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(searched for: doi:10.1007/s00431-006-0140-1)
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Jialing Li, , Yingyu Liu, Yang Cao, Jun He, Muxi Liao
Published: 2 June 2022
Frontiers in neurology, Volume 13;

Guillain–Barré syndrome(GBS) is an autoimmune-mediated peripheral neuropathy. Immune checkpoint inhibitors (ICIs) are the standard treatment for cancer and may lead to immune-related adverse events (irAEs) such as GBS. Corticosteroids, plasma exchange (PE), and intravenous immunoglobulin (IVIG) are currently accepted treatments for ICI-induced GBS. However, there are still adverse reactions, and the effect of relieving symptoms is not as good as expected. Safe and effective complementary replacement therapy to alleviate GBS symptoms and ameliorate the quality of life is urgently required. In this case, a 63-year-old man received ICI therapy and antitumor chemotherapy for lung malignancy. After two courses of treatment, the patient gradually developed limb weakness, numbness, and pain at the ends of the limbs, with cerebrospinal fluid (CSF) albuminocytological dissociation, and electromyography (EMG) suggested demyelinating changes and was diagnosed as GBS. Although the patient received high doses of intravenous gamma globulin and limb weakness symptoms were alleviated, there was still significant numbness and pain in the extremities. After four times of acupuncture treatments, the patient complained that the symptoms of limb numbness and fatigue were significantly alleviated without any discomfort. This case report may provide a new alternative and complementary therapy for immune checkpoint inhibitor-induced GBS, but more definitive and robust evidence is needed to support its efficacy.
Chee Keong Wong, Chen Fei Ng, Hui Jan Tan, Shahizon Azura Mohamed Mukari
Published: 24 May 2021
by BMJ
Journal: BMJ case reports
BMJ case reports, Volume 14;

Bickerstaff brainstem encephalitis (BBE) is a rare autoimmune encephalitis characterised by ataxia, ophthalmoplegia and altered consciousness. An overlap between BBE with Guillain-Barré syndrome (GBS) shows similar clinical and immunological features. We report a case of BBE with GBS overlap secondary to Chlamydia pneumoniae infection. The triad of altered consciousness, ataxia and ophthalmoplegia were present in the patient. The investigations included cerebrospinal fluid cytoalbuminological dissociation, nerve conduction test that showed prolonged or absent F wave latencies, hyperintensity in the left occipital region on brain MRI and diffuse slow activity on the electroencephalogram. The chlamydia serology was positive indicating a postinfectious cause of BBE syndrome. He required artificial ventilation as his consciousness level deteriorated with tetraparesis, oropharyngeal and respiratory muscle weakness. Immunotherapy with intravenous immunoglobulin and methylprednisolone was commenced. He made good recovery with the treatment. Prompt recognition of this rare condition following chlamydia infection is important to guide the management.
, Erin K. Meyer, Kimberly W. Sanford, Sarita K. Joshi, Edward C. C. Wong, Jay S. Raval
Published: 16 October 2020
Journal of clinical apheresis, Volume 36, pp 161-176;

The publisher has not yet granted permission to display this abstract.
, R. Trollmann, U. Felderhoff-Müser, G. Bernert, A. Hackenberg, M. Hufnagel, M. Pohl, G. Hahn, H.J. Mentzel, C. Sommer, et al.
Published: 1 March 2020
European Journal of Paediatric Neurology, Volume 25, pp 5-16;

This evidence- and consensus-based practical guideline for the diagnosis and treatment of Guillain-Barré Syndrome (GBS) in childhood and adolescence has been developed by a group of delegates from relevant specialist societies and organisations; it is the result of an initiative by the German-Speaking Society of Neuropediatrics (GNP), and is supported by the Association of Scientific Medical Societies (AWMF, Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften). A systematic analysis of the literature revealed that only a few adequately-controlled studies exist for this particular age group, while none carries a low risk of bias. For this reason, the diagnostic and therapeutic recommendations largely rely on findings in adult patients with GBS, for which there are a higher number of suitable studies available. Consensus was established using a written, multi-step Delphi process. A high level of consensus could be reached for the crucial steps in diagnosis and treatment. We recommend basing the diagnostic approach on the clinical criteria of GBS and deriving support from CSF and electrophysiological findings. Repetition of invasive procedures that yield ambiguous results is only recommended if the diagnosis cannot be ascertained from the other criteria. For severe or persistently-progressive GBS treatment with intravenous immunoglobulin (IVIG) is recommended, whereas in cases of IVIG intolerance or inefficacy we recommended treatment with plasmapheresis. Corticosteroids are ineffective for GBS but can be considered when acute onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) is suspected due to a prolonged disease course. The full German version of the Guideline is available on the AWMF website (
, Cynthia Wang
Therapeutic Advances in Neurological Disorders, Volume 13;

