Posterior reversible encephalopathy syndrome during systemic lupus erythematosus: four new cases and review of the literature
Abstract
Posterior reversible encephalopathy syndrome (PRES) associates various neurological manifestations (headaches, seizures, altered mental status, cortical blindness, focal neurological deficits, vomiting) and transient changes on neuroimaging consistent with cerebral edema. Posterior reversible encephalopathy syndrome mainly occurs in the setting of hypertension, eclampsia, renal failure and/or use of immunosuppressive drugs.
We report four cases of PRES complicating systemic lupus erythematosus (SLE). In all our cases, renal involvement and hypertension were present. Neurological symptoms were typical. Magnetic resonance imaging showed posterior cerebral edema and in one case hemorrhagic complication. With symptomatic treatment and immunosuppressor withdrawal when they were previously used, symptoms fully resolved within 15 days in all cases, but one who had only partial regression related to cerebral hemorrhage.
Including our cases, we reviewed a total of 46 patients with SLE and PRES. Their clinical and radiological presentation was not specific. The peculiar role of SLE itself in the occurrence of PRES was not clear, since hypertension (95%), renal involvement (91%), recent onset of immunosuppressive drugs (54%) and/or recent treatment with high intravenous dose of steroids (43%) were often present. The hypertension and other worsening factors should be treated. Finally, the evolution of this clinical and radiological spectacular syndrome is generally rapidly favorable. Lupus (2008) 17, 139—147.
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References
Casey SO, Sampaio RC, Michel E., Truwit CL Posterior reversible encephalopathy syndrome: utility of fluid-attenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions. AJNR Am J Neuroradiol 2000; 21: 1199—1206.
Hinchey J., Chaves C., Appignani B. et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996; 334: 494—500.
Bell CL, Partington C., Robbins M., Graziano F., Turski P., Kornguth S. Magnetic resonance imaging of central nervous system lesions in patients with lupus erythematosus. Correlation with clinical remission and antineurofilament and anticardiolipin antibody titers. Arthritis Rheum 1991; 34: 432—441.
Caminal-Montero L., Trapiella-Martinez L., Suarez-Casado H., Garcia-Garcia J. Reversible posterior leucoencephalopathy in systemic lupus. Med Clin (Barc) 2005; 125: 719.
Cassano G., Gongora V., Zunino A., Roverano S., Paira S. Reversible posterior leukoencephalopathy in systemic lupus erythematosus with thrombotic thrombocytopenic purpura. J Clin Rheumatol 2007; 13: 55—57.
Casali-Rey JI, Davalos EG, Lopez-Amalfara A., Julio-Munoz D., Pagano MA Posterior reversible encephalopathy syndrome: some case reports. Rev Neurol 2003; 37: 224—227.
Foocharoen C., Tiamkao S., Srinakarin J., Chamadol N., Sawanyawisuth K. Reversible posterior leukoencephalopathy caused by azathioprine in systemic lupus erythematosus . J Med Assoc Thai 2006; 89: 1029—1032.
Gocmen R., Ozgen B., Oguz KK Widening the spectrum of PRES: Series from a tertiary care center. Eur J Radiol 2007; 62: 454—9.
Kur JK, Esdaile JM Posterior Reversible Encephalopathy Syndrome- An Underrecognized Manifestation of Systemic Lupus Erythematosus. J Rheumatol 2006; 33: 2178—2183.
Lateef A., Lim AY Case reports of transient loss of vision and systemic lupus erythematosus . Ann Acad Med Singapore 2007; 36: 146—144.
Magnano MD, Bush TM, Herrera I., Altman RD Reversible posterior leukoencephalopathy in patients with systemic lupus erythematosus . Semin Arthritis Rheum 2006; 35: 396—402.
Mavragani CP, Vlachoyiannopoulos PG, Kosmas N., Boletis I., Tzioufas AG, Voulgarelis M. A case of reversible posterior leucoencephalopathy syndrome after rituximab infusion . Rheumatology (Oxford) 2004; 43: 1450—1451.
Mukherjee P., McKinstry RC Reversible posterior leukoencephalopathy syndrome: evaluation with diffusion-tensor MR imaging. Radiology 2001; 219: 756—765.
Ozyurek H., Oguz G., Ozen S. et al. Reversible posterior leukoencephalopathy syndrome: report of three cases. J Child Neurol 2005; 20: 990—993.
Pavlakis SG, Frank Y., Kalina P., Chandra M., Lu D. Occipital-parietal encephalopathy: a new name for an old syndrome. Pediatr Neurol 1997; 16: 145—148.
