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Plasmapheresis treatment in Guillain-Barre syndrome: potential benefit over intravenous immunoglobulin.

Acquired acute demyelinating peripheral polyneuropathy is a general classification of pathologies that affect the peripheral nervous system. They are characterised by an autoimmune process directed towards myelin. Clinically they are characterised by progressive weakness and mild sensory changes. Acute inflammatory demyelinating polyneuropathy is often referred to as Guillain-Barre syndrome (GBS). GBS includes acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, acute motor and sensory axonal neuropathy, Miller Fisher syndrome and acute pandysautonomia (1). GBS is the major cause of acute non-traumatic paralysis in healthy people and it is caused by autoimmune response to viral agents (influenza, coxsackie, Epstein-Barr virus or cytomegalovirus) or bacterial infective organisms (Campylobacter jejuni, Mycoplasma pneumoniae).

GBS is clinically described as an ascending and progressive muscular weakness with areflexia, which progresses over a few days to a few weeks. A detailed history, the symptoms and the typical demyelinating electromyography pattern supports the diagnosis. Progressive involvement of respiratory muscles and autonomic instability coupled with a protracted and unpredictable recovery normally results in the need for intensive care unit management.

GBS has an incidence of about 1 in 100,000 and is found to occur in both genders and all ages. Despite medical treatment, GBS often remains a severe disease; 3 to 10% of patients die and 20% are still unable to walk after six months; in addition, many patients have pain and fatigue that can persist for months or years (2).

Plasma exchange (PE) was the first treatment in GBS proven to be superior to supportive treatment alone but intravenous immunoglobulin (IVIg) is commonly used as first-line effective treatment (3). IVIg and PE have a similar ability to hasten recovery from GBS and the largest GBS trial conducted did not show significant differences between IVIg, PE or PE followed by IVIg (4,5). However it is not known if those patients who failed IVIg treatment would subsequently benefit from PE.

CASE HISTORY

We describe a case of a 78-year-old woman who presented with a two-day history of progressive general weakness and left facial nerve palsy, preceded by a flu-like illness lasting for one week. A provisional diagnosis of idiopathic facial paralysis (Bell's palsy) was made and the patient was discharged home after reassurance and no specific medications. One day later she returned with paralysis involving both sides of the face. During this hospitalisation she also developed severe pulmonary dysfunction and was transferred to the intensive care unit where she was intubated (despite the absence of significant abnormalities in the chest X-ray, arterial blood gases performed on passive oxygen therapy showed Fi[O.sub.2] 50%, [Po.sub.2] 84 mmHg, [Pco.sub.2] 36.6 mmHg) and mechanical lung ventilation was commenced.

Neurological examination revealed an absence of deep tendon reflexes, all other cranial nerves were intact and plantar reflexes were flexor throughout. Blood tests for full blood count, urea and electrolytes, serum angiotensin converting enzyme and a vasculitic screen were within normal limits. Blood culture was negative. No paraproteins were detected on serum protein electrophoresis. Cranial tomography was normal. There was characteristic slowing of conduction in peripheral nerves with axonal involvement shown on electromyography. Cerebrospinal fluid (CSF) examination showed 7 leucocytes/mm3 (100% lymphocytes), a protein level of 0.29 g/l (normal range: 0.020 to 0.045 g/l), an albumin level of 0.172 g/l (serum albumin was 2.4 g/l) and the CSF/serum albumin index increasing.

GBS was diagnosed based on the patient history and typical CSF findings. In 1983 she had suffered GBS and was successfully treated with PE; her brother had also presented with GBS after a flu vaccination at the age of 78 years, with a good response to PE. After neurological advice, a five-day course of daily IVIg (0.4 g/kg/day) was commenced without benefit but with a progressive clinical deterioration. Seven days after completion of IVIg treatment, PE was started with an exchange of about three litres of plasma every day for three days and every second day on two occasions (a total of five PE were performed). A gradual improvement of respiratory function and peripheral strength were observed after the first PE. Mechanical ventilation was weaned and she was extubated on the eighth day and she started to walk with assistance.

DISCUSSION

PE was the first treatment in GBS proven to be superior to supportive treatment alone. It reduces the time to independent locomotion by approximately one month when applied within the first two weeks of disease (6,7). The first randomised controlled trial of the use of IVIg was published in 1992 and showed that IVIg is as effective as PE (4). Since the publication of these results, IVIg in a regimen of 0.4 g/kg bodyweight daily for five consecutive days, has replaced PE as the preferred treatment in many centres, mainly because of its greater convenience and availability.

Is there any other evidence showing greater or less benefit from one or the other treatment? The Cochrane review on the use of IVIg in GBS contained four trials: no difference was found between IVIg and PE with respect to the improvement in disability grade after four weeks, the duration of mechanical ventilation, mortality or residual disability (8). The combination of PE followed by IVIg is not significantly better than PE or IVIg alone (4). A study in a small series of patients investigated the effect of a second course of IVIg in severe unresponsive patients with GBS. This uncontrolled study suggested that a repeated course of IVIg could be effective (9).

