Posterior Reversible Encephalopathy Syndrome in a 12-Year- Old Boy with Mixed Connective Tissue Disease

Abstract

Reversible posterior encephalopathy syndrome is a clinicoradiologic entity characterized by headaches, altered mental status, seizures, and visual loss and is associated with white matter vasogenic edema predominantly affecting the posterior occipital and parietal lobes of the brain. We herein, present a rare complication of mixed connective tissue disease. A 12-year old boy presented with generalized tonic-clonic fits, aphasia, and fever and generalized body swelling since 2 weeks. His labs showed hypercholestremia, hypoalbuminemia and massive proteinuria. Renal biopsy revealed secondary membranous glomerulopathy and he developed posterior reversible encephalopathy syndrome. His anti RNP and anti-smooth muscle came back positive. Evaluation by magnetic resonance scan of the brain showed that the child had hyper intense signal in the parie¬to-occipital regions. All signs of PRES started resolving after seven days of supportive treatment evidenced by subsequent radiological evaluation. PRES and secondary membranous glomerulopathy should be kept in mind in any connective tissue disease who is on prolonged immunosuppressive therapy.