DISSEMINATED PERIVENOUS NECROTIZING ENCEPHALOMYELITIS IN SYSTEMIC LUPUS-ERYTHEMATOSUS - REPORT OF AN AUTOPSY CASE

The patient, a 22-year-old woman who had been treated for systemic lup us erythematosus (SLE) for 10 years, was hospitalized for arthralgia, melena, and difficulty in walking. CT examination of the brain showed gram-like high-density lesions scattered throughout the cerebral white matter and basal ganglia. At autopsy, multiple perivenous, well-demar cated foci of brownish discoloration were seen scattered throughout th e cerebral white matter and basal ganglia. Histopathologically these l esions consisted of foci of coagulation necrosis surrounding the veins . The veins in the foci showed fibrous thickening of the walls, but th ere were no indications of vasculitis. At the periphery of the lesions , the axons were better preserved than their myelin sheaths. The neuro pathological findings in the present case closely resemble those of ac ute disseminated (perivenous) encephalomyelitis, although an inflammat ory cell infiltration had apparently already subsided. Although its pa thogenesis remains unclear, this finding should not be regarded as an incidental complication but rather as a rare subtype of central nervou s system lesion occurring with SLE.