Skip to Main Content

Key Features

  • 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

  • A symmetric sensory, motor, or mixed deficit, which may be most marked distally or proximally

  • See Guillain-Barré Syndrome

Diagnosis

  • 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

  • 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

  • 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

  • When both IVIG and corticosteroids are ineffective, plasma exchange may be worthwhile

  • Rituximab has shown promise

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.