Posterior reversible encephalopathy syndrome in a woman with focal segmental glomerulosclerosis

Abstract

Posterior reversible encephalopathy syndrome (PRES) is a rare syndrome characterized by reversible vasogenic edema in the posterior hemispheres. PRES is most often attributed to primary hypertension, pre-eclampsia, and neurotoxicity secondary to immunosuppressants such as cyclosporine. Renal disease is an infrequent cause of PRES with a majority of cases occurring in adults with complete renal failure or in pediatric cases with underlying renal parenchymal disease and concurrent immunosuppressive therapy. Typical symptoms include seizure, headache, altered mental status, and visual disturbances. PRES is rarely associated with cerebral hemorrhage, and even less so with subarachnoid bleeds. Herein we report on a 25-year-old female with focal segmental glomerulosclerosis who developed PRES. The patient’s presentation was more severe as she presented with seizure, nephrotic syndrome, and subarachnoid hemorrhage. Computed tomography and magnetic resonance imaging with concurrent symptoms led us to the final diagnosis. The patient was treated with antihypertensives, diuretics, and corticosteroids and follow-up imaging revealed resolution of PRES. Our case illustrates that underlying kidney disease even without immunosuppressive agents should be added to the list of possible causes for PRES. Symptoms are reversible with treatment of underlying cause or offending agent.

Posterior reversible encephalopathy syndrome in malignant hypertension secondary to focal segmental glomerulosclerosis

“…Its association is well established with a number of clinical conditions and has mostly been reported with eclampsia, malignant hypertension and immunosuppressive agents 5. However, PRES secondary to focal segmental glomerulosclerosis (FSGS) is extremely rare 6. This case report acknowledges the clinical findings of previously reported cases and also describes the involvement of new/unique/unexpected areas of the brain parenchyma in PRES due to FSGS.…”

“…PRES in association with nephrotic syndrome secondary to FSGS is rare6 and we found only three reported cases of PRES in association with FSGS 6 8 9. All the three patients were in their 20s and presented with uncontrolled hypertension, seizures, headache and visual changes.…”

Focal segmental glomerulosclerosis in children complicated by posterior reversible encephalopathy syndrome

“…All lesions are usually reversible after interruption of immunosuppressive therapy. 26 Oliveira et al 27 reported remission of PRES after 2 to 6 months. In our case, a full recovery was attained after 6 months.…”

Corticosteroid therapy and severity of vasogenic edema in posterior reversible encephalopathy syndrome

“…However, steroids are also used in the treatment of vasogenic edema, such as in patients with intracranial mass lesions [9]. Case reports have implicated steroids as both the precipitant [5, 8, 12, 19, 20, 23, 24, 30, 31, 34, 38, 47, 48] and treatment [1, 6, 8, 12, 13, 18, 25, 28, 30, 33, 36, 37] of PRES. Thus, the role of steroids in PRES remains unclear.…”