Saudi Journal of Kidney Diseases and Transplantation

: 2012  |  Volume : 23  |  Issue : 3  |  Page : 572--576

Posterior reversible encephalopathy syndrome in a patient with lupus nephritis

Huseyin Kadikoy1, Waqar Haque1, Vu Hoang1, Joseph Maliakkal1, John Nisbet2, Abdul Abdellatif3,  
1 Department of Medicine, Baylor College of Medicine, Houston, TX, USA
2 Department of Radiology, St. Luke's Hospital, Houston, TX, USA
3 Department of Medicine; Division of Nephrology, Baylor College of Medicine, Houston, TX, USA

Correspondence Address:
Abdul Abdellatif
Assistant Professor of Medicine, Department of Medicine, Division of Nephrology, Baylor College of Medicine, One Baylor Plaza, BCM 620, Houston, TX 77030


Posterior reversible encephalopathy syndrome (PRES) is characterized by acute onset of headache, nausea, focal neurological deficits or seizures along with radiological findings of white matter defects in the parietal and occipital lobes. Causes of PRES include uremia, hypertensive encephalopathy, eclampsia and immunosuppressive medications. Usually, the treat­ment of choice involves correcting the underlying abnormality. We describe an unusual case of recurrent PRES caused by uremia during a lupus flare in a patient with biopsy-proven Class IV Lupus Nephritis (LN) with vasculitis. PRES in systemic lupus erythematosis (SLE) is a rare clin­ical phenomenon and, when reported, it is associated with hypertensive encephalopathy. Our patient did not have hypertensive crisis, but had uremic encephalopathy. The patient«SQ»s PRES-related symptoms resolved after initiation of hemodialysis. The temporal correlation of the correc­tion of the uremia and the resolution of the symptoms of PRES show the etiology to be uremic encephalopathy, making this the first reported case of uremia-induced PRES in Class IV LN with vasculitis.

How to cite this article:
Kadikoy H, Haque W, Hoang V, Maliakkal J, Nisbet J, Abdellatif A. Posterior reversible encephalopathy syndrome in a patient with lupus nephritis.Saudi J Kidney Dis Transpl 2012;23:572-576

How to cite this URL:
Kadikoy H, Haque W, Hoang V, Maliakkal J, Nisbet J, Abdellatif A. Posterior reversible encephalopathy syndrome in a patient with lupus nephritis. Saudi J Kidney Dis Transpl [serial online] 2012 [cited 2019 Aug 18 ];23:572-576
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Full Text


Posterior reversible encephalopathy syndrome (PRES) is both a clinical and a radiological entity. [1],[2] Clinically, PRES is characterized by headache, vomiting, visual perception abnormalities [3] and seizures. [4],[5] Radiologically, PRES is characterized by abnormalities in the white matter of the parietal and occipital lobes. [6],[7] The findings consistent with PRES are typi­cally recognized in patients with medically diverse conditions including, but not limited to, systemic lupus erythymatosus (SLE), [8] eclam­psia and pre-eclampsia [9] and end-stage renal disease. [10] PRES has been reported as a compli­cation of various therapeutic intervention stra­tegies such as organ transplantation, [11],[12] phar­macological use of cyclosporine and tacrolimus [13],[14] as well as chemotherapy. [15] Neurolo­gical symptoms of patients diagnosed with Lupus Nephritis (LN), include seizures and psychosis. The seizures and psychosis associated with SLE can take place even in the absence of uremia, keto-acidosis and electrolyte imbalance. [16] While the involvement of the central nervous system in SLE has been well docu­mented, there have been only a few cases of LN associated with PRES, and in these cases the PRES was attributed to hypertension from LN. Here, we describe a patient with LN and vasculitis and acute renal failure who presents with recurrent PRES secondary to uremia.

