RESUMEN
The case of a 75-year-old man with a history of lymphoma, recent upper respiratory tract infection, and a protracted course of encephalopathy is presented. Radiologically, findings were consistent with posterior reversible encephalopathy syndrome. A brain biopsy revealed evidence of endothelial activation, T-cell trafficking, and vascular endothelial growth factor expression, suggesting that systemic immune system activation may be involved with triggering posterior reversible encephalopathy syndrome. In addition, underlying cerebral amyloid angiopathy may have contributed to the initial nonclassical edema distribution by compromising autoregulatory blood flow mechanisms.
Asunto(s)
Encefalopatías/diagnóstico por imagen , Edema Encefálico/diagnóstico por imagen , Angiopatía Amiloide Cerebral/diagnóstico por imagen , Encefalopatía Hipertensiva/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Factor A de Crecimiento Endotelial Vascular/metabolismo , Anciano , Biopsia , Encefalopatías/inmunología , Encefalopatías/metabolismo , Encefalopatías/patología , Edema Encefálico/inmunología , Edema Encefálico/metabolismo , Edema Encefálico/patología , Angiopatía Amiloide Cerebral/inmunología , Angiopatía Amiloide Cerebral/metabolismo , Angiopatía Amiloide Cerebral/patología , Comorbilidad , Humanos , Encefalopatía Hipertensiva/inmunología , Encefalopatía Hipertensiva/metabolismo , Encefalopatía Hipertensiva/patología , Masculino , SíndromeRESUMEN
BACKGROUND: The development of central nervous system (CNS) symptoms in patients with preexisting systemic lupus erythematosus (SLE) evokes a wide differential diagnosis. Reversible posterior leukoencephalopathy (RPLE) is a rapidly evolving neurologic syndrome with characteristic clinical and radiographic features. Conditions commonly associated with RPLE include hypertensive encephalopathy, eclampsia, immunosuppressive drugs, and inflammatory disorders. OBJECTIVES: To describe our experience with RPLE in patients with concomitant SLE and review the literature. METHODS: The details of 5 novel cases and a MEDLINE review of the literature concerning the development of RPLE in association with SLE are presented. RESULTS: All cases included patients with SLE who developed the acute onset of headache, altered mental status, visual changes, and seizures. Neuroimaging demonstrated posterior white matter edema involving the parietal, temporal, and occipital lobes. Complete clinical and radiographic recovery occurred with prompt antihypertensive treatment and supportive care. Literature review identified 16 additional cases of RPLE occurring in patients with active SLE; the majority of these reports was similar in presentation and outcome to our experience. CONCLUSIONS: It is likely that the clinical manifestations and neuroimages in these lupus patients were the result of the RPLE syndrome. Fortunately, this cause of "secondary" CNS symptoms in patients with SLE is readily reversible when diagnosed early and treated with blood pressure control and supportive care.