Guillain-Barre Syndrome Publications (7527)


Guillain-Barre Syndrome Publications

Neurol. Sci.
Neurol Sci 2015 Mar 5. Epub 2015 Mar 5.
Department of Neurology, Tokyo Metropolitan Neurological Hospital, 2-6-1 Musashidai, Fuchu, Tokyo, 183-0042, Japan,

Guillain-Barré syndrome (GBS) is an acute immune-mediated polyneuropathy. Although its pathogenic mechanism has been revealed and various therapeutic trials have been performed, a proportion of patients experience the severe sequelae associated with GBS. In this paper, we investigated whether the amount of the neuron-specific protein, ubiquitin carboxy-terminal hydrolase-L1 (UCH-L1), in the cerebrospinal fluid of patients with GBS was correlated with the clinical course of the disease. Read More

UCH-L1 protein levels were greater in patients with GBS than in controls. The patients with GBS whose UCH-L1 protein levels were higher than those of the controls presented with more severe symptoms at peak. UCH-L1 protein levels tended to become elevated as the total protein levels were increased; however, elevated UCH-L1 without an increase in total protein might be correlated with severe disease course (bedridden or ventilator supported). These results suggest that UCH-L1 could be a biomarker associated with the severity of the disease at the acute phase of GBS.

J Clin Diagn Res
J Clin Diagn Res 2015 Jan 1;9(1):SC09-12. Epub 2015 Jan 1.
Senior Resident, Department of Paediatrics, KMC Manipal , India .

Guillain-Barre Syndrome (GBS) is an acute monophasic demyelinating neuropathy characterized by progressive motor weakness of limbs with areflexia.
To study the clinical pattern and outcome of children with Guillain-Barre syndrome.
It was a cross-sectional study conducted in a pediatric unit of tertiary care hospital over a period of 18 months. Read More

We assessed the clinical manifestations, results of electro-diagnostic tests, functional status, treatment instituted and outcome of 20 children diagnosed with GBS.
Of the 20 (male to female ratio = 2.3:1) children studied, all had motor weakness, 5 (25%) had sensory loss, 4 (20 %) had cranial nerve palsies and 4 (20%) had autonomic disturbances. Respiratory paralysis was found in 7 (35%) children requiring assisted ventilation. Antecedent illness preceding GBS was recorded in 50% children. The GBS subtype distribution as per electrodiagnostic studies was as follows: acute motor axonal neuropathy (AMAN) in 7 (38.9%), acute motor sensory axonal neuropathy (AMSAN) in 4 (22.2%), acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 4 (22.2%) and both axonal and demyelinating neuropathy in 3 (16.7%). Intravenous immunoglobulins (IVIG) constituted the treatment given in majority of the patients. Plasmapharesis was performed in one child in view of poor response to IVIG. Complete recovery was observed in 14 children and the remaining 3 children experienced only incomplete recovery.
Male preponderance and presence of antecedent illness in a majority of subjects was observed in our study. Regardless of the severity of illness at admission and electrophysiological subtypes, a majority achieved full recovery. Intravenous Immunoglobulin and supportive care form the cornerstone of management in childhood GBS.

Vaccine 2015 Feb 26. Epub 2015 Feb 26.
Department of Pediatrics, Severance Children's Hospital, Epilepsy Research Center, Yonsei University College of Medicine, Seoul, Republic of Korea. Electronic address:
Asian J Transfus Sci
Asian J Transfus Sci 2015 Jan-Jun;9(1):87-8
Department of Immunohaematology and Blood Transfusion, B J Medical College and Civil Hospital, Ahmedabad, Gujarat, India.

