Cranial Nerve Injury as an Initial Sign of Wegener Granulomatosis: Misdiagnosis and Fatal Course in Two Cases

Abstract

Highest prevalence of all antineutrophil cytoplasmic antibody associated systemic vasculitides is the Wegener granulomatosis, and it’s typically characterized by a necrotizing granulomatous vasculitis of the respiratory tracts, kidneys, and skin. Neurologic involvement in Wegener granulomatosis ranges from 22% to 54%, but central nervous system involvement is from 2% to 8%. Cranial nerve involvement as an initial sign with concomitant systemic disease is extremely rare. Involvement of one or more cranial nerves is generally a seemingly isolated finding in Wegener granulomatosis. We present WG cases that presented with treatment-resistant cranial nerve involvement and acute fatal pulmonaryrenal syndrome.