Posterior reversible leukoencephalopathy s (PRES) PEARLS

First described at Tufts University Department of Neurology, 1996, by Judy Hinchey, Claudia Chaves et al.

1. Associations:
cytotoxic drugs (steroids, mtx, tacrolimus, cyclosporine, imuran)
hypertension
glomerulonephritis
porphyria
pheochromocytoma
malignant hypertension
eclampsia
NMO (neuromyelitis optica or NMO spectrum disorder)
autoimmune disease
sepsis
TTP
multiple organ dysfunction especially renal
2.  MRI pearls
--spares the calcarine cortex whereas stroke affects midline calcarine cortex
--may also affect frontal lobes, basal ganglia, brainstem, cerebellum
--no relationship between clinical severity, association, and location of lesions


3. treatment
remove drug
mag sulfate
control blood pressure

4 Review article Mayo Clin Proceedings May 2010 85:5: 427-432
120 cases in 113 patients over several years
-- may self resolve, but substantial Mortality and morbidity due to potential complications including massive infarction, hemorrhage, status epilepticus.
--hypertension present in 74 percent, but only 46 % of those had a history of chronic hypertension
--renal failure present in 57 %, but only half of those had chronic renal insufficiency
--clinical presentations included seizures in 74 % (partial or generalized, only rarely with history of seizures), 18 % with status epilepticus, 28 % with encephalopathy, 26 % with headaches, and 20 % with visual disturbances
-- Autoimmune disease was present in 45 %, of whom three fourths were women.These included TTP (27 %), SLE (18 %), hypothyroid (10 %), scleroderma (6%), Crohn's disease (6 %), ulcerative colitis or sclerosing cholangitis (4 %), RA (4%), DM ( 4%), and 1 patient each or 2 % for Graves' disease,  Hashimoto's thyroiditis, APL syndrome, anti glomerular basement disease, autoimmune hepatitis, polyarteritis nodosa, thromboangitis obliterans, polyglandular autoimmune syndrome, Sjogren's, gfrnulomatous interstitial nephritis, NMO
-- Of patients with autoimmune disease, 65 % had cerebellar involvement, and majority of septic patients had involvement of the cerebrum.
-- transplant and chemo and pediatric populations not represented in above article