Guillain - Barre syndrome (GBS) is an acute, frequently severe progressive illness of peripheral nervous system that is autoimmune in nature. GBS after myocardial infarction (MI) with ventricular septal defect (VSD) is uncommon with high mortality rate if not treated promptly.[1] We report a successful outcome of GBS post MI with VSD in a 60-year-old male patient who was on a ventilator treated successfully with therapeutic plasma exchange. Read More

J Oncol Pharm Pract
J Oncol Pharm Pract 2015 Feb 23. Epub 2015 Feb 23.
Department of Internal Medicine, Division of Hematology and Oncology, University of Arkansas for Medical Sciences (UAMS), Little Rock, AR, USA

Ipilimumab is a novel humanized monoclonal antibody directed against cytotoxic T lymphocyte antigen 4, a T-cell surface molecule involved in down-regulation and suppression of the T cell response to stimuli. Patients treated with ipilimumab are at risk for immune-related adverse events involving the skin, digestive tract, liver and endocrine organs. Few case reports of immune-related adverse effects involving central or peripheral nervous system due to ipilimumab are published. Read More

These include inflammatory myopathy, aseptic meningitis, severe meningo-radiculo-neuritis, temporal arteritis, Guillain-Barre syndrome, and posterior reversible encephalopathy syndrome. We report the first case of ipilimumab-induced progressive necrotic myelopathy.

J. Neurol.
J Neurol 2015 Feb 26. Epub 2015 Feb 26.
Department of Neurology, Gloucester Royal Hospital, Gloucester, GL1 3NN, UK,

Guillain-Barré syndrome (GBS) constitutes a spectrum of related post-infectious neuropathies, which are characterized by their anatomical patterns of weakness and neurological involvement. Historically, the term polyneuritis cranialis has been used to describe some patients with GBS presenting with multiple cranial neuropathies in the absence of limb weakness. We examine previous reports of polyneuritis cranialis to determine disease characteristics and define new diagnostic criteria. Read More

Disease characteristics were determined from 15 historical case reports of patients presenting with isolated 'polyneuritis cranialis', 'cranial polyneuropathy', 'multiple cranial neuropathy' and 'multiple cranial neuropathies' due to GBS. Median age was 40 years. 80 % displayed antecedent infectious symptoms. In all cases, disease course was monophasic with clinical improvement within weeks or months. Initial symptoms were ocular (73 %) or bulbar (33 %). Mean number of cranial nerves involved was 6 (range, 3-10). 93 % displayed ocular signs, 73 % facial weakness or numbness and 87 % bulbar signs. In 3 patients (20 %), there was significant asymmetry. Cerebrospinal fluid albuminocytological dissociation was present in 67 % of cases. Serum anti-ganglioside antibodies were tested for in 8 of 15 patients and anti-GQ1b antibodies were found in 3 patients, whilst anti-GT1a antibodies were found in 1 patient. Polycranial neuritis (the oculopharyngeal subtype of GBS) can be defined in patients with disease characteristics of GBS who display ocular and pharyngeal weakness in the absence of limb weakness or ataxia. In half of cases tested for, anti-ganglioside antibodies were present and most frequently against GQ1b.

Trop Parasitol
Trop Parasitol 2015 Jan-Jun;5(1):61-3
Department of Medicine, College of Medicine and Sagore Dutta Hospital, Kamarhati, West Bengal, India.

Malaria poses a major public health problem in India, where it is endemic, especially severe malaria caused by Plasmodium falciparum infestation. There have been great changes in the clinical manifestation of severe falciparum malaria over the past couple of decades, with a shift from cerebral malaria to fever with jaundice, renal failure, bleeding diathesis, and multi-organ dysfunction syndrome. Here, we discuss two cases of severe falciparum malaria which presented with extremely uncommon neurological manifestations. Read More

J Crit Care
J Crit Care 2015 Feb 7. Epub 2015 Feb 7.
ANZIC Research Center, Department of Epidemiology and Preventive Medicine, Monash University, Melbourne, VIC 3004, Australia; Transfusion Research Unit, Department of Epidemiology and Preventive Medicine, Monash University, Melbourne, VIC 3004, Australia; Réanimation médicale, Hôpital Universitaire de Brest site de la Cavale Blanche, Université de Bretagne Occidentale, 29200 Brest, France. Electronic address:

