Seizure as a unique presentation of pediatric wegener's granulomatosis

Introduction: Wegener's Granulomatosis (WG) is a necrotizing, granulomatous vasculitis that is rare in children. Patients with WG may develop nervous system involvement; however central nervous system (CNS) symptoms are rarely the initial presenting problem. Case: A previously healthy 17 year old girl presented with headaches and secondarily generalized seizures. Magnetic resonance imaging of the brain revealed right frontal and right temporal lobe enhancing lesions. An extensive infectious, immunologic, and rheumatologic work-up revealed no identifiable cause. A chest x-ray and a chest computed tomography scan (CT) showed no evidence of parenchymal or airway disease. A needle biopsy of the right frontal lobe lesion revealed extensive perivascular and parenchymal lymphocytic and plasma cell inflammation with focal granulomas. Microglial activation and reactive astrocytes were present suggesting a chronic process. The pathological impression was an autoimmune granulomatous disorder. She lacked evidence of pulmonary or renal involvement or other laboratory support, so the diagnosis of post-infectious encephalitis or variant acute disseminated encephalomyelitis was made. Over the next year she was treated with corticosteroids and mycophenolate mofetil. On this therapy she developed cough, chest pain, bleeding gums, and a vasculitic rash. Chest CT showed multiple necrotic pulmonary nodules throughout the right lung and two cavitary lesions in the left lung (Image 1). Lung biopsy revealed ill-defined granulomas containing histiocytes, multinucleated giant cells, lymphocytes, neutrophils, eosinophils, and plasma cells; central necrosis with suppurative infiltrate and vasculitis which was suggestive of WG (Image 2). Skin biopsy also supported the diagnosis of WG. Infectious and immunologic studies, including ANCA, were negative. C-reactive protein and ESR were elevated at 20.8 mg/L (0-8 mg/L) and 38 mm/h (0-20 mm/h), respectively. Treatment of WG was initiated, including cyclophosphamide. After three months, repeat imaging showed a decrease in all lesions/nodules and all clinical symptoms had resolved. Discussion: (1) CNS involvement in WG is uncommon. Seizure as a primary symptom of WG has been reported twice in the literature, once in a child and once in an adult. (2) CNS vasculitis due to WG is a diagnostic challenge as there are no features that uniquely distinguish it from other inflammatory lesions. ANCA testing can be useful in establishing the diagnosis of WG; however, our patient was c-ANCA negative throughout her course. (3) Features of rheumatologic diseases can develop asynchronously, so it is important to maintain a high index of suspicion with close follow-up as the underlying process likely will reveal itself in time. (Figure presented) Lung biopsy: Note vasculitis (top left), suppurative necrosis (center) and granulomatous inflammation (lower right). Hematoxylin and eosin stain, original magnification X100. Compatible with Wegener's Granulomatosis.