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- W1991528982 abstract "Guillain–Barre syndrome (GBS) is a rare but life-threatening polyneuropathy. We describe the novel use of eculizumab in a patient with this disorder. Case A previously well 13 year old boy presented with an acute onset of ascending paralysis and was diagnosed with GBS. Despite receiving intravenous immunoglobulins on Day 3 of presentation, he continued to deteriorate and required ventilatory support and a subsequent tracheostomy. He remained non-responsive to a further course of 2 g/kg intravenous immunoglobulin. His GBS was complicated by severe life-threatening autonomic instability so plasmapheresis was not felt to be a suitable option. A single dose of eculizumab was given, after which the patient showed signs of improvement. He continued to convalesce over the next 8 weeks, at which stage he was mobilising independently. Discussion GBS poses a mortality rate of 10% despite medical advances. Intravenous immunoglobulins and plasmapheresis have been shown to improve outcome but beyond these, management is mainly supportive at present. Eculizumab, a monoclonal antibody, is currently used in patients with other immunological disorders but not GBS. Recent animal studies suggest that complement activation may play a role in the development of paralysis in GBS. Complement inhibitors such as eculizumab have been shown to have promising benefits in murine models. Further research is necessary but we believe eculizumab may have a role in improving outcome, especially in those with severe GBS. As far as we are aware, this is the first case report in literature of eculizumab being used in a patient with GBS. Our case suggests potentially favourable outcomes and clinical trials are needed to assess this further." @default.
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- W1991528982 date "2014-10-01" @default.
- W1991528982 modified "2023-09-27" @default.
- W1991528982 title "G.P.299" @default.
- W1991528982 doi "https://doi.org/10.1016/j.nmd.2014.06.389" @default.
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