Guillain-Barre Syndrome Publications (7377)

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Guillain-Barre Syndrome Publications

2014Aug
Clin Neurophysiol
Clin Neurophysiol 2014 Aug 21. Epub 2014 Aug 21.
Service of Neurology, University Hospital "Marqués de Valdecilla", IDIVAL, UC and CIBERNED, Santander, Spain. Electronic address: jaberciano@humv.es.

Although prevailing spinal nerve involvement has been recognized in a few detailed Guillain-Barré syndrome (GBS) autopsy reports, imaging studies addressing this question in cervical nerves are lacking.
We describe clinical, electrophysiological, ultrasonographic (US) and pathological findings in six consecutive early GBS patients, evaluated within 10days of onset.
Patients' ages ranged from 37 to 80years.Read More

Five patients required mechanical ventilation, two of them having died 9 and 28days after onset. Upper- and lower-limb nerve US showed abnormal findings in just 8.8% of scanned peripheral nerves. In comparison with 46 aged-matched control subjects, US of the fifth to seventh cervical nerves showed changes in four cases, which consisted of significant nerve enlargement, blurred boundaries of the corresponding ventral rami, or both. Autopsy study in one case demonstrated that pathology, consisting of demyelination and endoneurial inflammatory oedema, mainly involved cervical and lumbar nerves.
In early GBS inflammatory oedema of spinal nerves is a pathogenically relevant feature to understanding the mechanism of ascending paralysis, particularly when conventional electrophysiological studies are normal or not diagnostic.
Findings advocate the use of cervical nerve US in early GBS.

2013Sep
Balkan Med J
Balkan Med J 2013 Sep 1;30(3):337-41. Epub 2013 Sep 1.
Department of Pediatrics, Şişli Etfal Training and Research Hospital, İstanbul, Turkey.

Pharyngeal-cervical-brachial (PCB) variant is a rare form of Guillan-Barre Syndrome (GBS). Antibodies against other membrane proteins like GM1b and GD1a have been found only in a small number of patients with Guillan Barre syndrome variant.
Here, we report a 5.Read More

5 year-old boy diagnosed early with positive GD1a and GD1b gangliosides of Guillan-Barre syndrome pharyngeal cervical-Brachial variant, who improved and recovered fully in a short period. This is in contrast to those whose recovery period prolongs in spite of early diagnosis and appropriate treatment and/or those who experience incomplete recovery.
In summary, diagnosis of PCB variant of GBS should be considered in infants with sudden onset bulbar symptoms and muscle weakness, and it should be kept in mind that early diagnosis and appropriate treatment can give successful outcomes.

2013Sep
Balkan Med J
Balkan Med J 2013 Sep 1;30(3):327-8. Epub 2013 Sep 1.
Department of Otolaryngology, Çanakkale Onsekiz Mart University Faculty of Medicine, Çanakkale, Turkey.

Sudden hearing loss developing after immunisation is a very rare situation. Rabies is a viral disease characterised by encephalitis and death. Treatment involves active and passive immunisation.Read More

Neurologic complications including Guillain-Barre syndrome or facial paralysis are reported in the literature as a side effect after rabies immunisation.
Sudden hearing loss was detected in an 11 year-old male patient who had taken the medication for rabies immunisation.
This study presents a case report of sudden hearing loss developing after rabies immunisation - no other aetiological factors were detected and clinical management is discussed in light of the literature.

2014Jan
Neural Regen Res
Neural Regen Res 2014 Jan;9(1):101-10
Department of Neurology, the First Bethune Hospital, Jilin University, Changchun 130021, Jilin Province, China.

Critical illness polyneuropathy and critical illness myopathy are frequent complications of severe illness that involve sensorimotor axons and skeletal muscles, respectively. Clinically, they manifest as limb and respiratory muscle weakness. Critical illness polyneuropathy/myopathy in isolation or combination increases intensive care unit morbidity via the inability or difficulty in weaning these patients off mechanical ventilation.Read More

Many patients continue to suffer from decreased exercise capacity and compromised quality of life for months to years after the acute event. Substantial progress has been made lately in the understanding of the pathophysiology of critical illness polyneuropathy and myopathy. Clinical and ancillary test results should be carefully interpreted to differentiate critical illness polyneuropathy/myopathy from similar weaknesses in this patient population. The present review is aimed at providing the latest knowledge concerning the pathophysiology of critical illness polyneuropathy/myopathy along with relevant clinical, diagnostic, differentiating, and treatment information for this debilitating neurological disease.

2014Sep
World Neurosurg
World Neurosurg 2014 Sep 5. Epub 2014 Sep 5.
Dept of Neurosurgery, Sree Balaji Medical College & Hospital, Bharath University, Chennai, India.

A case of the Guillain-Barré syndrome occurring after otherwise uneventful cardiac surgery using cardiopulmonary bypass is presented. Though the Guillain-Barré syndrome has been reported after surgical procedures, there are very few case reports after cardiopulmonary bypass surgery in the literature. The exact pathophysiological cause of the syndrome is still unknown.Read More

However, the most widely accepted hypothesis is that the syndrome is the result of an immune-mediated process. Cardiac surgery may be a trigger for immune-mediated response.

2014Sep
Clin Rehabil
Clin Rehabil 2014 Sep 8. Epub 2014 Sep 8.
Department of Health Sciences, Lund University, Lund, Sweden.

