Guillain-Barre Syndrome Publications (7364)

Guillain-Barre Syndrome Publications

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 September - October;64(5):369-372. 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.

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-Barre 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.

2014Aug
Saudi Med J
Saudi Med J 2014 Aug;35(8):861-4
Department of Pediatrics, Faculty of Medicine, King Abdulaziz University, PO Box 80215, Jeddah 21589, Kingdom of Saudi Arabia. Fax. +966 (12) 6408353. E-mail. osamam@hotmail.com.

Guillain-Barre syndrome (GBS) usually presents in a symmetrical ascending fashion of weakness. We present a 6-month-old male infant who presented to our emergency room with acute left-sided limb weakness and head lag 3 days after a febrile upper respiratory tract infection. A diagnosis of GBS was established by confirming high cerebrospinal fluid protein, motor nerve reduced amplitude, and prolonged conductions, and MRI T2 high signal intensity affecting the ventral roots of the spinal cord.Read More

He showed remarkable clinical and neurophysiological improvement after intravenous immunoglobulin and intensive physiotherapy. The occurrence of infantile acute hemiplegia as a presentation of GBS is rare. This report highlights the importance of considering GBS in the differential diagnosis so that early effective treatment may be started. 

2014Aug
J Foot Ankle Surg
J Foot Ankle Surg 2014 Aug 13. Epub 2014 Aug 13.
Chief, Podiatric Surgery Department, PinnacleHealth System, Harrisburg, PA.

Guillain-Barré syndrome is a serious disorder that occurs when the body's immune system mistakenly attacks the peripheral nervous system. This leads to nerve inflammation and damage that can cause muscle weakness and/or paralysis, including foot drop. Therapy ranges from supportive measures, such as physical therapy, to surgical intervention.Read More

It appears from the published data that these patients, generally, have been left to their own devices, because no known cure is available, necessitating reliance on others for their daily activities and ambulation. We present a unique surgical option, with a follow-up period of 24 months, in which bilateral subtalar and ankle fusion allowed unassisted ambulation to a patient who might otherwise have never walked again.

2014Jul
J. Neurol. Sci.
J Neurol Sci 2014 Jul 26. Epub 2014 Jul 26.
Institute of Neuroscience, Department of Clinical Neurophysiology, Uppsala University, Uppsala, Sweden. Electronic address: anna.rostedt.punga@neuro.uu.se.

T cells are important in the immunopathology of immune-mediated peripheral neuropathies (PNP) and activated vitamin D regulates the immune response through increasing the amount of regulatory T cells. An association between vitamin D deficiency and polyneuropathy has been stipulated; hence we assessed whether patients with primary immune-mediated PNP have low vitamin D [25(OH)D] levels.
Plasma levels of 25(OH)D were analyzed in 26 patients with primary immune-mediated PNP, 50 healthy matched blood donors and 24 patients with motor neuron disease (MND).Read More

INCAT score was assessed in patients with Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy. ALSFRS-R score was applied to MND patients and the modified Rankin (mRankin) scale compared disability among patient groups.
Mean 25(OH)D value in PNP patients was 40±16nmol/l, compared to 69±21nmol/l in healthy blood donors (p<0.001). MND patients had a higher mean 25(OH)D than PNP patients (59±26nmol/L; p=0.006) and comparable levels to healthy blood donors (p=0.15). Mean 25(OH)D value was not higher in PNP patients with pre-existing vitamin D3 supplementation of 800IU/day (N=6; 35±18nmol/L) than in unsupplemented PNP patients (42±16nmol). INCAT score ranged from 0 to 10 (mean 3.5) and ALSFRS-R ranged from 11 to 44 (mean 31). mRankin score was more severe in MND patients (mean 3.5) compared to PNP patients (mean 2.1).
All patients with primary immune-mediated PNP were diagnosed with vitamin D deficiency and they had significantly lower 25(OH)D values than healthy control persons and MND patients. We suggest monitoring of vitamin D status in patients with autoimmune PNP, since immune cells are responsive to the ameliorative effects of vitamin D.

2014Jul
J Emerg Trauma Shock
J Emerg Trauma Shock 2014 Jul;7(3):149-54
Department of Neurology, Post Graduate Institute of Medical Education and Research, Chandigarh, Punjab and Haryana, India.

Acute flaccid paralysis (AFP) is a complex clinical syndrome with a broad array of potential etiologies that vary with age. We present our experience of acute onset lower motor neuron paralysis.
One hundred and thirty-three consecutive adult patients presenting with weakness of duration less than four weeks over 12 months period were enrolled.Read More

Detailed history, clinical examination, and relevant investigations according to a pre-defined diagnostic algorithm were carried out. The patients were followed through their hospital stay till discharge or death.
The mean age was 33.27 (range 13-89) years with male preponderance (67.7%). The most common etiology was neuroparalytic snake envenomation (51.9%), followed by Guillain Barre syndrome (33.1%), constituting 85% of all patients. Hypokalemic paralysis (7.5%) and acute intermittent porphyria (4.5%) were the other important conditions. We did not encounter any case of acute polio mylitis in adults. In-hospital mortality due to respiratory paralysis was 9%.
Neuroparalytic snakebite and Guillain Barre syndrome were the most common causes of acute flaccid paralysis in adults in our study.

2014Sep
J. Formos. Med. Assoc.
J Formos Med Assoc 2014 Sep 8;113(9):634-9. Epub 2012 Sep 8.
Department of Neurology, First Hospital, Jilin University, Changchun, PR China.

