An 18-year-old man with Goodpasture's syndrome and ANCA-negative central nervous system vasculitis.

An 18-year-old man with Goodpasture's syndrome developed a recurrent seizure disorder refractory to anticonvulsant therapy spanning a full course of plasmapheresis and cytotoxic therapy. Status epilepticus developed during a taper of cytotoxic agents, with magnetic resonance imaging (MRI) findings consistent with multiple lacunar infarcts. Although the diagnosis of vasculitis was considered, anti-neutrophilic cytoplasmic antibody serology was consistently negative. A meningeal biopsy showed vasculitis, which was successfully treated with the reintroduction of high-dose steroids and cytotoxic agents in conjunction with anticonvulsants. Nearly 1 year later, with Goodpasture's disease in remission, he received a living-related renal transplant from his mother. He is currently leading a normal lifestyle. From an extensive review of the literature, this is the first case of anti-neutrophil cytoplasmic antibody-negative central nervous system (CNS) vasculitis in a patient with Goodpasture's syndrome.