ABSTRACT
Immune-mediated polyneuropathies are disorders of the peripheral nervous system, leading to muscle weakness and sensory disturbances. Most studies have been performed in patients with Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). GBS and CIDP have a wide range of variation in symptoms, severity and duration of progression1,2. GBS and CIDP seem to be variants of one disorder, with the very acute GBS patients on the one hand and slowly progressive CIDP patients on the other side of the clinical spectrum. Treatment trials have investigated whether prednisone, plasma exchange (PE) or intravenous immunoglobulin (IVIG) are effective. Recent trials have evaluated different dosage schedules of one type of treatment or have studied combinations of treatments. All major randomized controlled trials have been performed in adults. IVIG currently remains a cornerstone in the treatment of GBS, and in many centers also in CIDP. Studies of the mechanism of action of these various treatments in GBS and CIDP remain scarce. GBS is a disorder in which infections, antiganglioside antibodies and molecular mimicry may cause or at least contribute to the development of the disorder3. It is expected that PE at least removes possible pathogenic antibodies or other circulating agents, and IVIG interferes with either antibody production or function. Clinical indications and the effect of IVIG treatment are discussed for GBS and CIDP.