 Case Report

A 22-year-old Hispanic woman with a history of SLE presented to the emergency room (ER) with a three-day history of shortness of breath, swelling of the legs and headache. The patient was found to have acute kidney injury and kidney biopsy revealed Class IV LN with vasculitis [Figure 1]. The patient was treated with steroids, cyclophosphomide and adjunct rituximab. On day nine of hospitalization, while the patient's presenting symptoms were impro­ving, the patient had two seizures consisting of loss of consciousness, upper extremity sha­king, drooling, eyes rolling back and postictal confusion. The patient reported headache and dizziness, but denied double vision or confu­sion. The patient never smoked or used alcohol and she had no drug allergies. Family history was significant for a brother with type 1 dia­betes. The patient's vital signs included a blood pressure of 154/97 mmHg, pulse of 81 and temperature of 98.1°F. Neurologic exam showed cognitive slowing and slowed coordination, but normal cranial nerve function, equal and sym­metric deep tendon reflexes and 5/5 strength bilaterally.{Figure 1}

On laboratory evaluation, the patient had serum blood urea nitrogen (BUN) of 90 mg/dL, creatinine of 3.7 mg/dL, white blood cell count of 12.6 g/dL and hemoglobin of 9.2 g/dL. The other laboratory values including sodium, po­tassium, calcium, glucose, liver function tests and coagulation studies were normal. A non-contrast computed tomogoraphy (CT) was done on the day of the seizures, and it showed no acute intracranial pathology. A magnetic resonance imaging (MRI) T2 with fluid-atte­nuated inversion recovery (FLAIR) showed hyperintensity prominently involving the sub­cortical white matter of the bilateral posterior cerebral hemispheres with some additional involvement of the bilateral cerebellar hemis­pheres [Figure 2].{Figure 2}

The patient was treated with hemodialysis and anti-seizure medication (levetiracetam), after which the patient's clinical condition improved after initiation of hemodialysis, and the seizure episodes resolved. An MRI ten days after initiation of dialysis demonstrated resolution of the initial cerebral lesions [Figure 3]. The patient was discharged 20 days after presentation with a normal neurologic exami­nation. During close observation while the peritoneal dialysis catheter was healing, the patient presented again to the ER with recur­rent seizures two weeks after discharge. The patient's laboratory values showed urea of 85 mg/dL and creatinine of 3.5 mg/dL, white blood cell count of 11.3 g/dL and hemoglobin of 9.1 g/dL. The patient was treated with emer­gent hemodialysis and her clinical condition improved immediately. At this time, peritoneal dialysis (PD) was initiated and the patient was discharged to continue chemotherapy. At one-year follow-up, the patient is seizure-free of anti-seizure medications.{Figure 3}


PRES was initially described in 1996 as a cli­nical entity consisting of both neurologic and radiological findings. [17] It is characterized by acute onset of headache, altered mental status, visual disturbance and seizures along with ra­diological findings of vasogenic edema, prima­rily in the posterior fossa and parietal and occi­pital lobes of the brain. [4],[18]

PRES can be caused by a variety of clinical entities including uremia, hypertensive encephalopathy, pre-eclampsia/eclampsia, immunosuppressive therapy (primarily cyclosporine and tacrolimus) and connective tissue diseases. [1],[4] From 1988 to 1994, 15 patients were evaluated based on the radiological features of PRES using both CT and MRI. [17] The majority of these patients were either post-transplant pa­tients receiving immunosuppressive therapy or patients with acute hypertensive encephalopathy. Another search from the Medline data­base from 1966 to 2007 revealed 22 patients who were specifically described with rever­sible leukoencephalopathy in the background of SLE. [4] The PRES-associated neurological deficits in the all of these patients resolved either after the withdrawal of immunosuppressive medications or the initiation of antihypertensive therapies. PRES in the background of lupus nephritis is an uncommon phenomenon, and when it does occur, it is usually associated with hypertensive encephalopathy. [4] The pa­tient in our case presented with seizures and acute renal failure due to LN, with BUN and creatinine levels of 90 mg/dL and 3.7 mg/dL, respectively. Interestingly, this patient's blood pressure was not significantly high, but the uremia was most prominent. In this setting, hemodialysis was done to rule out uremia as the etiological factor for the patient's neuro­logical symptoms. Fortunately, her symptoms resolved with dialysis.

While the patient was being closely moni­tored for healing of PD catheter to prevent leak prior to dialysis initiation, she presented to the emergency room with recurrent seizures. All the laboratory values were normal except BUN of 85 mg/dL. This second presentation with elevated urea values similar to the previous one further confirms the association of PRES with uremia.

Although several cases of PRES have been observed and documented, the exact cause of PRES has not been determined. It is possible that multiple pathophysiological processes can generate the clinical and radiological findings consistent with PRES.

