New Study on Patients with Guillain-Barré Syndrome Treated with Therapeutic Plasma Exchange and Other Treatment Options – A Centre’s Experience

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European Neurological Review, a peer-reviewed, open access, bi-annual neurology journal published a cutting-edge article by Ilknur Kozanoglu, Yerdelen Deniz, Nurhilal Buyukkurt, Mahmut Yeral, Can Boga and Hakan Ozdogu.

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Guillain-Barré syndrome (GBS) is a rare but acute neuropathy occurring in 1.1–1.8/100,000 of the population (in Europe and North America).

Abstract: Therapeutic plasma exchange (TPE) has been shown to hasten recovery in Guillain-Barré syndrome (GBS). In this study, the objective was to show the outcome of disability grade in a retrospective analysis of data of clinical experience of TPE using the COBE Spectra Apheresis system and other treatment options in selected patients from a series of 56 patients with GBS at a single treatment centre in Turkey. Ten patients had the acute motor axonal neuropathy (AMAN) subtype; 46 had the acute inflammatory demyelinating polyneuropathy (AIDP) subtype of GBS. Three hundred and eighteen TPE procedures were performed taking 2 to 3 hours: in 6.3 % of them a peripheral catheter was used; in 93.7 % of them a central catheter was used. Replacement fluids were fresh frozen plasma (FFP), lactated Ringer’s solution or 3 % hydroxyethyl starch (HES). Among the patients, 12 (21.4 %) who had severe disease course received additional treatment to TPE – this was intravenous immunoglobulin (IVIG) in 11 patients. One patient was treated with steroids after rheumatology consultation due to another autoimmune disease. After 2 weeks, the mean GBS disability scores had significantly decreased from 3.75±0.48 to 2.44±0.96 (p=0.0001) and mean Medical Research Council (MRC) muscle strength scores significantly increased from 2.07±0.89 to 3.54±0.88(p=0.0001). No difference in efficacy was observed between AMAN and AIDP subtypes. Adverse events occurred in 20 procedures (6.3 %) of TPE and were mostly transient hypocalcaemia and allergic reactions that did not necessitate treatment discontinuation. Difficulty in venous access was observed in 3.14 % of procedures. TPE using the COBE Spectra Apheresis system provides effective treatment of GBS with an acceptable safety profile using various replacement fluids and is an essential part of disease management. Although the benefit is controversial, other treatment options may be applied as an additional therapy in selected patients.

Guillain-Barré syndrome (GBS) is a rare but acute neuropathy occurring in 1.1–1.8/100,000 of the population (in Europe and North America). GBS manifests as limb weakness, areflexia and sensory loss proceeding to neuromuscular paralysis involving facial, bulbar and respiratory function. Symptoms reach a maximum severity in 2–4 weeks. The neuropathy frequently causes severe and lasting disability, especially difficulty walking and can necessitate ventilator support: 3–13 % of patients die and 20 % are still unable to walk after 6 months. GBS is more frequent with increasing age (0.62/100/000 in 0–9 year olds rising to 2.66/100,000 in 80–89 year olds)5 and there is a small predominance of male gender. GBS has two subtypes: 1. acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (sensory motor symptoms resulting from demyelinating changes) and 2. acute motor axonal neuropathy (AMAN) (motor symptoms from axonal damage). The aetiology of GBS is not fully understood but it is believed to be a result of autoimmunity – in most cases triggered by infection with pathogens stimulating anti-ganglioside antibodies such as Campylobacter jejuni (diarrhoea), Mycoplasma pneumonia, Haemophilus influenzae, cytomegalovirus, Epstein Barr virus and influenza.

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Barney Kent