Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 24-29: Polyneuropathies & Mononeuritis Multiplex + Key Features Download Section PDF Listen +++ ++ Clinically similar to Guillain-Barré syndrome but with relapsing or progressive course over months or years and autoimmune dysfunction is generally less common In the relapsing form, partial recovery may occur after relapses, or no recovery between exacerbations Remission may occur spontaneously, but frequently there is progression to severe functional disability Usually ascending, symmetric weakness Paresthesias more variable + Clinical Findings Download Section PDF Listen +++ ++ A symmetric sensory, motor, or mixed deficit, which may be most marked distally or proximally See Guillain-Barré Syndrome + Diagnosis Download Section PDF Listen +++ ++ Electrodiagnostic studies Marked slowing of motor and sensory conduction and focal conduction block Signs of partial denervation may be present due to secondary axonal degeneration Nerve biopsy May show chronic perivascular inflammatory infiltrates in endoneurium and epineurium without accompanying evidence of vasculitis However, normal nerve biopsy or the presence of nonspecific abnormalities does not exclude the diagnosis + Treatment Download Section PDF Listen +++ ++ Corticosteroids Prednisone, 60–80 mg once daily orally for 2–3 mo If no response has occurred despite 3 mo of treatment, a higher dose may be tried In responsive cases, the dose is gradually tapered, but most patients become corticosteroid dependent, often requiring prednisone, 20 mg orally on alternate days, on a long-term basis Cytotoxics Patients unresponsive to corticosteroids may benefit from a cytotoxic drug, such as azathioprine Intravenous immunoglobulin (IVIG) Can be used in place of or in addition to corticosteroids Best used as initial treatment in pure motor syndromes (2 g/kg over 2–5 days followed by 1 g/kg every 3 weeks) A weekly regimen of 0.2–0.4 g/kg of a 20% subcutaneous immunoglobulin solution is an effective alternative but has not been compared directly to corticosteroids or IVIG Plasma exchange When both IVIG and corticosteroids are ineffective, plasma exchange may be worthwhile Rituximab has shown promise