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WHAT TO DO
patient with clinically typical Guillain-Barre syndrome (GBS) is admitted and given five days of intravenous immunoglobulin (IVIg) at a conventional dose of 0.4 gm/kg per day. His cerebrospinal fluid (CSF) is normal on day
4 of the illness and the nerve conduction studieslimited to conduction velocities in an upper and lower limbcome back as normal. He continues to deteriorate, requiring ventilation. After a further two weeks there is no sign of improvement. What should I do now?
J B Winer
Consultant Neurologist and Honorary Senior Lecturer in Neurology, University Hospital Birmingham, Edgbaston, Birmingham B15 2TH, UK; j.b.winer@bham.ac.uk www.practical-neurology.com
Nerve conduction studies are usually abnormal in GBS, even early in the course of the disease
motor neuron problem in the legs, with only equivocal nerve conduction studies consistent with proximal root damage. The CSF usually helps here but breast carcinoma can present with a normal CSF and a subacute neuropathy that causes confusion. Porphyria or acute toxin exposure are always difficult to diagnose, although a detailed history can help.
Winer 229
some have argued that it is more rational to treat with plasma exchange if the IVIg fails to produce a documented improvement rather than repeat the IVIg. But there is of course the inevitable consequence of washing out the IVIg still present in the blood which is at the very least undesirable and some would argue illogical. Furthermore, plasma exchange has more complications than IVIg even though it was well tolerated in clinical trials; it is also more complex and therefore difficult to deliver to sick patients, especially if they have prominent autonomic failure, which is not uncommon in severe GBS.
WHAT DO I DO?
In practice I usually try and wait for improvement unless there is clear evidence of either continuing deterioration of one disability grade two weeks after treatment, or a relapse of this severity. If this occurs I retreat with a further course of 0.4 g/kg of IVIg per day for a further five days. (I accept that at present there is no formal evidence but this may come in time!) If the patient still does not improve then I reconsider the diagnosis. I have occasionally gone on to treat such patients with plasma exchange. There is no doubt that some patients with CIDP do
Figure Hypothetical course of an attack of Guillain-Barre syndrome. Change in disability grade over weeks.
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PRACTICE POINTS
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REFERENCES
1. Hadden RD, Cornblath DR, Hughes RA, et al. Electrophysiological classification of Guillain-Barre syndrome: clinical associations and outcome. Plasma Exchange/Sandoglobulin Guillain-Barre Syndrome Trial Group. Ann Neurol 1998;44:7808. Hughes RAC, Raphael J-C, Swan AV, et al. Intravenous immunoglobulin for Guillain-Barre syndrome. Cochrane Database Syst Rev 2006;(1):CD002063. Raphael J-C, Chevret S, Harboun M, et al, for the French Guillain-Barre syndrome Study Group. Intravenous immune globulins in patients with Guillain-Barre syndrome and contraindications to plasma exchange: 3 days versus 6 days. J Neurol Neurosurg Psychiatry 2001;71:2358. Kleyweg RP, van der Meche FG. Treatment related fluctuations in Guillain-Barre syndrome after highdose immunoglobulins or plasma-exchange. J Neurol Neurosurg Psychiatry 1991;54:95760. Farcas P, Avnun L, Frisher S, et al. Efficacy of repeated intravenous immunoglobulin in severe unresponsive Guillain- Barre syndrome. Lancet 1997;350:1747. Plasma Exchange/Sandoglobulin Guillain-Barre Syndrome Trial Group. Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barre syndrome. Lancet 1997;349:22530.
IVIg 0.4 gm/kg per day for 5 days is the standard treatment for GBS. A small percentage of patients with GBS will develop CIDP with progressive deterioration or relapses. Treatment-related fluctuation leading to a deterioration of one grade usually responds to re-treatment with the same regime of IVIg. Patients who appear initially not to respond to IVIg may still benefit from that treatment as measured by the time taken to recover. Some patients who fail to respond may benefit from a second course of IVIg, or plasma exchange.
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respond to plasma exchange and not to IVIg and by analogy this might apply to GBS as well. Unfortunately the time window for effective treatment of GBS may be too short for several attempts at treatment.
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ACKNOWLEDGEMENTS
This article was reviewed by Hugh Willison, Glasgow, UK.
10.1136/jnnp.2009.176578