The aim was to describe experiences of disability in everyday life and managing the recovery process two years after falling ill with Guillain-Barré syndrome.
Qualitative interview study.
Interviews were conducted with 35 persons (22 male, mean age 50 years) two years after the onset of Guillain-Barré syndrome.Read More

The interviews were transcribed verbatim and analysed using content analysis.
The analysis revealed four categories and an overall theme: 'Striving for balance in everyday life'. The participants described persistent lived body restrictions that affected their arms, legs, and face. Bodily symptoms and loss of energy limited or restricted many everyday activities. In connection with healthcare, both satisfaction and feeling vulnerable in a critical situation were described. Experiences of the recovery process varied. The participants described acceptance and reappraisal of a new life situation despite their limitations, and having gained the knowledge that life can change suddenly. However, they also expressed disappointment following an overly positive prognosis in the early stages, and over a continuous wait for recovery. For some participants life had returned to as before.
The participants experienced limitations in everyday life and decreased functioning in several parts of the body. The recovery process may still be ongoing two years after onset. Rehabilitation intervention with an extended focus on supporting individualized coping processes could facilitate ways to live with persistent disability.

2014Jun
Stand Genomic Sci
Stand Genomic Sci 2014 Jun 15;9(3):1144-58. Epub 2014 Mar 15.
Unité de Recherche sur les Maladies Infectieuses et Tropicales Emergentes, Institut Hospitalo-Universitaire Méditerranée-Infection, Faculté de médecine, Aix-Marseille Université, Marseille cedex 05, France.

Collinsella massiliensis strain GD3(T) is the type strain of Collinsella massiliensis sp. nov., a new species within the genus Collinsella.Read More

This strain, whose genome is described here, was isolated from the fecal flora of a 53-year-old French Caucasoid woman who had been admitted to intensive care unit for Guillain-Barré syndrome. Collinsella massiliensis is a Gram-positive, obligate anaerobic, non motile and non sporulating bacillus. Here, we describe the features of this organism, together with the complete genome sequence and annotation. The genome is 2,319,586 bp long (1 chromosome, no plasmid), exhibits a G+C content of 65.8% and contains 2,003 protein-coding and 54 RNA genes, including 1 rRNA operon.

2014Sep
Eur. J. Neurol.
Eur J Neurol 2014 Sep 8. Epub 2014 Sep 8.
Division of Clinical Neurosciences, Turku University Hospital, Turku, Finland; Department of Neurology, University of Turku, Turku, Finland.

A Swiss study recently reported surgery as a potential risk factor for developing Guillain-Barré syndrome (GBS). It was sought to establish this in the Finnish adult population.
Persons over 16 years of age who received a diagnosis of GBS in 2004-2013 were identified from the patient register of Turku University Hospital and their patient records were analyzed to identify possible triggers.Read More


A cohort of 69 adult patients with GBS (63.8% men) was identified giving an annual incidence of 1.82/100 000. Of these, four (5.8%) had experienced a surgical procedure during the preceding 6 weeks with a relative risk of 6.28 (95% confidence interval 4.15-9.47, P < 0.001) compared with the general study population or a risk of 1.25/100 000 operations. No difference between genders was found. Only two (2.9%) patients had received a vaccination [one against seasonal influenza (P = 0.888) and one against pandemic influenza (Pandemrix(®) , GlaxoSmithKline Biologicals, Rixensart, Belgium, relative risk 2.85, 95% confidence interval 1.27-6.38, P = 0.011)] during the preceding 6 weeks. The most common GBS triggers identified were respiratory tract infections in 30 cases (43.5%) and gastroenteritis in 16 cases (23.2%) whilst two patients (2.9%) had had both.
The overall incidence of GBS in the adult population of southwestern Finland was similar to previous studies worldwide and the most common triggers were respiratory tract infections and gastroenteritis. Surgery was a rare risk factor and of vaccinations only the one against pandemic influenza raised the risk of GBS.

2014Sep
Case Rep Transplant
Case Rep Transplant 2014 11;2014:685010. Epub 2014 Aug 11.
Department of Pulmonary & Critical Care, University of Alabama at Birmingham, AL, USA ; UAB ECMO Program, Cardiothoracic Transplant, University of Alabama at Birmingham, 619 19th Street S., Jefferson Tower 1102, Birmingham, AL 35294, USA.

Guillain-Barré syndrome (GBS) has been described after solid organ and bone marrow transplantation mostly due to viral infections and possibly calcineurin inhibitors. Incidence after bone marrow transplant is 0.3-0.Read More

7%, though incidence in other transplants is not well known. We present the first description of tacrolimus associated GBS in lung transplant recipients in the English language literature. The pathophysiology of tacrolimus-induced polyneuropathy is not known, but some have hypothesized that tacrolimus induces an inflammatory phenomenon by differential effects on T cell subsets. Diagnosis of association may be challenging and requires high index of suspicion. The optimal treatment of GBS-associated with tacrolimus after lung transplantation is unknown, although drug discontinuation may result in improvement in some patients, while some reports suggest that the use of IVIG and/or plasmapheresis may be helpful and safe in organ transplant recipients with severe symptoms.

2014May
Asian Pac J Trop Biomed
Asian Pac J Trop Biomed 2014 May;4(Suppl 1):S70-2
Soundarya Mahalingam, Associate Professor, Department of Paediatrics, Kasturba Medical College, (affiliated to Manipal University), Mangalore, India.