Intravenous immunoglobulin (IVIg) use is growing dramatically internationally due to the increasing numbers of acute and chronic conditions that may benefit from IVIg. Patients with conditions that may benefit from IVIg might require intensive care unit (ICU) admission, supporting the need to review IVIg use in the critical care setting. The most common clinical indications for IVIg in adults that may require ICU admission and are commonly supported under clinical practice guidelines are Guillain-Barré syndrome, myasthenia gravis and Lambert-Eaton myasthenic syndrome, inflammatory myopathies, and primary or secondary immunodeficiency diseases complicated by severe bacterial sepsis. Read More

Other emerging indications include necrotizing fasciitis, toxic epidermal necrolysis/Stevens-Johnson syndrome, and toxic shock syndrome. The evidence for IVIg use in sepsis and septic shock remains controversial and insufficient to recommend its routine use. Intravenous immunoglobulin is expensive and also carries risks of adverse effects, including common and benign infusion-related reactions, as well as relatively rare and more serious problems, such as thromboembolic events, renal failure, and aseptic meningitis. In this article, we review the literature on conditions requiring ICU admission and IVIg, and we classify them as supported, emerging, or unsupported indications based on the available evidence and guidelines for clinical use of IVIg.

J. Neurol. Neurosurg. Psychiatr.
J Neurol Neurosurg Psychiatry 2015 Feb 19. Epub 2015 Feb 19.
Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.

Peripheral nerve diseases are traditionally classified as demyelinating or axonal. It has been recently proposed that microstructural changes restricted to the nodal/paranodal region may be the key to understanding the pathophysiology of antiganglioside antibody mediated neuropathies. We reviewed neuropathies with different aetiologies (dysimmune, inflammatory, ischaemic, nutritional, toxic) in which evidence from nerve conductions, excitability studies, pathology and animal models, indicate the involvement of the nodal region in the pathogenesis. Read More

For these neuropathies, the classification in demyelinating and axonal is inadequate or even misleading, we therefore propose a new category of nodopathy that has the following features: (1) it is characterised by a pathophysiological continuum from transitory nerve conduction block to axonal degeneration; (2) the conduction block may be due to paranodal myelin detachment, node lengthening, dysfunction or disruption of Na(+) channels, altered homeostasis of water and ions, or abnormal polarisation of the axolemma; (3) the conduction block may be promptly reversible without development of excessive temporal dispersion; (4) axonal degeneration, depending on the specific disorder and its severity, eventually follows the conduction block. The term nodopathy focuses to the site of primary nerve injury, avoids confusion with segmental demyelinating neuropathies and circumvents the apparent paradox that something axonal may be reversible and have a good prognosis.

Am J Case Rep
Am J Case Rep 2015 20;16:99-103. Epub 2015 Feb 20.
Department of Internal Medicine, Bridgeport Hospital, Yale University School of Medicine, Bridgeport, CT, USA.
J. Neurol. Neurosurg. Psychiatr.
J Neurol Neurosurg Psychiatry 2015 Feb 18. Epub 2015 Feb 18.
Department of Neurology, University Hospital Würzburg, Würzburg, Germany.

Autoantibodies against paranodal proteins have been described in patients with inflammatory neuropathies, but their association with pathology of nodes of Ranvier is unclear. We describe the clinical phenotype and histopathological changes of paranodal architecture of patients with autoantibodies against contactin-1, identified from a cohort with chronic inflammatory demyelinating polyradiculoneuropathy (n=53) and Guillain-Barré syndrome (n=21).
We used ELISA to detect autoantibodies against contactin-1. Read More

Specificity of the autoantibodies was confirmed by immunoblot assay, binding to contactin-1-transfected human embryonic kidney cells, binding to paranodes of murine teased fibres and preabsorption experiments. Paranodal pathology was investigated by immunofluorescence labelling of dermal myelinated fibres.
High reactivity to contactin-1 by ELISA was found in four patients with chronic inflammatory demyelinating polyradiculoneuropathy and in none of the patients with Guillain-Barré syndrome, which was confirmed by cell binding assays in all four patients. The four patients presented with a typical clinical picture, namely acute onset of disease and severe motor symptoms, with three patients manifesting action tremor. Immunofluorescence-labelling of paranodal proteins of dermal myelinated fibres revealed disruption of paranodal architecture. Semithin sections showed axonal damage but no classical signs of demyelination.
We conclude that anti-contactin-1-related neuropathy constitutes a presumably autoantibody-mediated form of inflammatory neuropathy with distinct clinical symptoms and disruption of paranodal architecture as a pathological correlate. Anti-contactin-1-associated neuropathy does not meet morphological criteria of demyelinating neuropathy and therefore, might rather be termed a 'paranodopathy' rather than a subtype of demyelinating inflammatory neuropathy.

