Guillain-Barre Syndrome Publications (7413)


Guillain-Barre Syndrome Publications

J Coll Physicians Surg Pak
J Coll Physicians Surg Pak 2014 Oct;24(10):778
Department of General OPD Ward, Combined Military Hospital, Peshawar.
Australas Med J
Australas Med J 2014 30;7(9):376-8. Epub 2014 Sep 30.
Department of Medicine, Medical College, Baroda, Gujarat, India 376.

We report the case of a 20-year-old male who presented to us with acute bilateral multiple cranial neuropathy in the form of bilateral total ophthalmoplegia and bulbar dysfunction. The patient had normal haematological and biochemical investigations, however, cerebrospinal fluid (CSF) analysis showed raised protein (96mg/dl) in the second week of illness. Peripheral nerve conduction studies and an MRI of the brain were normal. Read More

The patient showed gradual improvement after three weeks of supportive treatment. Considering the course of illness and the clinical and investigational profile, a diagnosis of an oculopharyngeal variant of Guillain-Barré syndrome (GBS) was made.

Bilateral facial nerve palsy is a relatively rare presentation and often points to a serious underlying medical condition. Several studies have reported presentation of bilateral facial nerve palsy in association with Lyme disease, Guillain-Barre syndrome, systemic lupus erythematosus, human immunodeficiency virus, sarcoidosis, diabetes and Hanson disease. While unilateral facial nerve palsy is sometimes associated with hemiplegia in sickle cell patients, no case of bilateral facial nerve palsy have been reported in the literature. Read More

A 29-year-old black African woman who is a known homozygous haemoglobin S (HbSS) presented with bilateral facial nerve palsy. She had the said condition 2 months post delivery of her first child and reported for physiotherapy 3 months post incidence. The pre-treatment House Brackmann Facial Grading Scale (HBFGS) Scores were 3 for right side and 4 for left side. This patient was not on any medication for the facial palsy. After 4 sessions of combination therapy consisting of faradism, facial exercises and massage there was remarkable improvement in the neurological status of the facial muscles. The post treatment House Brackmann Facial Grading Scale score was 2 bilaterally.
Bilateral facial nerve palsy may be an initial presentation of sickle cell anemia patients in the absence of other overt clinical presentations. Therefore sickle cell anemia should be considered among others, in the differential diagnosis of bilateral facial nerve palsy. Furthermore, this case report has highlighted the important role of physiotherapy in the management of bilateral facial nerve palsy.

Sci Rep
Sci Rep 2014 14;4:6058. Epub 2014 Aug 14.
YunnanCenter for Disease Control and Prevention, Kunming, Yunnan Province, People's Republic of China.
Case Rep Med
Case Rep Med 2014 15;2014:790458. Epub 2014 Sep 15.
Gauhati Medical College, Guwahati 781032, India.
Respir Care
Respir Care 2014 Oct 14. Epub 2014 Oct 14.
Department of Intensive Care Medicine, Cliniques Universitaires Saint-Luc, and

We report a case of Guillain-Barré syndrome complicated by respiratory failure requiring mechanical ventilation. Neurally adjusted ventilatory assist (NAVA) allowed proper patient-ventilator synchronization by pressure support proportional to the electrical activity of the diaphragm (Edi). Prolonged ventilation with NAVA seems feasible in patients with neuromuscular impairment, but the weaning process conducted by a continuous monitoring of Edi for pressure support titration needed to be assessed in a Guillain-Barré syndrome patient. Read More

Beginning on day 12 after hospital admission, the patient was ventilated with NAVA for 8 d. The NAVA level (pressure support per unit of Edi) was decreased from 1.2 cm H2O/μV to zero over the 8-d period. A simultaneous decrease in the tidal volume/Edi ratio was interpreted as a sign of recovery. A spontaneous breathing trial was successfully performed on day 20, followed by decannulation 4 d later. In conclusion, NAVA should be further investigated in patients with Guillain-Barré syndrome, particularly during the weaning period.