The clinical spectrum of dengue fever ranges from asymptomatic infection to dengue shock syndrome. Dengue is classically considered a non-neurotropic virus. Neurological complications are not commonly seen in dengue.Read More

The neurological manifestations seen in dengue are encephalitis, meningitis, encephalopathy, stroke and Guillain-Barré syndrome. Dengue encephalitis is a rare disease. We report an interesting case of dengue encephalitis from Southern India. A 49-year-old gentleman presented with fever, altered sensorium and seizures. Dengue NS-1 antigen test was reactive. Dengue IgM was also positive. CSF PCR was negative for herpes simplex 1 & 2. Dengue encephalitis should be considered in the differential diagnosis of fever with altered sensorium, especially in countries like India where dengue is rampant.

2014Sep
BMC Neurol
BMC Neurol 2014 3;14(1):174. Epub 2014 Sep 3.
Teaching Hospital, Kandy, Sri Lanka. sonaligunatilake@gmail.com.

Guillain-Barré syndrome is an immune mediated acute inflammatory polyradiculo-neuropathy involving the peripheral nervous system. Commonest presentation is acute or subacute flaccid ascending paralysis of limbs. Rarely autonomic dysfunction can be the presenting feature of Guillain-Barré syndrome.Read More

Raynaud's phenomenon, although had been described in relation to many disease conditions, has not been described in association with Guillain-Barré syndrome up to date.
We report the first case of Guillain-Barré syndrome presenting with Raynaud's phenomenon in a 21-year-old previously well boy. New onset Raynaud's phenomenon was experienced followed by acute ascending flaccid paralysis of lower limbs and upper limbs together with palpitations and postural giddiness. Nerve conduction studies showed acute inflammatory demyelinating polyneuropathy with cerebrospinal fluid cyto-protein dissociation. He was treated with intravenous immunoglobulin and showed a satisfactory clinical recovery of muscle weakness, Raynaud's phenomenon and autonomic disturbances.
Guillain-Barré syndrome presenting with Raynaud's phenomenon is not being reported in literature previously. Although the underlying mechanism is not fully understood, Raynaud's phenomenon should prompt the physician to consider Guillain-Barré syndrome with a complimentary clinical picture.

2014Aug
Autoimmun Rev
Autoimmun Rev 2014 Aug 27. Epub 2014 Aug 27.
Department of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Unit 09-01, Centre for Translational Medicine, 14 Medical Drive, 117599, Singapore; Department of Physiology, Yong Loo Lin School of Medicine, National University of Singapore, Unit 09-01, Centre for Translational Medicine, 14 Medical Drie, 117599, Singapore. Electronic address: mdcyuki@nus.edu.sg.

Guillain-Barré syndrome is classified into acute inflammatory demyelinating polyneuropathy and acute motor axonal neuropathy. Whereas autoantibodies to GM1 or GD1a induce the development of acute motor axonal neuropathy, pathogenic autoantibodies have yet to be identified in acute inflammatory demyelinating polyneuropathy and chronic inflammatory demyelinating polyneuropathy. This review highlights the importance of autoantibodies to peripheral nerve proteins in the physiopathology of acute inflammatory demyelinating polyneuropathy and chronic inflammatory demyelinating polyneuropathy.Read More

Moreover, we listed up other potential antigens, which may become helpful biomarkers for acute inflammatory demyelinating polyneuropathy and chronic inflammatory demyelinating polyneuropathy based on their critical function during myelination and their implication in hereditary demyelinating neuropathies.

2014Aug
Mol. Immunol.
Mol Immunol 2014 Aug 26. Epub 2014 Aug 26.
Division of Molecular Immunology, Medical Institute of Bioregulation, Kyushu University, Fukuoka 812-8582, Japan.

Axonal Guillain-Barré syndrome (GBS) is an autoimmune neuropathy characterized by limb weakness and/or paralysis due to the presence of autoantibodies against brain glycolipids. The immune receptors that recognize these autoimmune targets have not been described. In this study, 12 C-type lectin and 10 immunoglobulin-like receptors were screened for their potential ligands from the brain glycolipids, which are the binding targets for GBS autoantibodies.Read More

These glycolipids were GM1, GM2, GD1a, GD1b, GQ1b, crude gangliosides, and 3-O-sulfo-β-d-galactosylceramide C24:1 (designated as C24:1). A direct interaction between ligand and receptor was examined using an ELISA-based binding assay. C-type lectin (CLEC5a, SIGNR3) and immunoglobulin-like receptors (TREM2, TREM3, LMIR2, LMIR5, LMIR7, LMIR8) interacted with C24:1. In addition, TREM3 did bind to GQ1b. LMIR5 interacted with GD1a, GQ1b, and crude gangliosides. Binding with highest affinity was observed for the LMIR5-C24:1 interaction, which was selected for further verification. C24:1 was found to induce MCP-1 production, but not proinflammatory cytokines, in basophils. C24:1-induced MCP-1 production was significantly reduced in DAP12(-/-) basophils. Importantly, LMIR5 ligation by C24:1 resulted in NFAT activation through DAP12 in LMIR5-expressing reporter cells. Structural analysis showed that LMIR5 recognized the 3-O-sulfo-β-d-galactose moiety of C24:1. The findings indicated that C24:1 is a potential ligand for DAP12-coupled LMIR5.