Clin Neurophysiol
Clin Neurophysiol 2015 Feb 3. Epub 2015 Feb 3.
Department of Neurology, Basel University Hospital, Basel, Switzerland.

Ultrasound differentiation of neuropathies is a great challenge. We, therefore, suggest a standardized score to operationalize differentiation between several acute and subacute onset neuropathies.
We retrospectively analyzed the ultrasound data of 61 patients with acute or subacute neuropathies, e. Read More

g. chronic immune-mediated neuropathies, Guillain-Barré syndrome (GBS), and axonal/vasculitic neuropathies. We compared these data to 28 healthy controls. Based on these results an ultrasound pattern sum score (UPSS) with three sub-scores (UPS-A for the sensorimotor nerves, UPS-B for the cervical roots and the vagal nerve and UPS-C for the sural nerve) was developed. Afterwards, the applicability of the score was prospectively validated in 10 patients with chronic neuropathies and in 14 patients with unknown acute and subacute PNP before performing additional tests.
UPS-A and UPSS were significantly higher in CIDP than in other neuropathies and controls (p<0.001). UPS-B was significantly more often pathologic in GBS than in CIDP and other neuropathies (p<0.001). Using receiver operation characteristics curve analysis boundary values for each score were defined. Positive predictive value (PPV) of these scores for CIDP and GBS was >85%. Vasculitic neuropathies showed an intermediate type of UPSS compared to other axonal neuropathies (p<0.001). In the prospective application the pattern score could be used with good accuracy in several types of neuropathy.
UPS-A and UPSS operationalize to diagnose acute and subacute-onset CIDP and its variants with high sensitivity, specificity, and PPV. An increased UPS-B with normal UPSS and other sub scores may point to the diagnosis of GBS with high PPV and enables the differentiation from CIDP.
Using the UPSS and its sub-scores gives a new diagnostic power to the method of the peripheral nerve ultrasound.

FEMS Microbiol. Lett.
FEMS Microbiol Lett 2015 Feb 24;362(4):1-3. Epub 2014 Dec 24.
School of Life Sciences, Faculty of Science, Engineering and Computing, Kingston University, Kingston upon Thames, Penrhyn Road, KT1 2EE, UK

This report describes a draft genome sequence of Campylobacter jejuni strain G1. The strain contains no plasmids, despite being resistant to tetracycline (Tet). Comparison of the genome of this strain with that of a reference strain NCTC 11168 (Tet sensitive) revealed the presence of a candidate gene that may be responsible for antibiotic resistance, as well as the genes involved in the synthesis of ganglioside-mimicking molecules responsible for the development of a neurodegenerative disorder, Guillain-Barré syndrome. Read More

Trends Mol Med
Trends Mol Med 2015 Feb 10. Epub 2015 Feb 10.
Institut National de la Santé et de la Recherche Médicale, Unité 1138, Paris, F-75006, France; Centre de Recherche des Cordeliers, Equipe - Immunopathology and therapeutic immunointervention, Paris, F-75006, France; Sorbonne Universités, UPMC Univ Paris 06, UMR S 1138, Paris, F-75006, France; Université Paris Descartes, Sorbonne Paris Cité, UMR S 1138, Paris, F-75006, France; International Associated Laboratory IMPACT (Institut National de la Santé et de la Recherche Médicale, France - Indian Council of Medical Research, India), National Institute of Immunohematology, Mumbai, 400012, India. Electronic address:

Some patients with autoimmune and inflammatory diseases treated with intravenous immunoglobulin G (IVIg) as a first line therapy are refractory. Identification of predictive biomarker(s) to segregate responders and non-responders to IVIg therapy remains critical. A number of biomarkers, particularly in Kawasaki disease, have shown potential for predicting response to IVIg. Read More

Vaccine 2015 Feb 9. Epub 2015 Feb 9.
Immunization Safety Office, Division of Healthcare Quality Promotion, National Center for Emerging and Zoonotic Infectious Diseases, Centers for Disease Control and Prevention, United States.

