Complement factor I deficiency with recurrent aseptic meningitis

Abstract

Patients with deficiency of the complement regulatory protein factor I typically present with systemic pyogenic bacterial infections, including meningitis. We report a novel case with total deficiency of factor I in serum and plasma; the patient experienced nine consecutive episodes of aseptic meningitis within a 2-year period. There was no history of previous bacterial sepsis. Aseptic meningitis recurred despite attempted penicillin prophylaxis. Each episode resolved rapidly without sequelae, with or without antibiotic treatment. Serum complement profiles showed persistently low levels of C3, factor B, and factor H and undetectable factor I protein. Family complement studies could not be performed. Except for a minimally increased titer of antinuclear antibody, no other immunologic abnormality was detected. Results of an oral ibuprofen challenge were negative. We conclude that deficiency of factor I may predispose to aseptic, as well as pyogenic bacterial, meningitis.