2014Aug
Mol. Immunol.
Mol Immunol 2014 Aug 26. Epub 2014 Aug 26.
Division of Molecular Immunology, Medical Institute of Bioregulation, Kyushu University, Fukuoka 812-8582, Japan. Electronic address: vongsavanh@bioreg.kyushu-u.ac.jp.

Campylobacter jejuni causes gastroenteritis and Guillain-Barré syndrome. Host immunity plays an important role in the disease pathogenesis; however, little is known about the immune receptors for C. jejuni.Read More

We report here that C. jejuni targets C-type lectin (SIGNR1, SIGNR3) and immunoglobulin-like receptors (TREM2, TREM3, LMIR5, LMIR8). Among these, C. jejuni interacted preferentially with LMIR5, which was selected for further verification using reporter cells. LMIR5 ligation by C. jejuni activated transcriptional factor NFAT through adaptor protein DAP12. Furthermore, LMIR5 activators were identified as protein components, RNA-associated proteins, and 150-kDa high-molecular-weight glycoconjugates. This finding discloses potential receptors that might link C. jejuni to immunopathology.

2014Aug
Braz J Anesthesiol
Braz J Anesthesiol 2014 Sep-Oct;64(5):369-72. Epub 2014 Feb 12.
Universidade de Ribeirão Preto, Ribeirão Preto, SP, Brazil.

Guillain Barré syndrome (GBS) is an autoimmune neurological disease characterized by an acute or subacute demyelinating polyradiculoneuritis. It is an unusual event during pregnancy and a challenge for the anesthesiologist, due to the possibility of impairment of neuromuscular function and occurrence of respiratory complications in the postoperative period. The objective of this paper is to discuss the anesthetic management of a pregnant patient affected by the disease.Read More


Female patient, 30 years old, 38 weeks' pregnant, diagnosed with fetal death that occurred about a day, and with SGB. Cesarean section was performed under general anesthesia, progressing without complications perioperatively.
Although it is uncommon, GBS can affect pregnant women and the anesthesiologist may encounter such patients in his (her) daily practice. It is important to understand the peculiarities of GBS to adequately address the patient in the perioperative period, contributing to its better evolution.

2014Aug
Rev Bras Anestesiol
Rev Bras Anestesiol 2014 Sep-Oct;64(5):369-72. Epub 2013 Nov 11.
Universidade de Ribeirão Preto, Ribeirão Preto, SP, Brasil.

Guillain Barré syndrome (GBS) is an autoimmune neurological disease characterized by an acute or subacute demyelinating polyradiculoneuritis. It is an unusual event during pregnancy and a challenge for the anesthesiologist, due to the possibility of impairment of neuromuscular function and occurrence of respiratory complications in the postoperative period. The objective of this paper is to discuss the anesthetic management of a pregnant patient affected by the disease.Read More


Female patient, 30 years old, 38 weeks' pregnant, diagnosed with fetal death that occurred about a day, and with SGB. Cesarean section was performed under general anesthesia, progressing without complications perioperatively.
Although it is uncommon, GBS can affect pregnant women and the anesthesiologist may encounter such patients in his (her) daily practice. It is important to understand the peculiarities of GBS to adequately address the patient in the perioperative period, contributing to its better evolution.

2014Aug
J. Neurol. Neurosurg. Psychiatr.
J Neurol Neurosurg Psychiatry 2014 Aug 28. Epub 2014 Aug 28.
Department of Neuroscience and Imaging, University "G. d'Annunzio", Chieti-Pescara, Italy.

Patients with Parkinson's disease (PD) treated with oral levodopa have a higher prevalence of chronic, prevalently sensory, usually mild axonal polyneuropathy (PNP). Several studies showed a positive association among PNP, cumulative levodopa dosage, low serum B12 and high-homocysteine and methylmalonic acid level. Anecdotal severe acute or subacute PNPs thought to be Guillain-Barré syndrome have been reported in patients receiving continuous intraduodenal infusion of levodopa/carbidopa intestinal gel (LCIG).Read More

We report an additional acute case and by a systematic literature search we also reviewed the clinical and laboratory features of 13 other acute and 21 subacute PNP cases occurring during LCIG treatment. In series with at least nine patients, the mean frequency of acute and subacute PNP is 13.6% and the mortality rate at 6 months in acute cases is 14%. The great majority of PNP cases displayed axonal sensory-motor and reduced vitamin B12 levels, and alterations of metabolites of 1-carbon pathway were found in most patients. We discuss the possible role of high-levodopa dosage, vitamin B12, B6 and folate deficiency and accumulation of homocysteine and methylmalonic acid in the pathogenesis to conclude that there is enough, although circumstantial, evidence that alterations of 1-carbon pathway are implicated also in acute and subacute PNP during LCIG usage. There is no solid proof for a dysimmune pathogenesis and in our opinion acute, subacute and chronic PNP, either after oral levodopa or LCIG, represent a continuum. Finally, we propose recommendations for prevention and management of PNP occurring during LCIG treatment.

Guillain-Barré syndrome (GBS) and Fisher syndrome (FS) are immune-mediated peripheral neuropathies, and most of these cases were known to be associated with a preceding infection. Recent reports evidenced an increase in the number of infectious disease cases after the earthquake. The aim of this report is to investigate the incidence and clinical features of GBS and FS after the Great East Japan Earthquake.Read More

We found GBS and FS patients had markedly increased in 2011, the year of the earthquake. In regard to an antecedent illness, gastrointestinal infection was significantly increased in GBS patients after the earthquake. These results suggest environmental factors including infectious agents and stress caused by the earthquake might have been involved in the outbreak of the diseases.

