An atypical rare neurologic complication of histiocytic necrotizing lymphadenitis (Kikuchi–Fujimoto Disease)

Kikuchi–Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare disease first reported in 1972 by Kikuchi and Fujimoto et al . It is more common in young females under 30 years of age. It is a self-limited disease characterized by cervical lymphadenopathy. Headache accompanied by fever is a common symptom of this disease; however, the central nervous system (CNS) can also be involved. CNS involvement in KFD is extremely rare and remains a diagnostic challenge. We describe a KFD patient with aseptic meningitis who had a headache as the first symptom of aseptic meningitis. Only 41 cases of aseptic meningitis associated with KFD have been reported worldwide, with just five cases (including our case) of KFD with meningitis as the first symptom. We report a case of KFD accompanied by aseptic meningitis with leptomeningeal enhancement.