Epilepsy: an anticipatory presentation of pediatric Wegener's granulomatosis

Wegener's granulomatosis is a necrotizing, granulomatous vasculitis that primarily affects the respiratory tract and kidneys. It is rare in children. Few pediatric and adult case reports described seizures and central nervous system involvement at initial presentation, and none described central nervous system involvement in the absence of respiratory or renal disease. We describe a 17-year-old girl with secondarily generalized seizures and granulomatous lesions in her brain. Although a systemic inflammatory disorder was suspected, she lacked evidence of pulmonary or renal involvement, so her signs were initially labeled as postinfectious encephalitis or variant acute disseminated encephalomyelitis. After 1 year of immunosuppressive therapy, she developed additional signs, leading to histologic confirmation on lung biopsy of Wegener's granulomatosis. This case emphasizes the need for close follow-up to monitor the asynchronous development of diagnostic clues when a systemic vasculitis is suspected but cannot be confirmed.