A multitude of environmental factors can result in breakdown of immune tolerance in susceptible hosts. Infectious pathogens are among the most important environmental triggers in the pathogenesis of autoimmunity. Certain autoimmune disorders have a strong association with specific infections. Several neurological autoimmune disorders are thought to occur through post-infectious mechanisms. In this review, we discuss the proposed mechanisms underlying pathogen-induced autoimmunity, and highlight the clinical presentation and treatment of several post-infectious autoimmune neurological disorders. We also highlight post-infectious neurological disorders in the setting of recent outbreaks.
, , , Iraklis Vastardis, Hendrik P. Scholl, Nikos Kozeis
Klinische Monatsblatter fur Augenheilkunde, Volume 236, pp 469-471;

Das Miller-Fisher-Syndrom wurde 1956 erstmalig bei Patienten mit akuter Ophthalmoplegie, Ataxie und fehlenden Sehnenreflexen (Areflexie) beschrieben [1]. Es stellt eine Variante des Guillain-Barré-Syndroms dar und manifestiert sich selten auch im Kindesalter. Wir berichten über einen jungen Patienten mit Miller-Fisher-Syndrom, der mit Immunglobulinen und Methylprednisolon behandelt wurde.
Yuanyuan Huang, Zhaojian Ying, Weiwei Quan, Weiwei Xiang, Dewei Xie, , Xiang Li, ,
Published: 3 January 2018
International Journal of Neuroscience, Volume 128, pp 729-735;

Purpose/Aim of the study: Guillain Barré syndrome (GBS) is a severe peripheral nervous disease that leads to muscle weakness and areflexia. We now commonly accept a synthesis that inflammation and immunity play key role in GBS pathogenesis. Many studies pointed out that neutrophil-to-lymphocyte ratio (NLR) and monocyte-to-lymphocyte ratio (MLR) are novel promising markers of inflammation or immunity. Our study aimed to evaluate whether the NLR and the MLR were associated to GBS or not. Materials and Methods: We measured blood cell count in 334 individuals including 117 GBS and 217 healthy controls. Results: Our findings demonstrated that the GBS patients had higher levels of NLR and MLR than the healthy controls. The severe group also had higher levels of NLR and MLR compared to the mild group. We took the method of receiver-operating characteristic curve to find out the cut-off value of NLR for GBS occurrence and severity; it was 2.295 and 3.05, respectively. The cut-off values of MLR for GBS incidence and severity were the same, it was 0.235. Conclusion: In the setting of GBS, the NLR and MLR were significantly increased and they may be pathophysiologically and clinically relevant in GBS. The NLR and MLR would be new biomarkers of medical application.
Peter D. Donofrio
CONTINUUM: Lifelong Learning in Neurology, Volume 23, pp 1295-1309;

Purpose of Review: This article reviews the current state of Guillain-Barré syndrome (GBS), including its clinical presentation, evaluation, pathophysiology, and treatment.
Samuel S. Duffy, Brooke A. Keating, Chamini J. Perera,
Published: 10 May 2017
Journal of Neuroscience Research, Volume 96, pp 951-968;

The publisher has not yet granted permission to display this abstract.
, Maria Roberta Longo
Published: 1 January 2017
Autoimmunity Reviews, Volume 16, pp 96-101;