2014Aug
Int. J. Neurosci.
Int J Neurosci 2014 Aug 26:1-23. Epub 2014 Aug 26.
1Department of Neurosurgery, University of Texas Health Sciences Center at San Antonio, San Antonio TX USA 78229.

ABSTRACT Guillain-Barré Syndrome (GBS) is a demyelinating polyneuropathy resulting in sensory, motor and autonomic symptoms. The severity of the disease can range from mild to severe but it is classically described as an ascending demyelinating process. Initially thought to be the sequelae of a bacterial or viral infection, the clinical symptoms of post-infective GBS can present up to four weeks after sentinel injury.Read More

A rarely defined post-surgical GBS has been since described after major cranial, cardiothoracic and gastro-intestinal surgery. Post traumatic GBS is an even more unusual presentation with very few cases reported in contemporary academic literature. We present a case of GBS presenting two weeks after non-operative traumatic brain injury and a review of the literature.

2014Aug
J. Neurol. Neurosurg. Psychiatr.
J Neurol Neurosurg Psychiatry 2014 Aug 25. Epub 2014 Aug 25.
Department of Neuroimmunology, The Erasmus University Medical Center, Rotterdam, The Netherlands.

Multifocal motor neuropathy (MMN) and the Guillain-Barré syndrome (GBS) are immune-mediated motor neuropathies with antibodies against the ganglioside GM1. In GBS, these antibodies are induced by molecular mimicry, but in MMN their origin is elusive.
We compared the light-chain use of anti-GM1 IgM antibodies in serum from 42 patients with MMN and 23 patients with GBS by ELISA.Read More


Exclusive use of either κ or λ light chains was found in 38 (90%) patients with MMN and 9 (39%) with GBS (p<0.001).
Anti-GM1 IgM antibodies in most patients with MMN are produced by only a single or very limited number of B-cell clones, whereas in most patients with GBS, anti-GM1 IgM antibodies are most likely polyclonal.

2014Aug
Curr Alzheimer Res
Curr Alzheimer Res 2014 ;11(7):623-5
Department of Translational Science and Molecular Medicine, Department of Family Medicine, Michigan State University, 333 Bostwick Ave NE, Grand Rapids, MI 49503, USA.

Epidemiologic studies suggest that we are on the precipice of a worldwide epidemic of Alzheimer's disease (AD), yet current treatment options are limited to short term symptomatic relief. Recent advances in our knowledge of the neurobiology of AD have resulted in the development of several potential disease-modifying approaches based on immunotherapy. The present special 'Hot Topic' (HT) issue of "Current Alzheimer Research" deals primarily with the mechanisms of passive vaccination with Intravenous Immunoglobulin (IVIG), particularly within the context of neuroprotection in preclinical models of AD.Read More