The use of PE after IVIg treatment has received little attention; according to some authors, PE after IVIg is not advised because PE would probably wash out the IVIg previously administered (2). On the other hand, a retrospective review of 10 patients with GBS showed that three patients improved with PE after a failing IVIg treatment, and that all these patients had axonal involvement on electromyogram (10). A further report describes a 25-year-old male patient with GBS who, despite the IVIg infusion, manifested a rapid disease progression and obtained a gradual improvement of respiratory function and mobility only after six weeks of PE treatment (11). Furthermore severe familial and recurrent forms of GBS, as in the case presented, seem to present with more dramatic symptoms which made them responsive to PE but not to IVIg (12,13).

Based on the current evidences and on the present case we propose that patients with severe GBS should still be considered for PE for three reasons: the two treatments are of equal efficacy but IVIg may fail and the clinical condition deteriorates rapidly; PE does not necessarily affect the efficacy of IVIg; and GBS could be of the recurrent or familial form with axonal involvement and less responsive to IVIg.

Accepted for publication on August 13, 2009.

REFERENCES

(1.) Finsterer J. Treatment of immune-mediated, dysimmune neuropathie. Acta Neurol Scand 2005; 112:115-125.

(2.) van Doorn PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Guillain-Barre syndrome. Lancet Neurol 2008; 7:939-950.

(3.) Cattano D, O'Connor B, Shakir R, Giunta F, Palazzo M. Acute inflammatory demyelinating polyneuropathy and a unilateral Babinski/plantar reflex. Anesthesiology Research and Practice 2008; doi: 101155/2000/134958.

(4.) van der Meche FG, Schmitz PI. A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barre syndrome. Dutch Guillain-Barre Study Group. N Engl J Med 1992; 326:1123-1129.

(5.) Guillain-Barre Syndrome Trial Group. Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barre syndrome. Lancet 1997; 349:225-230.

(6.) The Guillain-Barre syndrome Study Group. Plasmapheresis and acute Guillain-Barre syndrome. Neurology 1985; 35:1096-1104.

(7.) French Cooperative Group on Plasma Exchange in Guillain-Barre syndrome. Efficiency of plasma exchange in Guillain-Barre syndrome: role of replacement fluids. Ann Neurol 1987; 22:753-761.

(8.) Hughes RAC, Raphael JC, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barre syndrome. Cochrane Database Syst Rev 2006; n/a:CD002063.

(9.) Farcas P, Avnun L, Frisher S, Herishanu YO, Wirguin I. Efficacy of repeated intravenous immunoglobulin in severe unresponsive Guillain-Barre syndrome. Lancet 1997; 350:1747.

(10.) Dada MA, Kaplan AA. Plasmapheresis treatment in Guillain-Barre syndrome: potential benefit over IVIg in patients with axonal involvement. Ther Apher Dial 2004; 8:409-412.

(11.) Szczeklik W, Jankowski M, Wegrzyn W, Krolikowski W, Zwolinska G, Mitka I et al. Acute respiratory failure in patients with Guillain-Barre syndrome and myasthenic crisis treated with plasmapheresis in the intensive care unit. Pol Arch Med Wewn 2008; 118:239-242.

(12.) Kuitwaard K, van Koningsveld R, Ruts L, Jacobs BC, van Doorn PA. Recurrent Guillain-Barre syndrome. J Neurol Neurosurg Psychiatry 2009; 80:56-59.

(13.) Geleijns K, Brouwer BA, Jacobs BC, Houwing-Duistermaat JJ, van Duijn CM, van Doorn PA. The occurrence of Guillain-Barre syndrome within families. Neurology 2004; 63:1747-1750.

S. B. BUZZIGOLI *, M. GENOVESI [[dagger]], P. LAMBELET [[double dagger]], C. LOGI [[section]], S. RAFFAELLI **, D. CATTANO [[dagger][dagger]]

ASL 12 Versilia, Viareggio Hospital, Lido di Camaiore, Italy

* M.D., Director, Anesthesia and Intensive Care Unit.

[[dagger]] M.D., Attending Anesthesiologist.

[[double dagger]] M.D., Director, Department of Internal Medicine.

[[section]] M.D., Neurologist, Department of Medicine.

** M.D., Physician, Department of Transfusional Medicine.

[[dagger][dagger]] M.D., Ph.D., Assistant Professor, Department of Anesthesiology, University of Texas Medical School, Houston, Texas, USA.

Address for correspondence: Dr D. Cattano, Department of Anesthesiology, UTHSC-Houston, School of Medicine, 6431 Fannin, MSB 5.020, Houston, Texas 77030, USA.
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Article Details
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Author:Buzzigoli, S.B.; Genovesi, M.; Lambelet, P.; Logi, C.; Raffaelli, S.; Cattano, D.
Publication:Anaesthesia and Intensive Care
Article Type:Case study
Date:Mar 1, 2010
Words:1564
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