Inflammatory demyelinating neuropathies

Abstract

The primary goal of therapy in patients with the Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is improved strength and functional ability. Improvement in pain, sensory loss, gait disorder, and autonomic instability are other goals of therapy. Patients with very mild symptoms that do not interfere with activities of daily living can be observed for deterioration without treatment. For GBS, standard care includes plasma exchange (PE) or human immune globulin (HIG), both of which have similar efficacy. Supportive care in the intensive care unit may be needed for those patients with severe bulbar or respiratory weakness. We treat most patients with PE, usually performing an exchange every other day for a total of five exchanges. We use HIG in children, if there are antiglycolipid antibodies (eg, anti-GM1 or anti-GQ1b) or if there is a contraindication to PE, such as hemodynamic instability; severe renal, hepatic, or cardiac disease; or poor venous access. For CIDP, there are no guidelines concerning the initial choice of therapy. Corticosteroids, HIG, and PE have all been shown to be effective in prospective, randomized controlled trials, and comparison trials have shown equal efficacy among these three immunomodulating therapies. The choice of therapy depends on several factors including disease severity, concomitant illnesses, side-effect profile, potential drug interactions, venous access, age-related risks, and cost of treatment. In patients with moderate to severe symptoms, treatment with corticosteroids or HIG should be used. We usually use high-dose, intermittent methylprednisolone as the initial drug of choice. We believe intermittent corticosteroids are better than HIG because of their good safety profile, low cost, ease of administration (can be given intravenously or by mouth), and proven efficacy. If there is a major contraindication to corticosteroids, then HIG is offered. PE is less well tolerated and is primarily used as a third choice and only for a few weeks to months to induce initial improvement. Once symptoms are improving, the dose of corticosteroids or HIG should be tapered with the goal of eventual discontinuation depending on patient response. Patients who do not respond to initial therapy, experience adverse effects from the initial immunomodulating agent, or require chronic treatment can be treated with another first-line agent or one of several second-line agents.