Ugeskr. Laeg.
Ugeskr Laeger 2014 Oct;176(42)
Risengård 9, 8700 Horsens.

Guillain-Barré syndrome is the leading cause of acute flaccid paralysis in the industrialized world. Approximately 25% of the patients suffering from Guillain-Barré syndrome develop respiratory failure requiring mechanical ventilation and intensive therapy. We seek answers to when it is optimal to start respiratory supportive therapy and review various complications associated with Guillain-Barré syndrome. Read More

BackgroundGuillain-Barré syndrome (GBS) is an autoimmune condition characterized by peripheral neuropathy. The pathogenesis of GBS is not fully understood, and the mechanism of how intravenous immunoglobulin (IVIG) cures GBS is ambiguous. Herein, we investigated lymphocyte subsets in patients with two major subtypes of GBS (acute inflammatory demyelinating polyneuropathy, AIDP, and acute motor axonal neuropathy, AMAN) before and after treatment with IVIG, and explored the possible mechanism of IVIG action. Read More

MethodsSixty-four patients with GBS were selected for our study and divided into two groups: AIDP (n¿=¿38) and AMAN (n¿=¿26). Thirty healthy individuals were chosen as the control group. Relative counts of peripheral blood T and B lymphocyte subsets were detected by flow cytometry analysis.ResultsIn the AIDP group, the percentage of CD4+CD45RO+ T cells was significantly higher, while the percentage of CD4+CD45RA+ T cells was notably lower, than in the control group. After treatment with IVIG, the ratio of CD4+/CD8+ T cells and the percentage of CD4+CD45RA+ T cells increased, while the percentages of CD8+ T cells and CD4+CD45RO+ T cells decreased significantly, along with the number of CD19+ B cells. However, there were not such obvious changes in the AMAN group. The Hughes scores were significantly lower in both the AIDP and AMAN groups following treatment with IVIG, but the changes in Hughes scores showed no significant difference between the two groups.ConclusionsThis study suggested that the changes in T and B-lymphocyte subsets, especially in CD4+T-lymphocyte subsets, might play an important role in the pathogenesis of AIDP, and in the mechanism of IVIG action against AIDP.

Aliment. Pharmacol. Ther.
Aliment Pharmacol Ther 2014 Oct 10. Epub 2014 Oct 10.
Cornwall Gastrointestinal Unit, Royal Cornwall Hospital Truro, Cornwall, UK.

Autochthonous (locally acquired) hepatitis E is increasingly recognised in developed countries, and is thought to be a porcine zoonosis. A range of extra-hepatic manifestations of hepatitis E infection have been described, but have never been systematically studied.
To report the extra-hepatic manifestations of hepatitis E virus. Read More

Retrospective review of data of 106 cases of autochthonous hepatitis E (acute n = 105, chronic n = 1).
Eight (7.5%) cases presented with neurological syndromes, which included brachial neuritis, Guillain-Barré syndrome, peripheral neuropathy, neuromyopathy and vestibular neuritis. Patients with neurological syndromes were younger (median age 40 years, range 34-92 years, P = 0.048) and had a more modest transaminitis (median ALT 471 IU/L, P = 0.015) compared to cases without neurological symptoms [median age 64 years (range 18-88 years), median ALT 1135 IU/L]. One patient presented with a cardiac arrhythmia,twelve patients (11.3%) presented with thrombocytopenia, fourteen (13.2%) with lymphocytosis and eight (7.5%) with a lymphopenia, none of which had any clinical consequence. Serum electrophoresis was performed in 65 patients at presentation, of whom 17 (26%) had a monoclonal gammopathy of uncertain significance. Two cases developed haematological malignancies, acute myeloid leukaemia and duodenal plasmacytoma, 18 and 36 months after presenting with acute hepatitis E infection.
A range of extra-hepatic manifestations can occur with hepatitis E. Neurological and haematological features of hepatitis E infection are relatively frequent in this UK cohort, and result in significant morbidity which warrants further study.