This HT issue is not meant to report exhaustively on the many other research efforts in the broader immunotherapy arena. Indeed, this journal has recently covered various other aspects of immunotherapy relevant to AD and related disorders. However, we will briefly overview current immunotherapeutic strategies for AD prior to discussing the main topic of IVIG neuroprotection. One of the most significant approaches involves the removal of brain amyloid-β peptide (Aβ) using anti-Aβ antibodies. Aβ immunotherapy emerged as a promising treatment strategy based on human neuropathology and preclinical studies. The hallmark accumulation of parenchymal and vascular Aβ pathology observed in the brains of AD subjects suggested a logical target, and naturally occurring anti-Aβ antibodies were found to be reduced in the cerebrospinal fluid and blood of AD patients [1, 2]. In addition, both active and passive amyloid immunization of AD transgenic mouse models resulted in increased clearance of amyloid plaquelike deposits and improved cognitive performance [3, 4], whereas brain imaging and neuropathological studies suggested the ability of both active and passive anti-Aβ immunotherapies to clear Aβ deposits from the AD brain. AN1792 was the first active immunotherapy strategy for AD using full length Aβ42 as the immunogen; however, a Phase II trial of this anti-amyloid vaccine was halted when meningo-encephalitis appeared in a small subset of patients [5]. Despite this setback, long-term follow-up of patients immunized with AN1792 showed reduced functional decline in antibody responders [6], supporting the hypothesis that Aβ immunotherapy may have long-term functional benefits. In this regard, novel Aβ immunogens with shorter peptide sequences are in development which may avoid the autoimmune responses to full length Aβ42 [7]. The first passive anti-Aβ immunotherapy for AD focused on bapineuzumab. Bapineuzumab, which is composed of humanized anti-Aβ monoclonal antibodies, reduced Aβ burden in the brains of AD patients in two Phase II trials. However, bapineuzumab did not improve clinical outcomes in patients with AD, despite treatment differences in biomarkers observed in APOE β4 carriers [8, 9]. Other recent approaches, such as systemic co-administration of clioquinol and Aβ42 vaccines, significantly reduce Aβ deposits in the brains of transgenic AD mice [10]. In non-rodent models, a rapid improvement of canine cognitive dysfunction with amyloid immunotherapy suggests the important use of the canine model in testing vaccines for AD [11]. So far, the limitations of Aβ-based immunotherapy include the development of encephalitis, the lack of clinical improvement, and the lack of effect on neurofibrillary tangles (NFTs), another major neuropathological feature of AD. Other critical points relate to the study design and several variables in imunotherapy trials, which are essential for optimizing trial designs and improving conditions for participants [12]. Due to the central role of NFTs in dementia, immunotherapy targeting these tau proteinous aggregates is an important area of research [13, 14]. Notably, an active immunotherapy targeting the tau pathological epitope phospho- Ser422 was found to be efficient, resulting in tau clearance and improved cognitive deficits promoted by tau pathology in a well-defined tau transgenic model [15]. Like Aβ oligomers, the putative role of tau oligomers in AD pathophysiology has prompted an investigation into tau oligomers as potential immunotherapeutic targets for AD and tauopathies [16]. Taken together, these results suggest that immunotherapies targeting Aβ alone may be insufficient for disease modification. To this end, researchers also began testing whether IVIG might serve as an alternative immunotherapeutic strategy. IVIG is a mixture of naturally occurring human IgG antibodies derived from the plasma of healthy young volunteers. Notably, IVIG has been used for nearly half a century for primary humoral immune deficiencies and autoimmune syndromes and, more recently, a number of neurologic disorders such as chronic inflammatory demyelinating polyradiculoneuropathy and Guillain-Barré syndrome [17, 18]. The rationale for using IVIG for the treatment of AD gained traction for a number of reasons. IVIG was found to contain elevated levels of antibodies against multiple conformations of Aβ monomers and aggregates [19, 20], yet its repertoire of naturally occurring antibodies might also be predicted to normalize the inflammatory component of AD. The safety profile of IVIG for other diseases also mitigated concerns for AD clinical trials. Furthermore, if IVIG was found to be beneficial in AD, the potential existed for identifying treatment-specific antibodies to elucidate pathogenic mechanisms and allow for more targeted therapeutic designs. However, despite the initial promise of Phase I and II clinical trials conducted in Germany and the US, a recent multicenter double-blinded Phase III study of 390 subjects, called the Gammaglobulin Alzheimer's Partnership (GAP), did not meet primary endpoints of slowing cognitive and functional decline [21]. Then again, the GAP study results continued to support IVIG's positive safety profile and showed potentially beneficial effects for pre-specified moderate AD and apoE4 carrier subgroups. Concurrent with these clinical trials, several preclinical studies demonstrated that IVIG was neuroprotective against Aβ toxicity in vitro and enhanced microglia- mediated Aβ clearance ex vivo, whereas in vivo IVIG delivery reduced inflammation in AD transgenic mice [22, 23]. Hence, the mechanism of action for IVIG is still of considerable interest in the field and there remains the opportunity for testing the extent to which optimized doses of IVIG delivered early enough in the AD trajectory might yet prove beneficial for modifying disease progression. In the present HT issue, we summarize the state of the field with respect to IVIG as a potential therapy for AD and explore further the potential mechanisms of IVIG neuroprotection in preclinical models of AD. Puli and colleagues review our current understanding of the biologic and therapeutic properties of IVIG relevant to AD therapy and highlight their in vitro and in vivo studies on IVIG biological activities, including the suppression of neuroinflammatory microglial activation and concomitant increase in neurogenesis in APP/PS1 mice [24]. Gong and colleagues expound upon IVIG immunomodulatory mechanisms by showing that IVIG regulates complement-derived anaphylatoxins, such as C5a and C3, which in turn upregulates AMPA receptor-PKACREB signaling pathways and improves synaptic function and cognition in the Tg2576 mouse model of AD [25]. Lahiri and Ray add to the diverse repertoire of IVIG neuroprotection by reporting that treatment with IVIG protects neuronal viability and synaptic proteins in primary rat hippocampal neurons as well as in primary human brain cultures challenged with oxidative stress, suggesting a potent neuropreservatory effect of IVIG against oxidative insults [26]. In addition, although IVIG has been reported to reduce amyloid burden in some AD transgenic models, its potential effects on tau NFT-like pathology in rodent models of disease are unclear. Counts and colleagues show that IVIG reduces hippocampal tau pathology in the 3xTg mouse model of AD that exhibits NFT as well as plaque-like deposits. In addition, this study reveals that IVIG preserves plasma levels of mRNAs regulating neuronal cytoskeletal plasticity function and calcium-mediated signaling compared to placebo [27]. It is important to note that not all IVIG preclinical studies have produced consistently positive results [28]. In this issue, Joly-Amado and colleagues describe how four weeks of IVIG infusion in Tg2576 mice led to widespread distribution of human IgG in the forebrain, but had no effect on amyloid burden or cognition [29]. However, the authors conclude by agreeing that the beneficial effects of IVIG in mouse models of AD are not likely due to its anti-Aβ antibody components alone, but must also involve its wide range of antiinflammatory, anti-oxidant, and other prosurvival and neuroprotective properties. Hence, despite the negative topline results from the GAP trial, these unique properties of IVIG suggest that this polyclonal IgG mixture can potentially be a safe and highly effective "top down" therapy for a complex multifactorial disease like AD. Moreover, the data derived from preclinical study designs may help guide current and future IVIG clinical trials targeting early stage patients with more optimized treatment regimens to prevent or delay the onset of AD symptomology [30]. Finally, since efforts to immunize against tau [31] and other AD-related targets have been encouraging, these studies would potentially be excellent subject matters for a future HT issue of the journal.

