The upper thoracic spinal cord compression as the initial manifestation of Wegener's granulomatosis: a case report.

There are only a few reported cases about spinal cord involvement with Wegener's granulomatosis (WG) in the literature. In these cases, the spinal cord is usually indented or compressed by dural and meningeal masses which are characterized by necrotizing granuloma formation and vasculitis. And, it usually cannot be correctly diagnosed. A 53-year-old woman suffered from Wegener's granulomatosis, in whom the upper thoracic spinal cord compression is the initial manifestation. The surgical biopsy and thoracic laminectomy were performed and the histologic examination was done. This patient was finally diagnosed as WG when the pathologic examination revealed as Wegener's granulomatosis and the serum antineutrophil cytoplasmic antibodies (ANCA) were reported positive; titers of antimyeloperoxidase (MPO) antibodies were markedly elevated. After treatment with cyclophosphamide and corticosteroids this patient partially recovered from neurological involvement. In a case such as this, careful monitoring of clinical parameters is essential for assessing disease activity with repeated MRI if neurologic status changes. Serial measurement of ANCA titers may also be helpful to establish the diagnosis. Cyclophosphamide and corticosteroids are the agents of choice for induction of remission of WG.