Comput Math Methods Med
Comput Math Methods Med 2014 15;2014:432109. Epub 2014 Sep 15.
Hospital General de Especialidades "Dr. Javier Buenfil Osorio", Avenida Lázaro Cárdenas 208, Colonia Las Flores, 24097 San Francisco de Campeche, CAM, Mexico.
Continuum (Minneap Minn)
Continuum (Minneap Minn) 2014 Oct;20(5 Peripheral Nervous System Disorders):1261-73

This article discusses the clinical features, pathophysiology, and management of primary and secondary acquired immune axonal neuropathies.
Although there are many collagen vascular disorders associated with vasculitic neuropathy, a quarter of cases have been described to be due to nonsystemic vasculitis of the peripheral nervous system. Enhanced surveillance and aggressive treatment of conditions such as cryoglobulin-related vasculitic neuropathy with cyclophosphamide, rituximab, and alfa interferons has led to improved morbidity and mortality, however, many cases of immune axonal acquired neuropathy are still associated with poor outcomes. Read More

Acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN) are well-characterized variants of Guillain-Barré syndrome.
Characterizing the clinical and electrophysiologic phenotype can help diagnose conditions such as nonsystemic vasculitic neuropathy, AMAN, AMSAN, and immune small fiber neuropathy, while careful evaluation of systemic features is key to identifying secondary immune axonal neuropathies such as vasculitic neuropathy related to collagen vascular disease. Additional research is needed to determine the exact immune pathogenesis and optimized treatment regimens for all acquired immune axonal neuropathies.

Continuum (Minneap Minn)
Continuum (Minneap Minn) 2014 Oct;20(5 Peripheral Nervous System Disorders):1241-60

Acquired immune demyelinating neuropathies refer to a group of disorders that share overlapping sensory, motor, and autonomic clinical, laboratory, and electrodiagnostic features. It is important to recognize acquired immune demyelinating neuropathies as they are generally responsive to immunosuppressive or immunomodulatory therapies.
This article reviews recently developed early prognostic tools in Guillain-Barré syndrome and discusses the evolving understanding of chronic demyelinating phenotypes with differing treatment responsiveness. Read More

While weakness and numbness progress over 2 to 4 weeks in Guillain-Barré syndrome, they continue to evolve beyond 8 weeks in chronic inflammatory demyelinating polyradiculoneuropathy and over 4 to 8 weeks in subacute inflammatory demyelinating polyradiculoneuropathy. Acquired immune demyelinating neuropathies present uncommonly as variants with predominance of ocular, bulbar, sensory, autonomic, or motor manifestations in addition to regional variants, such as paraparetic acquired immune demyelinating neuropathies. Establishing the correct diagnosis is important as these immune disorders differ in response to corticosteroids and other immunosuppressive therapies.

Ugeskr. Laeg.
Ugeskr Laeger 2014 Sep;176(36)
Neurologisk Afdeling, Glostrup Hospital, Ndr. Ringvej 57, 2600 Glostrup. E-mail:

This case report describes a 63-year-old male presenting with five days progressing bilateral peripheral facial paralysis and mild sensory symptoms. Cerebrospinal fluid showed albumin-cytological dissociation. Nerve conduction studies showed slightly reduced conduction velocity, slightly prolonged F-wave latency and temporal dispersion. Read More

Following treatment with IV immunoglobulin (0.4 g/kg/day for five consecutive days) recovery was prominent, though incomplete. This is believed to be the first described case in Denmark of the very rare variant of Guillain-Barré syndrome termed "facial diplegia and paraesthesias".

Exp Ther Med
Exp Ther Med 2014 Nov 11;8(5):1397-1401. Epub 2014 Sep 11.
Department of Neurology, First Affiliated Hospital of Dalian Medical University, Dalian, Liaoning 116011, P.R. China.

