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[Neurological involvement in Wegener's granulomatosis: report of one case]
Raimundo López-Rodríguez1, Jorge García-González, Joaquín Campos-Franco
1Servicio de Medicina Interna, Complejo Hospitalario Universitario de Santiago de Compostela, Santiago de Compostela, España. lopezrodriguez.rm@gmail.caom
Abstract:
Wegener's granulomatosis is a systemic necrotizing vasculitis that affects medium size and small vessels. Neurological involvement occurs in 22% to 54% of patients, mainly in the form of mononeuritis multiplex. Central nervous system involvement is reported in only 2% to 8% of the cases. We report a 42-year-old male who presented with headache, diplopia, third and sixth cranial nerve palsies and left eye amaurosis associated to mass located in the Meckel cavum and diffuse meningeal involvement. A biopsy of the mass disclosed a chronic granulomatous necrotizing inflammation with Langhans giant cells. A chest CAT scan showed three cavitated lung nodules and ANCA antibodies were positive in a titer of 1:80. Treatment with steroid and cyclophosphamide was started and cranial nerve palsies resolved and the number and size of lung nodules decreased. The patient was lost from follow up.