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Journal of Neurology Neurosurgery and Psychiatry 2003;74:ii9 
© 2003


Robert D M Hadden1 and Richard A C Hughes2

Disease modifying treatment
Plasma exchange (PE) was the first disease modifying therapyproven to be superior to supportive treatment alone (fig 1, level 1a evidence). It reduced the median time to regain the ability to walk unaided from 85 to 53 days in one study andfrom 111 to 70 days in another, and improved long term disability at one year. A large French study showed that for mild GBS (patients able to stand unaided but unable to run) two 1.5 plasma volume exchanges were better than none, for intermediate severity four exchanges were better than two, and for ventilated patients six exchanges were no better than four (level 1b evidence). There were more adverse events with fresh frozen plasma as thereplacement fluid than albumin. Plasma exchange is more dangerous in patients with coagulopathy, unstable blood pressure or uncontrolled sepsis.

Variations of plasma exchange have been developed to try toimprove safety. Immunoadsorption selectively removes immunoglobulin without requiring administration of foreign blood products, thereby avoiding risks of infection and allergic reaction, and may be done with columns containing staphylococcal protein A, phenylalanine or tryptophan. In small studies, immunoadsorptionand double filtration plasmapheresis showed no significant difference in outcome compared with PE (level 2b evidence). A small trial of CSF filtration also showed no difference from PE. However, none of these studies were large enough to prove equivalence and use of these alternative treatments is not warranted outside clinical trials.

Intravenous immunoglobulin (IVIg) has become the treatment ofchoice for GBS in most countries. Although it has not been adequately tested against placebo in a randomised trial, it has similar short and long term efficacy to PE (fig 2level 1a evidence)and avoids adverse effects related to hypotension and the requirement for a large venous catheter. It costs about the same as PE in the UK. The conventional dose is 0.4 g/kg/day for five days. In a trial of 39 patients requiring ventilation, six days of 0.4 g/kg/day was more effective than three days (level 1b evidence). Combined PE and IVIg was not significantly better than either alone in one trial.