Sensory Guillain-Barré syndrome (GBS) is an acute demyelinating neuropathy that presents clinically with involvement of the sensory peripheral nerve only. To date, <10 cases of pure sensory GBS have been reported; thus, the clinical and pathological features of sensory variant GBS are yet to be well characterized. The current study reports the case of a 43-year-old female that presented with acute, symmetric and monophasic sensory neuropathy, without motor weakness. Read More

Patient history, clinical examination, routine nerve conduction studies and sural nerve biopsy were reviewed. All the observations were consistent with a diagnosis of pure sensory GBS. In particular, the pathological features of the sural nerve biopsy revealed that the form of regenerated nerve fibers have complete structure of myelinated nerve fascicles, and these myelinated nerve fibers are thicker than other parts of the biopsy. The patient received small-dose (20 mg/day) prednisone initially, but without any benefit. Satisfactory improvements were observed with one course of intravenous immunoglobulin.

Int. J. Infect. Dis.
Int J Infect Dis 2014 Oct 2. Epub 2014 Oct 2.
Department of Epidemiology and Preventive Medicine, Monash University, The Alfred Centre, 99 Commercial Road, Melbourne 3004, Victoria, Australia.

To estimate and compare disease burden attributable to six gastrointestinal pathogens (norovirus, rotavirus, Campylobacter, non-typhoidal Salmonella, Giardia, and Cryptosporidium) in Australia, 2010.
We estimated the number of acute gastroenteritis (AGE) cases and deaths, disability-adjusted life years (DALYs), and DALY/case for each pathogen. We included AGE cases that did not require medical care. Read More

Sequelae were included for Campylobacter (Guillain-Barré syndrome, reactive arthritis (ReA), irritable bowel syndrome (IBS)) and Salmonella (ReA, IBS).
We estimated 16626069 AGE cases in Australia in 2010 (population 22 million). Of the pathogens studied, most AGE cases were attributed to norovirus (2180145), Campylobacter (774003), and Giardia (614740). Salmonella caused the fewest AGE cases (71255) but the most AGE deaths (90). The DALY burden was greatest for Campylobacter (18222 DALYs) and Salmonella (3856 DALYs), followed by the viral and protozoal pathogens. The average DALY/case was greatest for Salmonella (54.1 DALY/1000 cases), followed by Campylobacter (23.5 DALY/1000 cases).
The pathogen causing the greatest disease burden varied according to the metric used, however DALYs are considered most useful given the incorporation of morbidity, mortality, and sequelae. These results can be used to prioritize public health interventions toward Salmonella and Campylobacter infections and to measure the impact of these interventions.

Neurosciences (Riyadh)
Neurosciences (Riyadh) 2014 Oct;19(4):301-5
HLA Typing Research Unit, Al-Kindy College of Medicine, Baghdad University, Baghdad, Iraq.
Eur Rev Med Pharmacol Sci
Eur Rev Med Pharmacol Sci 2014 Sep;18(17):2496-9
Unit of Pediatrics and Pediatric Emergency, University Hospital, "Vittorio Emanuele Polyclinic", Catania, Italy.

Three young patients with Bickerstaff's brainstem encephalitis (BBE) are reported. Some weeks following an upper tract infection, the children after a short period of recovery, showed acute onset of symmetric weakness of the lower limbs with difficulty in standing by and walking. The distal muscle weakness had a rapid progression with involvement of the cranial nerve, and then with severe impairment of the consciousness till to coma in one of the three children. Read More

BBE is a rare and often underdiagnosed affection in childhood. Common neuro-immune pathogenesis, overlap of clinical signs and strict correlation among BBE with Fisher syndrome and Guillain-Barrè syndrome lead to think that these affections represent an unique spectrum with different central and peripheral involvement. In these children, treatment with intravenous immunoglobulins resulted in a progressive and rapid resolution of the clinical features.