Prasad N., Gulati S., Gupta RK, Kumar R., Sharma K., Sharma RK Is reversible posterior leukoencephalopathy with severe hypertension completely reversible in all patients? Pediatr Nephrol 2003; 18: 1161—1166.
Primavera A., Audenino D., Mavilio N., Cocito L. Reversible posterior leucoencephalopathy syndrome in systemic lupus and vasculitis. Ann Rheum Dis 2001; 60: 534—537.
Schwartz RB, Jones KM, Kalina P. et al. Hypertensive encephalopathy: findings on CT, MR imaging, and SPECT imaging in 14 cases . AJR Am J Roentgenol 1992; 159: 379—383.
Sheth RD, Riggs JE, Bodenstenier JB, Gutierrez AR, Ketonen LM, Ortiz OA Parietal occipital edema in hypertensive encephalopathy: a pathogenic mechanism . Eur Neurol 1996; 36: 25—28.
Shin KC, Choi HJ, Bae YD, Lee JC, Lee EB, Song YW Reversible posterior leukoencephalopathy syndrome in systemic lupus erythematosus with thrombocytopenia treated with cyclosporine. J Clin Rheumatol 2005; 11: 164—166.
Thaipisuttikul I., Phanthumchinda K. Recurrent reversible posterior leukoencephalopathy in a patient with systemic lupus erythematosus . J Neurol 2005; 252: 230—231.
Yong PF, Hamour SM, Burns A. Reversible posterior leukoencephalopathy in a patient with systemic sclerosis/systemic lupus erythematosus overlap syndrome. Nephrol Dial Transplant 2003; 18: 2660—2662.
Punaro M., Abou-Jaoude P., Cimaz R., Ranchin B. Unusual neurologic manifestations (II): posterior reversible encephalopathy syndrome (PRES) in the context of juvenile systemic lupus erythematosus. Lupus 2007; 16: 576—579.
Dy GK, Swaroop VS 31-year-old woman with confusion and labile behavior. Mayo Clin Proc 2002; 77: 557—560.
Ishimori ML, Pressman BD, Wallace DJ, Weisman MH Posterior reversible encephalopathy syndrome: another manifestation of CNS SLE? Lupus 2007 ; 16: 436—443.
Ozgencil E., Gulucu C., Yalcyn S. et al. Seizures and loss of vision in a patient with systemic lupus erythematosus. Neth J Med 2007; 65: 274.
Schwartz RB, Mulkern RV, Gudbjartsson H., Jolesz F. Diffusion-weighted MR imaging in hypertensive encephalopathy: clues to pathogenesis. AJNR Am J Neuroradiol 1998; 19: 859—862.
Schwartz RB, Feske SK, Polak JF et al. Preeclampsia-eclampsia: clinical and neuroradiographic correlates and insights into the pathogenesis of hypertensive encephalopathy. Radiology 2000; 217: 371—376.
Servillo G., Bifulco F., De Robertis E. et al. Posterior reversible encephalopathy syndrome in intensive care medicine. Intensive Care Med 2007; 33: 230—236.
Lamy C., Oppenheim C., Meder JF, Mas JL Neuroimaging in posterior reversible encephalopathy syndrome. J Neuroimaging 2004; 14: 89—96.
Min L., Zwerling J., Ocava LC, Chen IH, Putterman C. Reversible posterior leukoencephalopathy in connective tissue diseases . Semin Arthritis Rheum 2006; 35: 388—395.
Bartynski WS, Boardman JF, Zeigler ZR, Shadduck RK, Lister J. Posterior reversible encephalopathy syndrome in infection, sepsis, and shock . AJNR Am J Neuroradiol 2006; 27: 2179—2190.
Giorgi D., David V., Afeltra, A. Gabrieli CB Transient visual symptoms in systemic lupus erythematosus and antiphospholipid syndrome. Ocul Immunol Inflamm 2001; 9: 49—57.
Calabrese LH, Molloy ES, Huang D., Ransohoff RM Progressive multifocal leukoencephalopathy in rheumatic diseases: evolving clinical and pathologic patterns of disease . Arthritis Rheum 2007; 56: 2116—2128.
Ay H., Buonanno FS, Schaefer PW, et al. Posterior leukoencephalopathy without severe hypertension: utility of diffusion-weighted MRI. Neurology 1998; 51: 1369—1376.
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Article first published online: February 1, 2008
Issue published: February 2008
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PubMed: 18250139
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