The term Guillain-Barré syndrome (GBS), the most frequent cause of acute paralytic neuropathy, covers a number of recognisably distinct variants. The exact cause of GBS is unknown, but 50-70% of cases appear 1-2weeks after a respiratory or gastrointestinal infection, or another immune stimulus that induces an aberrant autoimmune response targeting peripheral nerves and their spinal roots. The interplay between the microbial and host factors that dictate whether and how the immune response shifts towards autoreactivity is still unclear, and nothing is known about the genetic and environmental factors that affect an individual's susceptibility to the disease. All patients with GBS need meticulous monitoring, and can benefit from supportive care and the early start of specific treatment. This review summarises the clinical features and diagnostic criteria of GBS and proposes an algorithm for its management. An analysis of the literature showed that, about one century after it was first described, new information concerning its etiopathogenesis has allowed the development of new treatment strategies that should be started immediately after diagnosis; however, the available therapies are not sufficient in many patients, especially in the presence of the acute inflammatory demyelinating polyneuropathy. New post-infectious forms, such as those caused by Zika virus and enterovirus D68, need to be carefully analysed and, in order to improve patient outcomes, research should continue to aim at identifying new biomarkers of disease severity and better means of avoiding axonal injury.
, Shalini Desai, Dorothy Moore, Barbara J. Law, , ,
The Pediatric infectious disease journal, Volume 34, pp 1411-1413;

Guillain–Barré syndrome (GBS) cases admitted to Canadian pediatric tertiary care centers were ascertained through active surveillance. From 1996 to 2012, 246 cases were identified, and 24 (10%) had onset ≤30 days after immunization. Annual rate of postimmunization GBS was 2.0 per 100,000 hospitalizations. Postimmunization GBS was an infrequent cause of pediatric hospitalization.
, Véronique Manel, Dorothée Ville, Etienne Javouhey, Fabienne Bordet
Published: 26 October 2015
Child Neurology Open, Volume 2;

Guillain-Barré syndrome is a rare acute polyradiculoneuropathy. Several variants and unusual presentations have been described, particularly in pediatrics. In most cases, making an early diagnosis is challenging due to the treatments that consist in the rapid administration of intravenous immunoglobulin or plasma exchange. The authors present the case of a 7-year-old boy with an atypical and severe axonal Guillain-Barré syndrome, associated with Mycoplasma pneumonia. When he was admitted, febrile respiratory failure was the main focus, and then he presented signs of acute polyneuropathy with cranial nerve palsy and brief hyperreflexia. Mechanical ventilation was required for 48 days as well as 2 cycles of intravenous immunoglobulin. The authors describe all the medical challenges that the authors encountered. This case highlights the fact that respiratory distress can be the main clinical symptom in children. This delays the establishment of a correct diagnosis, even more so when neurological manifestations are abundant and unusual.
, Giovanna Zanoni
Published: 14 November 2014
Journal: L'Endocrinologo
L'Endocrinologo, Volume 15, pp 260-279;

The publisher has not yet granted permission to display this abstract.
Scott A. Bloch, Mahsa Akhavan, Jahn Avarello
Published: 30 January 2013
Case Reports in Emergency Medicine, Volume 2013, pp 1-5;

