2009 Southern Regional Meeting Abstracts

Case Report: Introduction: Posterior reversible encephalopathy syndrome (PRES), may present with a variety of neurological manifestations: headache, seizure, altered mental status, cortical blindness, focal neurological deficits, or vomiting in association with transient changes in brain MRI imaging of cerebral edema primarily involving the parietal and occipital lobes. PRES most often occurs in the clinical setting of hypertension, eclampsia, renal failure, or immunosuppression.
Case: A 17-year-old African-American girl with an 8 year history of SLE who presented with new onset seizures, altered mental status, hypertension, vomiting and blood pressure of 164/122. Diagnosed in 2005 with WHO Class IV Lupus Nephritis, the patient had nephrotic range proteinuria for the past year. She received a 6-month course of cyclophosphamide one-year ago but was switched to mycophenolate mofetil, due to cyclophosphamide intolerance. The patient also received one treatment course with rituximab four months prior to admission. MRI on admission showed hyper-intense T2/FLAIR lesions of the cerebellar hemispheres bilaterally as well as abnormalities of the bilateral cerebral hemispheres localized to the occipital lobes. The patient was treated with intravenous solumedrol for active lupus nephritis and thrombocytopenia. Blood pressure was controlled by intravenous anti-hypertensive, which resolved the vomiting and altered mental status. Follow-up MRI four days later showed significant resolution of the cerebellar lesions although persistence of the occipital lesions. There was marked improvement of all lesions on the MRI done one month later.
Discussion and Conclusion: PRES is not unique to lupus but should be considered in an SLE patient presenting with neurological symptoms. PRES needs to be distinguished from lupus cerebritis, infection related to immunosuppressive therapy and thrombotic events as the treatment for each is obviously very different. Currently, the pathophysiology of PRES is unclear and controversial. Further research is needed to determine the mechanism, but regardless of the etiology, prompt control of blood pressure leads to resolution of symptoms and MRI changes.