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Spring 2009







Prognosis and New Treatment Studies in Guillain-Barre Syndrome
Dr. Bart C. Jacobs Department of Neurology and Immunology Erasmus MC, Rotterdam, The Netherlands

The acute phrase of the Guillain-Barre syndrome (GBS) is an uncertain period for patients and their relatives, but also for treating physicians. Shortly after diagnosis the clinical course of GBS is so variable, that it is difficult to say whether a patient will soon recover or further deteriorate. It is even more difficult to predict the long-term outcome and possible remaining handicap. Such information on the clinical course of individual patients, however, would be highly useful to improve the care and treatment of GBS. New and more effective forms of treatments could be reserved for those patients who need it most, while patients with good prognosis are not exposed unnecessarily to these side effects. Some patient characteristics related to outcome have been identified in previous studies, but only very recently a model was developed that can accurately predict the outcome of individual patients in clinical practice. This so called Erasmus GBS outcome score (EGOS) is based on three simple clinical features available in the acute stage of disease: age, preceding diarrhea and GBS disability score.' This EGOS model, however, needs confirmation and further refinement in an independent study.

At a recent meeting of a GBS/CIDP Foundation International Research Project in January, an initiative was launched to conduct such a new study to better predict outcome in GBS. We aim to include more than 600 patients for a follow-up period of at least 1 year. Such an extensive study is only possible by intensive international collaboration. Help is provided by the members of the Inflammatory Neuropathy Consortium (INC), a unique collaboration between neuromuscular specialists from all over the world. This INC study will hopefully already start this year and will generate the world's largest prospective database on GBS. In addition, blood samples from these patients will be collected that may help to refine the prognostic models. All this information will be helpful to better inform individual patients and their relatives about the prognosis, but also to conduct trials selectively in patients with poor outcome.

Several initiatives for new treatment trials were also presented at the meeting. First, a trial will start to examine whether patients with a severe form of GBS may profit from a second course of intravenous immunoglobulin. Second, a study is planned with a new drug that may prevent complement mediated nerve damage in GBS. Third, a training study will be conducted to try to help GBS and CIDP patients with severe fatigue. Previous studies indicated that physical exercise may be an effective treatment for this disabling complaint. In the new study several regimes of training will be compared to see which one is most effective.
'van Koningsveld et al. A clinical prognostic scoring system for Guillain-Barre syndrome. Lancet Neurol 2007;6:589-594.

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