Case Presentation:

A 52–year–old female with history of well–controlled hypertension, diabetes on insulin, end–stage renal disease status post transplant and prior breast cancer presented with complaints of painless, progressive visual loss over the past five days. Her symptoms began with occipital headaches five days ago that were “pounding.” These did not radiate, progressively worsened and were not associated with any precipitating factors. Three days PTA, her headache abruptly worsened from “from pounding to piercing” and she developed loss of vision. She initially described blurring of vision (she could not see the faces on bills) to complete loss of vision with some light perception. The visual loss involved both eyes and prompted medical attention. Her current transplant medical regimen included a stable dose of Prograf 2mg BID. In the ED, exam was significant for a middle aged F who appeared appropriately apprehensive. VS revealed a HR 90, BP 200/120mmHg (multiple readings). Dedicated neurological examination revealed that she was AA0 x 3 with fluent speech. Cranial nerve exam was significant for loss of light perception in right eye, faint light perception in left eye, normal direct and indirect pupillary reflexes, no ptosis and normal eye movements. Fundoscopy was negative for papilledema. No other neurologic or systemic abnormalities were detected. All laboratory tests were within normal limits. In the ED, the patient subsequently developed a tonic–clonic seizure that was treated with Ativan with resolution; a stat head CT revealed vasogenic edema involving bilateral occipital lobes.

Discussion:

The patient’s history and findings are most consistent with Posterior Reversible Encephalopathy Syndrome, or PRES. This entity was first described by Hinchey et al. in a case series of 15 patients who presented with seizures, confusion, visual loss; 7/15 patients were on immuno–suppressive Rx after solid organ transplantation. The diagnosis is confirmed by clinical presentation and head CT that classically shows bilateral white matter edema in the occipital and posterior lobes of the brain. PRES is thought to be a localized manifestation of hypertensive encephalopathy, in that absence of sympathetic innervation in posterior circulation predisposes to leakage of plasma into interstituim and preferential edema in this area in the setting of systemic hypertension. Other proposed mechanisms include direct cytotoxicity and endothelial damage by immune modulating drugs and focal vasospasm, ischemia/edema.

Conclusions:

Immune suppression in transplant patients is highly associated with PRES, a common reversible condition that manifests with headaches, blindness and seizures. Hospitalists should be aware of this benign condition as prompt treatment directed towards blood pressure control and consideration of alternative immune suppressive therapy is associated with improved outcomes. Our patient improved with return of vision and resolution of headaches with BP control.

Table 1Frequency of Signs/Symptoms in PRES

Figure 1MRI FLAIR images show high signal symmetrically involving bilateral parieto–occipital, posterior frontal, and temporo–occipital regions.