Standardized neurologic evaluations of 128 patients with Wegener granulomatosis

Objective: To assess the frequency and type of neurologic involvement in a cohort of patients with generalized Wegener granulomatosis (WG). Patients and Methods: In a prospective analysis the clinical, electrophysio logic, radiological, and serologic data of 128 patients have been studied o ver a median observation period of 19 months (range, 1-60 months). Results: Sixty-four patients (50%) revealed central or peripheral nervous s ystem involvement. Peripheral neuropathy (PN) affected 56 patients, in 9 ca ses the central nervous system was involved, and in 6 cases the cranial ner ves were involved. Thirty-one patients showed a distal symmetrical polyneur opathy, 25 a mononeuritis multiplex. Within the first 2 years of the diseas e course 47 of the 56 patients had developed their PN, sometimes as the ini tial symptom of WG. Patients with PN were significantly more often male (34 of 65 patients) than female (22 of 63 patients, P=.04), were significantly older at the onset of WG (median age, 53 vs 44 years; P=.001), had a signi ficantly larger disease extent (P=.001), and had higher classic antineutrop hil cytoplasmic antibody titers (P=.002) than neurologically unaffected pat ients. Response to immunosuppression was moderate concerning peripheral ner vous system manifestations. Conclusions: Peripheral neuropathy is frequent in generalized WG, occurring early in the disease course. As PN can be the first and sole symptom of a beginning systemic vasculitis, it is important that in cases of PN of an un clear origin, interdisciplinary investigations are initiated to detect, tre at, and closely follow-up a possible underlying WG, especially as these pat ients seem to have a more severe disease course.