We presented a case of an 8-year-old boy with Guillain-Barré syndrome characterized by severe intractable pain in the soma and lower extremities, which appeared 2 weeks after a febrile cold. At his first visit to our hospital, he could not stand or walk because of the severe pain, and muscle weakness and absence of deep tendon reflexes were observed. Guillain-Barré syndrome was diagnosed on the basis of cerebrospinal fluid study results, nerve conduction velocity, and spinal cord magnetic resonance imaging.Read More

His pain was scored as a five on a six-point visual analog scale, and it persisted despite routine supportive therapy. The pain was successfully controlled with parenterally infused fentanyl. It is suspected that opioid analgesics are useful for severe pain control in patients with Guillain-Barré syndrome.

2014Aug
Zhonghua Xue Ye Xue Za Zhi
Zhonghua Xue Ye Xue Za Zhi 2014 Aug;35(8):694-7
Department of Hematology, Changhai Hospital Affiliated to Secondary Military Medical University, Shanghai 200433, China.

To investigate the clinical characteristics, treatment and prognosis of Guillain-Barre syndrome (GBS) after allogeneic hematopoietic stem cell transplantation (allo-HSCT).
Two cases with GBS after allo-HSCT were admitted to our hospital and a review of literatures concerning GBS developed after allo-HSCT. The clinical characteristics, treatment and prognosis were investigated.Read More


Two patients experienced sensory disturbance and progressive muscle weakness 2 months after HSCT. The diagnosis of GBS was established after cranial MRI, lumbar puncture and EMG. Both patients died of GBS progression even after the treatment of steroid, intravenous immunoglobulin (IVIG) and plasma exchange.
GBS was a rare complication after allo-HSCT. The common clinical practices in treating GBS included IVIG and plasma exchange. Due to the primary malignant disease and low immunity posttransplant, infection, GVHD and other complications, prognosis of GBS was poor with high mortality.

2014Aug
Adv Neurobiol
Adv Neurobiol 2014 ;9:543-66
Institute of Infection, Immunity and Inflammation, College of Medical, Veterinary and Life Sciences, University of Glasgow, Glasgow, UK, hugh.willison@glasgow.ac.uk.

A wide range of neuroimmunological diseases affect the central and peripheral nervous systems. These disorders are caused by autoimmune attack directed against structurally and functionally diverse nervous system antigens. One such category comprises peripheral nervous system (PNS) diseases, termed peripheral neuropathies, in which the target antigens for autoantibody-directed nerve injury are glycan structures borne by glycoproteins and glycolipids, particularly gangliosides that are concentrated in peripheral nerve.Read More

The archetypal PNS disorder is the acute paralytic disease, Guillain-Barré syndrome (GBS) in which autoantibodies against glycolipids arise in the context of acute infections that precede the clinical onset, notably Campylobacter jejuni enteritis. In addition, several chronic autoimmune neuropathies are associated with IgM antibodies directed against nerve glycans including sulphated glucuronic acid epitopes present on myelin-associated glycoprotein and sulphated glucuronyl paragloboside, a range of disialylated gangliosides including GD1b and GD3, and GM1 ganglioside. This chapter describes the immunological, pathological and clinical features of these disorders in the context of our broader knowledge of the glycobiology underpinning this neuroimmunological field.

2014Aug
J Stroke Cerebrovasc Dis
J Stroke Cerebrovasc Dis 2014 Aug 20. Epub 2014 Aug 20.
Department of Neurology, University Hospital Cologne, Cologne, Germany.

The association of a posterior reversible encephalopathy syndrome (PRES) without arterial hypertension with autoimmune-mediated inflammatory neuropathies such as Guillain-Barré syndrome (GBS) is a rare and poorly understood phenomenon. To date, PRES has been described as initial manifestation, coincidental finding, or adverse event subsequent to immunomodulatory treatment with intravenous immunoglobulin (IVIG) in cases of axonal and demyelinating GBS as well as in Miller-Fisher syndrome (MFS). We here report a case of MFS/Bickerstaff brain stem encephalitis (BBE)-overlap syndrome and nonhypertensive PRES that occurred in close temporal association with IVIG treatment and caused stroke.Read More

Immunoadsorption ameliorated the disease course. Our case supports the notion that in severe cases, immunoadsorption should be considered as first-line therapy instead of IVIG for rapid removal of IgG and thus to hasten recovery and improve functional outcome.

2014Aug
Eur J Pediatr Surg
Eur J Pediatr Surg 2014 Aug 21. Epub 2014 Aug 21.
Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.