Guillain-Barre syndrome (GBS) is an acquired disease of the peripheral nervous system which causes demyelination and leads to weakness, ataxia, and areflexia. There are a variety of forms of the syndrome, and although it is found in all age groups, it is rare in children less than two years of age. The present complaint of weakness, ataxia, or lower extremity pain in the pediatric population should cause the practitioner to consider GBS in the differential. We describe a case of a 14-month-old girl presenting with weakness and the inability to ambulate who was diagnosed with GBS. The purpose of this paper is to review the emergency medicine diagnosis and management of Guillain-Barre syndrome in children.
Published: 1 January 2013
Guillain-Barré syndrome (GBS) is an acute, immune-mediated, postinfectious polyneuropathy with symmetrical ascending weakness, diminished deep tendon reflexes, and nonspecific sensory symptoms. CSF protein is raised with normal or only slightly elevated cell count. Based on electrophysiological and pathological findings, a demyelinating variant (acute inflammatory demyelinating polyneuropathy, AIDP) and an axonal variant (acute motor axonal neuropathy, AMAN) can be differentiated. Molecular mimicry with common epitopes between infective agents and peripheral nerves is discussed as an important pathophysiological principle. The symptoms progress for a mean of 10 days (up to 4 weeks) and after a plateau of 1-2 weeks remit spontaneously. At the height of the disease 60% of children are unable to walk and 10-15% need artificial ventilation. Treatment with plasmapheresis and intravenous immunoglobulins (IVIG) has been proven in placebo-controlled studies in adults with severe disease to speed up recovery significantly. In children, mostly open studies have shown similar treatment effects, although their spontaneous course is frequently less severe. Children with GBS should be treated with IVIG when they have lost the ability to walk, or when they are still deteriorating significantly and are expected to lose the ability to walk. The long-term prognosis is more favorable than that in adults. Whereas 25% of patients maintain mild neurological symptoms and signs, disability in the long term is very rare and usually due to complications such as myelitic involvement or chronic inflammatory demyelinating polyneuropathy (CIDP).
, , , A. Verrotti, D. Castellano-Chiodo, F. Greco, R. Falsaperla, L. Pavone
International journal of immunopathology and pharmacology, Volume 25, pp 513-517;

Guillain—Barré syndrome (GBS) is an inflammatory polyneuropathy characterized by acute onset, rapid progression, symmetric muscular weakness, pain, and paresthesias. The incidence of GBS in the pediatric age group is 0.8 cases per 100,000; 50%-70% of the cases are preceded by respiratory or gastrointestinal infectious episodes or vaccination. The etiopathogenesis of GBS has been hypothesized to involve a direct immune-mediated mechanism against the peripheral nerves. A series of 20 patients managed in the Department of Pediatrics of the University of Catania between 2003 and 2011 and evaluated according to epidemiologic, clinical, and therapeutic features is reported.
John T. Sladky, Stephen Ashwal
Published: 1 January 2012
The publisher has not yet granted permission to display this abstract.
C. Gitiaux
Published: 1 January 2011
EMC - Pédiatrie - Maladies infectieuses, Volume 6, pp 1-8;

Published: 31 December 2010
Seminars in pediatric neurology, Volume 17, pp 245-253;

Autoimmune diseases make up a significant portion of the acute and chronic caseload of all pediatric neurologists. By comparing these diseases and their treatments side by side, common themes become evident. Therapeutic decisions follow patterns dependent on the clinical situation. Physicians must adapt therapy based on individual clinical responses. This article provides an overview of the current therapeutic options as they relate to the more common pediatric neuroimmune disorders.
Eitan Israeli, Nancy Agmon-Levin, Miri Blank, Joab Chapman,
Published: 5 October 2010
Clinical Reviews in Allergy & Immunology, Volume 42, pp 121-130;

Guillain–Barré syndrome (GBS) is a rare autoimmune disorder, the incidence of which is estimated to be 0.6–4/100,000 person/year worldwide. Often, GBS occurs a few days or weeks after the patient has had symptoms of a respiratory or gastrointestinal microbial infection. The disorder is sub-acute developing over the course of hours or days up to 3 to 4 weeks. About a third of all cases of Guillain–Barré syndrome are preceded by Campylobacter jejuni infection. C. jejuni strains isolated from GBS patients have a lipooligosaccharide (LOS) with a GM1-like structure. Molecular mimicry between LOS and the peripheral nerves as a cause of GBS was demonstrated in animal models of human GBS. Following the “swine flu” virus vaccine program in the USA in 1976, an increase in incidence of GBS was observed and the calculated relative risk was 6.2. Later studies have found that influenza vaccines contained structures that can induce anti-GM1 (ganglioside) antibodies after inoculation into mice. More recent information has suggested that the occurrence of GBS after currently used influenza and other vaccines is rare. GBS involves genetic and environmental factors, may be triggered by infections or vaccinations, and predisposition can be predicted by analyzing some of these factors.
, Annick Galetto, Charles A. Haenggeli, Alain Gervaix
Published: 1 June 2010
The Journal of Emergency Medicine, Volume 38;

Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in childhood. Respiratory symptoms can mask neurologic signs, leading to a delay in diagnosis.
, Mersini Mavrikou, Konstantinos A. Voudris, Lela Stamoyannou
Published: 1 June 2009
Clinical Pediatrics, Volume 49, pp 400-403;

The authors present the case of a 6.5-year-old girl with bilateral temporomandibular joint (TMJ) pain, generalized arthralgias, inability to walk, and absence of deep tendon reflexes in the context of Guillain—Barrè syndrome. TMJ pain was the sole manifestation for 3 days, before other typical symptoms appeared, an issue that initially led to an improper diagnosis. A thorough clinical examination along with laboratory and radiographic evaluation excluded other possible causes of TMJ pain. To the best of the authors’ knowledge, this is the first case of Guillain—Barrè syndrome in the pediatric population initially presenting with bilateral TMJ pain. Guillain—Barrè syndrome may be quite atypical in its expression, especially in young children, with pain being a common presenting symptom, and pediatricians should be alert to avoid misdiagnosis.
, Yoram Nevo
Published: 31 May 2009
European Journal of Paediatric Neurology, Volume 13, pp 209-218;

Immune-mediated polyradiculoneuropathies are divided into Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). In children subacute inflammatory demyelinating polyradiculoneuropathy is included in CIDP. Immune polyradiculoneuropathies are not exclusively demyelinating, and axonal forms also responding favourably to immunotherapy occur. Evidence-based data on efficacy of therapy in children is lacking, relying on retrospective data, open label studies on small numbers of children, and mainly adult derived data. Immunotherapy (intravenous human immunoglobulin [IVIg] and plasmapheresis) shortens GBS recovery time with most children recovering completely. Childhood CIDP usually responds to corticosteroids and slow tapering is required to prevent relapses. IVIg and plasmapheresis are also effective. CIDP children resistant to steroids, IVIg, and steroid-dependent patients present a therapeutic challenge. Immunosuppressive agents including methotrexate, azathioprine and cyclosporine are helpful in some cases. Anecdotal reports of treatment with interferons alpha or beta and monoclonal antibodies against specific B-cell antigens (Rituximab, Alemtuzumab) have been described in limited case reports. Childhood CIDP prognosis is mostly favourable. However, a proportion of cases have residual neurological deficit.
J Schessl, M Koga, K Funakoshi, , W Muellges, A Weishaupt, R Gold,
Published: 1 January 2007
by BMJ
Archives of Disease in Childhood, Volume 92, pp 48-52;

Background: Antiganglioside antibodies have been reported to play a part in the pathophysiology of Guillain–Barré syndrome (GBS). Aims: To investigate the prevalence and correlation of anti-ganglioside antibodies with clinical data in children with GBS in a multicentre clinical trial. Methods: Immunoglobin (Ig)G and IgM to GM1, GM1b, GD1a, GalNAc–GD1a, GD1b, GT1a, and GQ1b were measured by ELISA in sera obtained before treatment. In addition, serological testing for Campylobacter jejuni was carried out. In parallel, a group of adults with GBS and a control group of children without GBS or other inflammatory diseases were evaluated. Results: Sera from 63 children with GBS, 36 adults with GBS and 41 children without GBS were evaluated. Four of the children with GBS showed positive IgG to GM1, in one case combined with anti-GalNAc–GD1a and in one with anti-GD1b. Two others showed isolated positive IgG to GD1b and GT1a. One showed increased anti-GalNAc–GD1a IgM. In 5 of the 63 children, serological evidence of a recent infection with C jejuni was found, and this correlated significantly with the raised antibodies (p = 0.001). In the control group without GBS, no child showed positive IgG, but one showed anti-GalNAc–GD1a IgM. Compared with the adults with GBS, the frequency of antibodies in children was insignificantly lower. In our study, patients with positive antibodies did not show a more severe GBS course or worse outcome than those who were seronegative, and we could not show an increased incidence of axonal dysfunction. Conclusions: In some children with GBS, one can detect raised IgG against various gangliosides, similar to that in adults. A recent infection with C jejuni is markedly associated with the presence of these antibodies. However, in contrast with what has been reported in adults, in this study we were unable to show a negative effect of these findings on the clinical course.
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