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Home arrow Environment arrow Inflammatory Disorders of the Nervous System: Pathogenesis, Immunology, and Clinical Management
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Immunomodulatory Treatments

GBS was associated with mortality in 10% of patients and severe residual neurological deficit in 20 of cases before the introduction of immunotherapy [54]. As detailed below, immunomodulatory treatments directed at removal (plasma exchange (PLEX)) or modulation of immunoglobulins and probably T cell responses (intravenous immunoglobulins (IVIG)) have been proven to be effective in GBS. In contrast to many other autoimmune neurological diseases, steroids have not shown to hasten recovery nor affect the long-term outcome [55], and their use is not recommended in GBS, neither alone nor combined with PLEX or IVIG [1, 2].

Plasma Exchange (PLEX)

The immunomodulatory action of PLEX is through the removal of autoantibodies and complement components. It is usually administered at five plasma volume exchanges (50 ml/kg each) usually every other day, over a period of up to 2 weeks [56, 57]. PLEX is more effective if done early in the course of the illness, preferentially the first week after the onset of symptoms [58]. However, larger exchanges of 1.5 plasma volumes have also been used. Hughes et al. reviewed four clinical studies involving 585 severely affected GBS patients and concluded that there is significant improvement and less disability in the treated patients after 4 weeks and 1 year after of randomization [56-60]. The treated patients also had a higher chance of full strength recovery (odds ratio 1.24, confidence interval 1.07-1.45), as well as lower disability and higher likelihood for full recovery in 1 year [59]. In milder GBS patients who did not lose the ability to ambulate, patients who received two sessions of PLEX over 3 days had shorter onset of motor recovery (4 vs. 8 days) and better improvement after 1 month compared to those who did not receive PLEX [57]. On the other hand, in GBS patients who could not stand unaided, there was a higher likelihood of regaining full motor strength in 1 year after four sessions of PLEX (x1.5 plasma volume each) than after two sessions (64% vs. 48%) [57]. Six exchanges were similar in efficacy to four in the severe GBS cases in the latter study.

 
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