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Journal of Neurology Neurosurgery and Psychiatry
2003;74:ii9
© 2003
MANAGEMENT OF INFLAMMATORY NEUROPATHIES
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.
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