Manifestaciones neurológicas de la granulomatosis de Wegener: Caso clínico

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 rep...

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Autores principales: López-Rodríguez,Raimundo, García-González,Jorge, Campos-Franco,Joaquín, Mallo-González,Nieves, Alende-Sixto,María Rosario
Lenguaje:Spanish / Castilian
Publicado: Sociedad Médica de Santiago 2007
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Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872007000700012
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Sumario: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 ¡eft eye amaurosis associated to mass located in the ¡eft 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