VASCULITIS CONFINED TO PERIPHERAL-NERVES

The clinical, electrophysiological and pathological features and progn osis of 25 patients with vasculitis selectively affecting the peripher al nervous system were evaluated. Although most patients had a history of mononeuritis multiplex or an asymmetrical neuropathy six out of 25 had a symmetrical neuropathy, both clinically and on neurophysiologic al testing, by the time of presentation. There were no signs of accomp anying systemic vasculitis in any of the patients and serological abno rmalities were limited to an elevated erythrocyte sedimentation rate ( ESR) in nine out of 21 patients and low titre anti-nuclear antibodies in four out of 20 patients. Most patients had a necrotizing vasculitis on nerve biopsy, although in some cases the diagnosis was made on the association of inflammatory cell infiltrates with extensive axonal de generation and immune complex deposition on immunofluorescence studies . The mean time from symptom onset to diagnosis was 46 weeks. All pati ents were treated with corticosteroids and most with additional immuno suppressive therapy In contrast to vasculitic neuropathy associated wi th systemic vasculitis the prognosis was good with 24 out of 25 surviv ors at a mean of 176 weeks follow-up having a mean improvement of 1.4 units on a six-point disability scale.