptoms, and seizures. The clinical presentation is often nonspecific, and therefore the diagnosis of PRES has come to increasingly rely on magnetic resonance imaging (MRI) abnormalities consistent with PRES with documented recovery clinically and on repeated neuroimaging. The diagnosis has important therapeutic and prognostic implications because the reversibility of the clinical and radiologic abnormalities is contingent on the prompt control of blood pressure and/or discontinuing the offending drug.

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