Quadrivalent live attenuated influenza vaccine (LAIV4) was approved in 2012 for healthy persons aged 2-49 years. Beginning with the 2013-2014 influenza season, LAIV4 replaced trivalent live attenuated influenza vaccine (LAIV3).
We analyzed LAIV4 reports to VAERS, a national spontaneous reporting system. Read More

LAIV4 reports in 2013-2014 were compared to LAIV3 reports from the previous three influenza seasons. Medical records were reviewed for non-manufacturer serious reports (i.e., death, hospitalization, prolonged hospitalization, life-threatening illness, permanent disability) and reports of selected conditions of interest. We conducted Empirical Bayesian data mining to identify disproportional reporting for LAIV4.
In 2013-2014, 12.7 million doses of LAIV4 were distributed and VAERS received 779 reports in individuals aged 2-49 years; 95% were non-serious. Expired drug administered (42%), fever (13%) and cough (8%) were most commonly reported in children aged 2-17 years when LAIV4 was administered alone, while headache (18%), expired drug administered (15%) and exposure during pregnancy (12%) were most common in adults aged 18-49 years. We identified one death report in a child who died from complications of cerebellar vascular tumors. Among non-death serious reports, neurologic conditions were common in children and adults. In children, seizures (3) and Guillain-Barré syndrome (2) were the most common serious neurologic outcomes. We identified three serious reports of asthma/wheezing following LAIV4 in children. Data mining detected disproportional reporting for vaccine administration errors and for influenza illness in children.
Our analysis of VAERS reports for LAIV4 did not identify any concerning patterns. The data mining finding for reports of influenza illness is consistent with low LAIV4 vaccine effectiveness observed for influenza A disease in children in 2013-2014. Reports of LAIV4 administration to persons in whom the vaccine is not recommended (e.g., pregnant women) indicate the need for education, training and screening regarding indications.

Rinsho Shinkeigaku
Rinsho Shinkeigaku 2014 ;54(12):1056-7
Department of Neurology, Graduate School of Medicine, Chiba University.

This review described current status and perspectives of treatment for immune-mediated neuropathies, such as Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy, and demyelinating neuropathy with anti-MAG neuropathy. corticosteroids, immunoglobulin therapy, and plasmapheresis are conventional treatments for these neuropathies, but the responsiveness to the treatments significantly differ among the disorders. Promising new treatment options include anti-complement monoclonal antibody (Eculizumab, anti-C5) for Guillain-Barre syndrome, and rituximab (anti-CD20) for anti-MAG neuropathy. Read More

For CIDP, different treatments would be required according to the clinical subtypes; typical CIDP and asymmetric variabt). For multifocal neuropathy, maintainance treatment with immunoglobulin is necessary.

Korean J Pediatr
Korean J Pediatr 2014 Dec 31;57(12):542-5. Epub 2014 Dec 31.
Department of Pediatrics, Konyang University College of Medicine, Daejeon, Korea.
J. Neurol. Neurosurg. Psychiatr.
J Neurol Neurosurg Psychiatry 2015 Feb 4. Epub 2015 Feb 4.
Prince of Wales Clinical School, University of New South Wales, Australia Central Clinical School, The University of Sydney, Australia.

Peripheral nerve ultrasound (US) has emerged as a promising technique for the diagnosis of peripheral nerve disorders. While most experience with US has been reported in the context of nerve entrapment syndromes, the role of US in the diagnosis of peripheral neuropathy (PN) has recently been explored. Distinctive US findings have been reported in patients with hereditary, immune-mediated, infectious and axonal PN; US may add complementary information to neurophysiological studies in the diagnostic work-up of PN. Read More

This review describes the characteristic US findings in PN reported to date and a classification of abnormal nerve US patterns in PN is proposed. Closer scrutiny of nerve abnormalities beyond assessment of nerve calibre may allow for more accurate diagnostic classification of PN, as well as contribute to the understanding of the intersection of structure and function in PN.

