Posterior Reversible Encephalopathy Syndrome in a Patient with Microscopic Polyangiitis (P01.127)

Abstract

Objective: To discuss neurologic manifestations of microscopic polyangiitis (MPA) vasculitis. Background Central nervous system manifestations of MPA are uncommon. Only two cases of posterior reversible encephalopathy syndrome (PRES) in patients with MPA have been reported. Design/Methods: A case report. Setting: A tertiary care center. Patient: A 69-year old man with chronic kidney disease of unknown etiology came to the ED with new-onset right foot numbness. He was febrile and normotensive. Labs revealed a leukocytosis, anemia and an elevated creatinine. Urinalysis showed 21-30 wbcs, rbcs too numerous to count, 3+ protein, and 3+ blood. CT head was unremarkable. A diagnosis of MPA vasculitis was made based on serum positivity for myeloperoxidase (MPO)-ANCA and renal biopsy. He received steroids and one dose of cyclophosphamide but had a complicated hospital course, with respiratory distress requiring intubation and renal failure requiring CVVH followed by hemodialysis. On Day 28 he went from interactive and able to follow commands to acutely delirious. Hours later he had a generalized seizure and went into status epilepticus. CT scan revealed extensive edema bilaterally in the posterior portions of both hemispheres, raising concern for PRES. CT on hospital day 35 was again consistent with PRES. The patient remained intubated and sedated and was unable to recover. On Day 43, the patient9s family opted for terminal extubation. Results: Our diagnosis was PRES as indicated by the CT scans. Conclusions: The pathophysiology of PRES in patients with MPA vasculitis is unclear. Endothelial damage might predispose to PRES by increasing blood-brain barrier permeability. Our patient had renal failure and was immunosuppressed, which may have also contributed to his development of PRES. MPA itself, the renal failure that it may induce, and the agents used to treat it all may be associated with the development of PRES. Disclosure: Dr. Minen has nothing to disclose. Dr. Gold has nothing to disclose. Dr. Badjatia has nothing to disclose.