Electrodiagnostic characteristics of Wegener's granulomatosis-associated peripheral neuropathy.

Abstract

Wegener's granulomatosis is a multisystem disease distinguished by a triad of necrotizing granulomatous vasculitis involving the upper and lower respiratory tracts, glomerulonephritis and systemic, small vessel vasculitis. The latter can cause neurologic manifestations when the vasa nervorum are affected. A 53-yr-old male presented with a 3-mo history of chronic nasal congestion, arthralgias, pruritic maculopapular eruption, epistaxis and lower extremity weakness. Subsequent lung and chest wall biopsies confirmed diagnosis of Wegener's granulomatosis. Summary of electrodiagnostic data obtained on initial presentation and comparison with later study indicated a sensorimotor polyneuropathy with wide-spread axonal involvement noted particularly in the distal lower extremity musculature. Electrodiagnostic documentation of rapid progression proved useful in directing alteration of immunosuppressive therapy, with favorable clinical and functional outcome. We believe this is the second case presented of a patient with documented Wegener's granulomatosis and overt clinical evidence of poly-neuropathy in whom both electroneurographic and electromyographic studies are described. Electrodiagnostic results are presented with discussion of pertinent literature.