The major final common pathway mechanism that explains the radiological findings consis­tent with PRES is vasogenic edema [15] ; how­ever, in a minority of the cases, cytotoxic ede­ma is also observed. The increased prevalence of vasogenic edema over cytotoxic edema in patients with PRES may be explained by the fact that the changes associated with vasogenic edema tend to be reversible. Numerous reports describe the role of hypertensive encephalopathy in the generation of vasogenic edema. [19],[20],[21],[22] When hypertension-induced hyperperfusion of the brain overwhelms the capability of cerebrovascular autoregulation to regulate blood flow, the excess hydrostatic pressure causes increased flow of fluid out of the vasculature into the brain parenchyma. Hypertension-in­duced injury of the endothelial cells lining the capillaries of the cerebrovasculature also causes increased capillary permeability resulting in increased movement of fluid from the vasculature of the brain to the brain parenchyma. [23] Clinically, hypertensive encephalopathy requires that a patient experience sudden increase in blood pressure for a period creating a hyper­tensive emergency. In a study of the clinical characteristics of SLE patients who presented with PRES, [4] 87% of the patients demonstrated a systolic blood pressure of at least 165 mmHg and a diastolic pressure of at least 100 mmHg, unlike the blood pressure in our patient, which was consistently in the 150s/90s. The norma­lization of the blood pressure in these patients resulted in the resolution of the clinical and radiological findings of PRES.

Azotemia is believed to cause vasogenic edema by causing an increase in the permeability of the capillary membrane and cytotoxic edema by direct toxicity to cells in the brain paren­chyma. Azotemia can cause vasogenic edema when the elevated plasma levels of nitroge­nous waste products in uremic encephalopathy alter the permeability of capillary membranes, allowing more fluid to move from the intravascular space into the brain parenchyma. Azotemia can cause cytotoxic edema when some nitrogenous waste products, specifically guanidine compounds, can enter the brain parenchyma and activate neuronal NMDA re­ceptors leading to ultimate excite-toxic cell death. [24] Destruction of neurons leads to the release of intracellular fluid into the extracel­lular space causing cytotoxic edema. Because dialysis in a patient with renal failure decrea­ses the presence of nitrogenous waste products in the plasma, the vasogenic edema due to uremic encephalopathy can be reversed with dialysis alone, as demonstrated in the manage­ment of our patient. Our patient had a BUN level of 85-90 mg/dL during the symptomatic phase of PRES. The clinical and radiological findings of PRES resolved immediately after hemodialysis decreased the BUN level to less than 60 mg/dL. The temporal correlation of the correction of the BUN and the resolution of the symptoms of PRES show the pathophysiological etiology in this patient was uremic encephalopathy, making this the first reported case of uremia-induced PRES in Class IV LN with vasculitides.

The diagnosis of PRES rests upon demonstra­tion of both clinical and radiologic findings consistent with the disorder. FLAIR MRI is the modality of choice in detecting subcortical leukoencephalopathy in the regions that are characteristic of PRES, particularly the poste­rior fossa and the parietal and occipital lobes. The utilization of the diffusion-weighted ima­ging (DWI) MRI technique can be helpful in differentiating between ischemic lesions (cytotoxic edema) and lesions due to PRES (vasogenic edema); ischemic lesions would display hyperintensity on DWI while PRES lesions would display hypointensity. [18] CT study can also be helpful in ruling out acute infarct or hemorrhagic stroke in the same vicinity.

The treatment for PRES depends on the underlying cause; however, antiepileptic drugs should be used to control acute seizures. [4] As was demonstrated in our patient, if uremia is deemed to be significant in the explanation of PRES, then hemodialysis should be carried out to improve the patient's condition. In hypertensive urgency, the treatment of PRES would entail lowering the blood pressure with anti-hypertensive medications. [10] Patients presenting with PRES who are also on immunosuppresive therapy should have their medications temporarily withheld or the dosages decreased until the neurological symptoms of PRES im­prove. The neurological deficits due to PRES itself are only transient so long as the under­lying cause is addressed in a timely manner; therefore, observation of neurological symp­toms such as headache, visual loss, altered mental status and seizures in the setting of uremia should prompt immediate investigation with brain imaging techniques. [14]


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