Manifestaciones neurológicas de la granulomatosis de Wegener: Caso clínico / Neurological involvement in Wegener's granulomatosis: Report of one case

ABSTRACT

Wegener's granulomatosis is a systemic necrotizing vasculitis that affects medium size and small vessels. Neurological involvement occurs in 22 percent to 54 percent of patients, mainly in the form of mononeuritis multiplex. Central nervous system involvement is reported in only 2 percent to 8 percent of the cases. We report a 42-year-old male who presented with headache, diplopia, third and sixth cranial nerve palsies and íeft eye amaurosis associated to mass located in the íeft 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 180. 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.