Neuroradiology 2015 Feb 5. Epub 2015 Feb 5.
Section of Neuroradiology, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center, 4401 Penn Avenue, Floor 2, Pittsburgh, PA, 15224, USA,
Neurology 2015 Feb;84(5):e30
From the Departments of Neurology (C.G., J.A.P., U.S., A.A.T.) and Neuroradiology (A.P.), University Hospital Zurich, Switzerland.
Rev Neurol
Rev Neurol 2015 Feb;60(3):120-31
Hospital Clinico Universitario de Valencia, 46010 Valencia, Espana.

INTRODUCTION. Plasma exchange is a technique used in the treatment of some neurological autoimmune disorders since the 80s, especially in acute conditions. In recent years new data about it use has been published in many diseases with autoimmune basis, expanding the range of use of this technique. Read More

AIM. To update the current indications of this technique in the treatment of neurological diseases. DEVELOPMENT. We conducted a thorough review of all articles about the efficacy of plasma exchange in the treatment of different neurological diseases published since the 80s. We have also carried out a detailed analysis of recommendations and evidence of the use of this procedure by analyzing the guidelines of different scientific societies. CONCLUSIONS. Plasma exchange has proven to be an effective alternative treatment with high grade scientific evidence in diseases such as Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy and myasthenia gravis. It has been effective in treating acute demyelinating episodes unresponsive to other therapies, neuromyelitis optica relapses and other central nervous system diseases induced by antibodies. In comparative studies with intravenous immunoglobulin efficacy of both therapies is similar. Comparative studies should continue to be conducted in order to better understand the mechanisms of action, prioritize indications and compare the cost-effectiveness ratio of both procedures.

Medicine (Baltimore)
Medicine (Baltimore) 2015 Jan;94(3):e392
From the Department of Rehabilitation Medicine, Hanyang University College of Medicine, Seoul, Korea.

Acute motor axonal neuropathy (AMAN) is a variant of Guillain-Barre syndrome. It has been reported to have no sensory symptoms and is diagnosed by typical electrophysiological findings of low-amplitude or unobtainable compound muscle action potentials with normal sensory nerve action potentials. However, the authors experienced atypical case of general electrophysiological findings of AMAN with pain and paresthesia and presented it. Read More

This case implies that clinician should be on the alert to atypical sensory symptoms from the classical presentation of AMAN even if the patient is diagnosed with AMAN electrophysiologically and should consider proper treatment options based on clinical presentations.

Neurosci. Lett.
Neurosci Lett 2015 Jan 21. Epub 2015 Jan 21.
Departments of Neuroscience and Neurology, USA. Electronic address:

Peripheral neuropathy is a common complication of a variety of diseases and treatments, including diabetes, cancer chemotherapy, and infectious causes (HIV, hepatitis C, and Campylobacter jejuni). Despite the fundamental difference between these insults, peripheral neuropathy develops as a combination of just six primary mechanisms: altered metabolism, covalent modification, altered organelle function and reactive oxygen species formation, altered intracellular and inflammatory signaling, slowed axonal transport, and altered ion channel dynamics and expression. All of these pathways converge to lead to axon dysfunction and symptoms of neuropathy. Read More

The detailed mechanisms of axon degeneration itself have begun to be elucidated with studies of animal models with altered degeneration kinetics, including the slowed Wallerian degeneration (Wld(S)) and Sarm knockout animal models. These studies have shown axonal degeneration to occur through a programmed pathway of injury signaling and cytoskeletal degradation. Insights into the common disease insults that converge on the axonal degeneration pathway promise to facilitate the development of therapeutics that may be effective against other mechanisms of neurodegeneration.

Int Braz J Urol
Int Braz J Urol 2014 november-december;40(6):858-859
Faculty of Medical Sciences of Paraiba and Department of Urology, Police Military Hospital Edson Ramalho, João Pessoa, PB, Brazil.
Mediators Inflamm.
Mediators Inflamm 2014 22;2014:758639. Epub 2014 Sep 22.
Department of Microbiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow 226014, India.