Aim The ideal repair of esophageal atresia (EA) is primary anastomosis with closure of the fistula if present. Long gap or local circumstances prompt other procedures that occasionally lead to disastrous complications. The aim of this study was to analyze the management of these complications in a tertiary referral center.Read More

Patients and Methods A retrospective review of patients treated for EA between 1993 and 2013 was conducted. Both the patients were primarily treated by us, and referrals from elsewhere after two or more failed operations were included. Results In total, 23 patients were included (3/176 cases of EA treated primarily by us and 20 referrals). Of the 23 patients, 6 had type I EA, 15 type III (four long gaps), 1 type IV, and 1 type V. Cardiac anomalies were associated in seven cases, duodenal atresia in three, and Down syndrome in two patients. Primary anastomosis was initially achieved in 12 patients. Primary or secondary Foker lengthening was used in seven cases. The causes of the failure were anastomotic leaks in nine, unmanageable strictures in seven, and refistulization in five patients. These patients required 66 reoperations (median of 3 [2-7]) before inclusion in the study. Radical tertiary treatment consisted of 15 esophageal replacements (11 colonic grafts and 4 gastric pull-ups), and 1 esophageal-gastric disconnection. Five patients previously treated with esophageal replacement and referred for graft problems required 13 interventions. Two families did not give consent for one replacement and one disconnection. Complications appeared in 12 patients, and 9 additional operations were required in 7 patients. With a follow-up of 31 months (range, 4-139 months) 15 patients take all their meals per os, 5 occasionally use the gastrostomy, and 2 and 1 are fed exclusively via gastrostomy or jejunostomy. All tracheoesophageal fistulas were closed, but 15 cases are below p3 for weight and 12 for height. Three patients (13%) ultimately died 32 months (range, 9-56 months) after the first operation (due to aspiration in one, and for causes unrelated to it in the other two [tracheostomy obstruction and Guillain-Barré syndrome]). Conclusions When repeated complications appear after EA repair, radical surgical attitudes may be justified. If esophageal continuity cannot be reestablished, the native esophagus may have to be discarded and replaced. Many complications should be expected, but the end result can be good. These patients should be referred to centers with large experience in the management of this complex condition.

2014Sep
BMC Infect. Dis.
BMC Infect Dis 2014 20;14:448. Epub 2014 Aug 20.
School of Medicine, Faculty of Medical Sciences, University of Duhok, Duhok, Kurdistan Region, Iraq. jagar.jasem@osumc.edu.

Acute flaccid paralysis surveillance (AFP) is an essential strategy of the WHO's Polio Eradication Initiative. This is the first study conducted to estimate the incidence, etiology, distribution, and surveillance performance of AFP in Iraq.
Surveillance data about the AFP cases under the age of 15 years reported from Iraq during January 1997 to December 2011 were depended in the current study.Read More


A total of 4974 cases of AFP were reported from Iraq during the study period, with an annual incidence of 2.5/100,000 population. Guillain-Barré syndrome represented more than half of the reported cases (N = 2611, 52.5%), followed by traumatic neuritis (N = 715, 14.4%), and other CNS infections (N = 292, 5.9%). Poliomyelitis accounted for 166 (3.3%) of cases, the last reported case being in January 2000. Surveillance performance showed that all, but two, indicators were below the required WHO recommended levels.
AFP surveillance remains the gold standard method for poliomyelitis detection. It witnessed dramatic changes over the last two decades. This has raised people's and clinicians' awareness to the importance of promptness in notifying suspected cases and timely transportation of stool specimens to the National Poliovirus Laboratory in Baghdad, or alternatively having more than one laboratory for poliovirus detection in the country, all of which are very useful measures to increase the surveillance performance in the country.

2014Aug
Viral Immunol.
Viral Immunol 2014 Aug 20. Epub 2014 Aug 20.
1 Department of Neurology, Shanghai Jiao Tong University Affiliated Sixth People's Hospital , Shanghai, China .

Abstract Background: Guillain-Barré syndrome (GBS) is preceded by an infection in about two-thirds of patients. However, the infectious organism is often not identified. GBS secondary to Japanese encephalitis virus (JEV) infection has been reported only in India.Read More

Herein, we report a case of GBS preceded by JEV infection in China. Case presentation: A 23-year-old male had generalized weakness, numbness in the extremities, and bilateral facial nerve paralysis. One week prior, he had a high fever with headache, and several days later, he developed facial diplegia and sensory disturbances. Physical examination revealed facial diplegia and a weak gag reflex, quadriparesis more pronounced distally, generalized hyporeflexia, and no Babinski sign. JEV IgM and hepatitis B surface antibody (HbsAb) tests were positive. Other tests for hepatitis B infection were negative. Nerve electrophysiology suggested an acute demyelinating sensorimotor polyradiculoneuropathy. His cerebrospinal fluid was clear, the leukocyte count was 5×10(6)/L (normal range: 0-5×10(6)/L), protein 0.62 g/L (normal range: 0.15-0.45 g/L), and JEV IgM was weakly positive. He was diagnosed with GBS associated with a recent JEV infection. Intravenous (IV) immunoglobulins combined with IV methylprednisone was administered for 5 days, and at the 3-month follow-up, a complete neurological recovery was noted. Conclusion: GBS may be associated with JEV infection. GBS exhibits a good response to intravenous immunoglobulin or plasma exchange and has a good prognosis making prompt diagnosis important.

2014Jun
Klin. Mikrobiol. Infekc. Lek.
Klin Mikrobiol Infekc Lek 2014 Jun;20(2):50-4
Department of Infectious Diseases, Faculty Hospital Brno, Czech Republic, e-mail: pavel.polak@fnbrno.cz.

Campylobacteriosis belongs to the most frequent bacterial gastrointestinal infections worldwide. In the past several years, an increasing trend in the prevalence of campylobacteriosis has been observed in many countries. The rapid spread of antibiotic resistance in Campylobacter spp.Read More

also poses a major challenge. The authors review current knowledge on the microbiology of Campylobacter spp., complex pathogenetic as well as pathophysiological mechanisms in the development and course of campylobacteriosis and related complications such as Guillain-Barré syndrome.