Posterior reversible encephalopathy syndrome is not associated with mutations in aquaporin-4.

Neurol Genet

Department of Neurology (M.M.), Massachusetts General Hospital and Brigham and Women's Hospital, Harvard Medical School, Boston, MA; Department of Neuroscience (R.M.), Winthrop University Hospital, Mineola, NY; and Department of Neurology (D.S., A.A.R., B.G.W.), Mayo Clinic, Rochester, MN.

Published: August 2015

Posterior reversible encephalopathy syndrome (PRES) is characterized by acute reversible subcortical vasogenic edema that is typically bilateral and self-limiting. It preferentially affects posterior regions of the brain. Clinical manifestations include encephalopathy, seizures, headache, and cortical blindness. PRES may be precipitated by hypertensive crises such as eclampsia and by immunosuppressive agents. The pathophysiology of PRES is incompletely understood. Disordered cerebral autoregulation leading to protein and fluid extravasation is thought to be important.(1) Other theories implicate endothelial dysfunction or vasospasm.(2).

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4807907PMC
http://dx.doi.org/10.1212/NXG.0000000000000013DOI Listing

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