Standardized Neurologic Evaluations of 128 Patients With Wegener Granulomatosis

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Abstract
WEGENER granulomatosis (WG) has the highest incidence of all antineutrophil cytoplasmic antibody (ANCA)–associated systemic vasculitides and is characterized by necrotizing small vessel vasculitis and granulomatous lesions.1 The reported frequency of neurologic involvement in WG ranges from 22% to 54%2-5 and clinical manifestations are variable. Drachman4 had already recognized the following 3 pathophysiologically relevant processes: (1) contiguous spread of granulomatous disease from the ear, nose, and throat tract into the brain and cranial nerves; (2) formation of granuloma primarily in the nervous system; and (3) vasculitis affecting the central nervous system (CNS) and/or the peripheral nervous system.