Title:
Granulomatosis with polyangiitis and multiple bilateral cranial nerve palsies: a diagnostic challenge
Authors:
J. Kristin, C. Beutner, T. Klenzner and F. B. Knapp
Institutions:
Department of Otorhinolaryngology, Head and Neck Surgery, University of Duesseldorf, Duesseldorf, Germany
Keywords:
Wegeners granulomatosis; c-ANCA; cranial nerve palsies
Pages:
231 - 235
Abstract:
Granulomatosis with polyangiitis and multiple bilateral cranial nerve palsies: a diagnostic challenge. Background: Granulomatosis with polyangiitis is characterized by vasculitis of small and medium sized vessels and non- caseating granulomas with head and neck symptoms in 95% of those affected. Cranial nerve palsies are rare; while, chronic rhinosinusitis and ear problems are common. Case report: We describe the serious course and the diagnostic challenge of a patient with granulomatosis with polyangiitis of bilateral mastoids and the right temporal lobe. Initially, the patient showed metachronous bilateral facial palsy with chronic mastoiditis. Repeated surgeries and rheumatologic examinations did not determine a diagnosis. The patient developed additional cranial nerve palsies. Due to progression into the temporal lobe, we removed the affected parts. After 6 months, the diagnosis was revealed by histology. Results and conclusion: Granulomatosis with polyangiitis is a diagnostic challenge. Persistent reevaluations were necessary for a final diagnosis and to limit the life-threatening disease. Once diagnosed, therapy began with the standard FAUCI-Scheme.
Issue:
Vol. 10, 2014, 3rd trimester


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Granulomatosis with polyangiitis and multiple bilateral cranial nerve palsies: a diagnostic challenge