Four cases had TDP-43–positive glial inclusions.8,9,17,23 The other 2 cases had no intraneural inclusions and stained negative for ubiquitin.1,5 Cutaneous nerve and muscle biopsies show a loss of myelinated fibers and Wallerian degeneration of the nerves without signs of vasculitis or amyloid deposition.1,4,5,17,20,21 Genetic analysis was positive in 7 cases. The gene discussed is TARDBP; the disease is vasculitis.