Am. J. Trop. Med. Hyg.
Am J Trop Med Hyg 2014 Sep 29. Epub 2014 Sep 29.
Division of Infectious Diseases, Department of Medicine, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
J. Neuroimmunol.
J Neuroimmunol 2014 Sep 18. Epub 2014 Sep 18.
Neuroinflammation Group, Brain & Mind Research Institute, University of Sydney, Sydney, Australia. Electronic address:

Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy are autoimmune disorders of the peripheral nervous system in which autoantibodies are implicated in the disease pathogenesis. Recent work has focused on the nodal regions of the myelinated axon as potential autoantibody targets. Here we screened patient sera for autoantibodies to neurofascin and assessed the pathophysiological relevance of anti-neurofascin antibodies in vivo. Read More

Levels of anti-neurofascin antibodies were higher in sera from patients with Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy when compared with those of controls. Anti-neurofascin antibodies exacerbated and prolonged adoptive transfer experimental autoimmune neuritis and caused conduction defects when injected intraneurally.

PLoS One 2014 26;9(9):e107772. Epub 2014 Sep 26.
Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore; Department of Physiology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore.
Vaccine 2014 Sep 22. Epub 2014 Sep 22.
Immunization Safety Office, Division of Healthcare Quality Promotion, National Center for Emerging and Zoonotic Infectious Diseases (NCEZID), Centers for Disease Control and Prevention (CDC), 1600 Clifton Rd NE, Atlanta, GA 30333, USA.

Trivalent live attenuated influenza vaccine (LAIV3) was licensed and recommended for use in 2003 in children and adults 2-49 years of age. Post-licensure safety data have been limited, particularly in adults.
We searched Vaccine Adverse Event Reporting System (VAERS) for US reports after LAIV3 from July 1, 2005-June 30, 2013 (eight influenza seasons) in adults aged ≥ 18 years old. Read More

We conducted descriptive analyses and clinically reviewed serious reports (i.e., death, life-threatening illness, hospitalization, prolonged hospitalization, or permanent disability) and reports of selected conditions of interest. We used empirical Bayesian data mining to identify adverse events (AEs) that were reported more frequently than expected. We calculated crude AE reporting rates to VAERS by influenza season.
During the study period, VAERS received 1207 LAIV3 reports in adults aged 18-49 years old; 107 (8.9%) were serious, including four death reports. The most commonly reported events were expired drug administered (n=207, 17%), headache (n=192, 16%), and fever (n=133, 11%). The most common diagnostic categories for non-fatal serious reports were neurological (n=40, 39%), cardiovascular (n=14, 14%), and other non-infectious conditions (n=20, 19%). We noted a higher proportion of Guillain-Barré syndrome (GBS) and cardiovascular reports in the Department of Defense (DoD) population compared to the civilian population. Data mining detected disproportional reporting of ataxia (n=15); clinical review revealed that ataxia was a component of diverse clinical entities including GBS.
Review of VAERS reports are reassuring, the only unexpected safety concern for LAIV3 identified was a higher than expected number of GBS reports in the DoD population, which is being investigated. Reports of administration of expired LAIV3 represent administration errors and indicate the need for education, training and screening regarding the approved indications.

J. Child Neurol.
J Child Neurol 2014 Sep 24. Epub 2014 Sep 24.
The Children's Hospital of Zhejiang University, School of Medicine Hangzhou, Hangzhou, China.
J Pediatr Neurosci
J Pediatr Neurosci 2014 May;9(2):148-9
Department of Pediatrics, Maulana Azad Medical College and Associated Chacha Nehru Bal Chikitsalaya, New Delhi, India.

