Aseptic meningoencephalitis in primary Sjögren's syndrome. Response to plasmapheresis and absence of CNS vasculitis at autopsy.

It has been suggested that cerebral vasculitis is the pathogenetic mechanism of the central nervous system manifestations of primary Sjögren's syndrome. We present a fatal case of aseptic meningoencephalitis in an 18-year-old woman with primary Sjögren's syndrome in whom there was no evidence of cerebral vasculitis at autopsy. On two occasions her condition improved markedly following plasmapheresis.