GBS and CIDP Spectrum and IVIg treatment

Abstract

The spectrum of GBS, CIDP and its subforms is investigated in this thesis. We identified 32 relapsing GBS patients and compared their clinical characteristics with 476 non-recurrent GBS patients. Recurrences occurred more frequently in younger patients, those with milder symptoms and in the Miller Fisher syndrome. While neurological symptoms and signs were often similar, the nature of the preceding event often varied which may indicated that genetic and immunological host factors play an important role in recurrent GBS. We described four patients who had separate episodes of both GBS and CIDP. In 106 GBS patients who received one or more flu vaccinations, none reported a recurrence thereafter, therefore seasonal flu vaccinations seem relatively safe in patients who have had GBS. The second part of the thesis is focussed on the treatment of CIDP. In a randomised controlled trial we found no significant differences between two different immunoglobulins in CIDP. IVIg is an effective first-line treatment since 76% improves significantly to IVIg. Sixteen percent needs only one IVIg course but most patients need IVIg for a long period of time. Of the IVIg non-responders, about three quarters of patients still responds to plasma exchange, corticosteroids or both. CIDP patients with pronounced pain or a difference in weakness between arms and legs are less likely to be IVIg responsive. GBS patients all receive the same arbitrary dose of 2 g IVIg per kg of body weight. Not all patients however show a good recovery. Patients show considerable variability in the increase in serum IgG level two weeks after after this standard dose. Patients with a low increase in serum IgG level two weeks after IVIg have a more severe disease course, and are less likely to be able to walk unaided after six months. Even after adjustment for known prognostic factors a low increase in serum IgG level was independently associated with a worse outcome. This indicates that some GBS patients may benefit from a higher dosage or second course of IVIg. We also investigated serum IgG levels in clinical stable but IVIg dependent CIDP patients . All received an individually optimised dosage and interval, which did not correlate with body weight. Clinical stable CIDP patients seem to show a steady-state in serum IgG after serial IVIg infusions