Guillain-Barre' syndrome is a rare complication of typhoid fever, and only a few such cases have been reported in the pediatric age group. We report a young boy with blood culture proven typhoid fever that developed this very rare neurological complication quite early in the course of the disease. Following treatment with intravenous antibiotics and intravenous immunoglobulin, he improved. Read More

Dtsch Arztebl Int
Dtsch Arztebl Int 2014 Sep;111(35-36):577-83
Department of Gastroenterology, Hepatology and Endocrinology, Hannover Medical School, Hanover, German Centre for Infection Research, Hanover, TWINCORE Institute for Experimental Infection Research, Hanover, Health Care Center at the University Medical Center Hamburg-Eppendorf, Robert Koch Institute Berlin, Consiliary Laboratory for Hepatitis A and Hepatitis E, Institute for Medical Microbiology and Hygiene, University Hospital Regensburg, Regensburg.

At least 17% of the population in Germany has been infected with the hepatitis E virus (HEV); thus, HEV infections are more frequent than was previously assumed. However, fewer than 500 HEV infections were reported to the Robert Koch Institute in 2013.
Review of pertinent literature retrieved by a selective search in PubMed. Read More

Persons living in Germany generally acquire hepatitis E infection within the country by consuming infected and undercooked pork; in rare cases, hepatitis E infections are imported from the tropics. HEV can be transmitted via blood products, blood transfusions, and organ transplantation. More than 99% of HEV infections are asymptomatic and self-limiting, but there are also severe cases with acute liver failure. Immunosuppressed persons can develop chronic HEV infection, potentially leading, within a few years, to liver cirrhosis with life-threatening sequelae. Moreover, HEV infection may be associated with extrahepatic manifestations such as Guillain-Barré syndrome. In two retrospectively evaluated case series, ribavirin was found to be active against HEV and can be used to treat either acute or chronic HEV infection.
Hepatitis E must be considered in the differential diagnosis of elevated hepatic enzyme levels and of systemic and neurological conditions of uncertain origin. The infection is usually self-limiting but can take a severe course in immunosuppressed persons. In such cases, ribavirin can be used as an antiviral treatment.

Pract Neurol
Pract Neurol 2014 Sep 19. Epub 2014 Sep 19.
Departments of Medicine and Physiology, Yon Loo Lin School of Medicine, National University of Singapore, Singapore.

Guillain-Barré syndrome (GBS) and its variant, Miller Fisher syndrome (MFS) have several subtypes, together forming a continuous spectrum of discrete and overlapping syndromes. Such is the heterogeneity within this spectrum that many physicians may be surprised to learn that these disorders are related pathophysiologically, and therefore share certain clinical features. These include history of antecedent infection, monophasic disease course and symmetrical cranial or limb weakness. Read More

The presence of cerebrospinal fluid albuminocytological dissociation (raised protein, normal cell count), antiganglioside antibodies and neurophysiological evidence of axonal or demyelinating neuropathy also support a diagnosis in many cases, but should not be relied upon. Mimics of GBS and MFS can broadly be divided into those presenting with symmetrical limb weakness and those presenting with brainstem signs. MFS and the pharyngeal-cervical-brachial variant of GBS are frequently mistaken for brainstem stroke, botulism or myasthenia gravis, whereas Bickerstaff's brainstem encephalitis is often diagnosed as Wernicke's encephalopathy. Chameleons or atypical presentations of GBS-related disorders include: paraparetic GBS, bifacial weakness with paraesthesias, acute ataxic neuropathy, acute ophthalmoparesis, acute ptosis and acute mydriasis. Many neurologists may also not be aware that deep tendon reflexes remain present or may even appear brisk in up to 10% of patients with GBS. Correct diagnosis of GBS-related disorders helps to avoid unnecessary investigations and allows early immunotherapy if appropriate.

Cochrane Database Syst Rev
Cochrane Database Syst Rev 2014 19;9:CD002063. Epub 2014 Sep 19.
MRC Centre for Neuromuscular Diseases, National Hospital for Neurology and Neurosurgery, PO Box 114, Queen Square, London, UK, WC1N 3BG.

