Pediatric Wegener's granulomatosis complicated by central nervous system vasculitis

An unusual case of central nervous system vasculitis in pediatric Wegener's granulomatosis, a rare disorder that infrequently presents during childhood, is reported. A 13-year-old girl with Wegener's granulomatosis, whose initial presentation resembled Henoch-Schonlein purpura, developed recurring seizures. MRI of the brain demonstrated multiple areas of increased signal in the occipital, parietal, and frontal lobes, consistent with central nervous system vasculitis. Although both peripheral and cranial neuropathies have been reported in patients with Wegener's granulomatosis, cerebral vasculitis is unusual, particularly in childhood. This case emphasizes the need to consider Wegener's granulomatosis in the differential diagnosis of both unexplained seizures and central nervous system vasculitis in children with systemic illness.