Guillain-Barré syndrome (GBS) is an acute, paralysing, inflammatory peripheral nerve disease. Intravenous immunoglobulin (IVIg) is beneficial in other autoimmune diseases. This is an update of a review first published in 2001 and previously updated in 2003, 2005, 2007, 2010 and 2012. Read More

Other Cochrane systematic reviews have shown that plasma exchange (PE) significantly hastens recovery in GBS compared with supportive treatment alone, and that corticosteroids alone are ineffective.
We had the following four objectives.1. To examine the efficacy of intravenous immunoglobulin (IVIg) in hastening recovery and reducing the long-term morbidity from Guillain-Barré syndrome (GBS).2. To determine the most efficacious dose of IVIg in hastening recovery and reducing the long-term morbidity from GBS.3. To compare the efficacy of IVIg and plasma exchange (PE) or immunoabsorption in hastening recovery and reducing the long-term morbidity from GBS.4. To compare the efficacy of IVIg added to PE with PE alone in hastening recovery and reducing the long-term morbidity from GBS.
We searched the Cochrane Neuromuscular Disease Group Specialized Register (2 December 2013), CENTRAL (2013, Issue 12 in The Cochrane Library), MEDLINE (January 1966 to November 2013) and EMBASE (January 1980 to November 2013). We checked the bibliographies in reports of the randomised trials and contacted the authors and other experts in the field to identify additional published or unpublished data.
Randomised and quasi-randomised trials of IVIg compared with no treatment, placebo treatment, PE, or other immunomodulatory treatments in children and adults with GBS of all degrees of severity. We also included trials in which IVIg was added to another treatment.
Two authors independently selected papers, extracted data and assessed quality. We collected data about adverse events from the included trials.
Twelve trials were found to be eligible for inclusion in this review. Seven trials with a variable risk of bias compared IVIg with PE in 623 severely affected participants. In five trials with 536 participants for whom the outcome was available, the mean difference (MD) of change in a seven-grade disability scale after four weeks was not significantly different between the two treatments: MD of 0.02 of a grade more improvement in the intravenous immunoglobulin than the plasma exchange group; 95% confidence interval (CI) 0.25 to -0.20. There were also no statistically significant differences in the other measures considered. Three studies including a total of 75 children suggested that IVIg significantly hastens recovery compared with supportive care. The primary outcome for this review, available for only one trial with 21 mildly affected children, showed significantly more improvement in disability grade after four weeks with IVIg than supportive treatment alone, MD 1.42, 95% CI 2.57 to 0.27.In one trial involving 249 participants comparing PE followed by IVIg with PE alone, the mean grade improvement was 0.2 (95% CI -0.14 to 0.54) more in the combined treatment group than in the PE alone group; not clinically significantly different, but not excluding the possibility of significant extra benefit. Another trial with 34 participants comparing immunoabsorption followed by IVIg with immunoabsorption alone did not reveal significant extra benefit from the combined treatment.Adverse events were not significantly more frequent with either treatment, but IVIg is significantly much more likely to be completed than PE.Small trials in children showed a trend towards more improvement with high-dose compared with low-dose IVIg, and no significant difference when the standard dose was given over two days rather than five days.
A previous Cochrane review has shown that PE hastens recovery compared with supportive treatment alone. There are no adequate comparisons of IVIg with placebo in adults, but this review provides moderate quality evidence that, in severe disease, IVIg started within two weeks from onset hastens recovery as much as PE. Adverse events were not significantly more frequent with either treatment but IVIg is significantly much more likely to be completed than PE. Also, according to moderate quality evidence, giving IVIg after PE did not confer significant extra benefit. In children, according to low quality evidence, IVIg probably hastens recovery compared with supportive care alone. More research is needed in mild disease and in patients whose treatment starts more than two weeks after onset. Dose-ranging studies are also needed and one is in progress.