Since little has been reported in previous studies, we aimed to find the clinical and electrophysiologic characteristics associated with childhood Guillain-Barré Syndrome (GBS) in Northeast China.
The clinical and electrophysiologic data were collected and reviewed retrospectively in 33 children and 105 adults with GBS during the period between 2006 and 2010 from the First Hospital of Jilin University.
Most of the children with GBS were older than 8 years of age and symptoms were severe at GBS onset.Read More

Simultaneous involvement of four limbs was the most common clinical feature, and cranial nerve involvement was common; however, previous infection, sensory nerve involvement and elevated proteins in cerebrospinal fluid occurred much less in the children with GBS than those in adult patients. Recruited children were classified as having acute inflammatory demyelinating polyneuropathy (AIDP; 41%), acute motor axonal neuropathy (AMAN; 38%), and were unclassified (21%). Electrophysiologic features and prognosis in these children were not different from those in adults. For children with AMAN, the efficacy of intravenous immunoglobulin (IVIg) was not different from that in adults; however, IVIg was not significantly effective for AIDP in these children.
Childhood GBS in Northeast China exhibits characteristics of clinical and electrophysiologic alternations; early diagnosis and appropriate treatments should be provided accordingly.

2014Aug
Biomed Res Int
Biomed Res Int 2014 30;2014:936891. Epub 2014 Jun 30.
Department of Anatomy and Cell Biology, College of Medicine, Dong-A University, Busan 602-714, Republic of Korea.

Studies have shown that lysosomal activation increases in Schwann cells after nerve injury. Lysosomal activation is thought to promote the engulfment of myelin debris or fragments of injured axons in Schwann cells during Wallerian degeneration. However, a recent interpretation of lysosomal activation proposes a different view of the phenomenon.Read More

During Wallerian degeneration, lysosomes become secretory vesicles and are activated for lysosomal exocytosis. The lysosomal exocytosis triggers adenosine 5'-triphosphate (ATP) release from peripheral neurons and Schwann cells during Wallerian degeneration. Exocytosis is involved in demyelination and axonal degradation, which facilitate nerve regeneration following nerve degeneration. At this time, released ATP may affect the communication between cells in peripheral nerves. In this review, our description of the relationship between lysosomal exocytosis and Wallerian degeneration has implications for the understanding of peripheral nerve degenerative diseases and peripheral neuropathies, such as Charcot-Marie-Tooth disease or Guillain-Barré syndrome.

2014Jul
Pak J Med Sci
Pak J Med Sci 2014 Jul;30(4):914-6
Petrick Periyasamy, Department of Medicine, Universiti Kebangsaan Malaysia Medical Centre, Jalan Yaacob Latif, Bandar Tun Razak, 56000 Kuala Lumpur, Malaysia.

Fungaemia due to Paecilomyces lilacinus is generally not considered in AIDS patients because this condition is not categorised as an AIDS-indicator illness. We report a case of a 25-year-old lady who presented to our hospital with Guillain-Barré Syndrome, with the subsequent development of refractory fungaemia, multi-organ failure and disseminated intravascular coagulopathy. Amphotericin B was given as empirical antifungal therapy.Read More

HIV screening was reactive and Paecilomyces lilacinus was isolated from her blood. The fungaemia did not resolve after one week of amphotericin B treatment. The addition of itraconazole was also unsuccessful in clearing the fungaemia. Accurate mycological diagnosis is important in the care of AIDS patients with fungaemia because of the risk of treatment failure with empirical therapy.

2014Aug
J. Neurol. Neurosurg. Psychiatr.
J Neurol Neurosurg Psychiatry 2014 Aug 5. Epub 2014 Aug 5.
Departments of Medicine and Physiology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore.
2014Aug
Acta Clin Belg
Acta Clin Belg 2014 Aug 4:1551214Z00000000085. Epub 2014 Aug 4.

We report the case of a 62-year-old man who developed Guillain-Barré syndrome (GBS) following Hantavirus infection. Only three similar cases have been described in the literature so far. GBS is an autoimmune disease characterized by progressive symmetrical weakness of lower limbs extending to upper limbs and face and low or absent tendon reflexes.Read More

Prompt diagnosis is mandatory as GBS is a potentially life threatening disorder and needs timely treatment to ensure fast recovery and fewer complications.

2014Aug
Rinsho Shinkeigaku
Rinsho Shinkeigaku 2014 ;54(7):577-80
Department of Neurology, Brain Attack Center Ota Memorial Hospital.

Recurrence of Guillain-Barré syndrome (GBS) and Fisher syndrome (FS) is uncommon. We retrospectively studied the cases of 93 consecutive patients with GBS and FS who were admitted to our hospital between January 2000 and March 2013. We analyzed the clinical features of and anti- glycolipid antibodies in patients who experienced recurrence.Read More

Of the 93 patients, 53, 37, and 3 had GBS, FS, and overlapping GBS and FS, respectively. There were 6 recurrences in 4 patients, all of whom were women; their onset age ranged from 26 to 51 years, and the average time to recurrence ranged from 9 months to 25 years. The recurrence rate of FS was 10.8%. On the recurrence, 2 patients showed FS (5.4%) and 2 patients showed overlap of GBS and FS (5.4%). All patients with recurrence showed good prognosis and increased anti-GQ1b glycolipid antibody levels both at the initial episode and at recurrence. Immunological examinations, including those for detecting changes in anti-glycolipid antibodies, are important for clarifying the pathomechanism of recurrence in GBS and FS.

2014Aug
PLoS ONE
PLoS One 2014 1;9(8):e104074. Epub 2014 Aug 1.
Neuroscience Center, Department of Neurology, the First Hospital of Jilin University, Jilin University, Changchun, China; Department of Neurobiology, Care Sciences and Society, Karolinska Institute, Stockholm, Sweden.

Ganglioside as a neurotrophic drug has been hitherto widely used in China, although Guillain-Barré syndrome (GBS) following intravenous ganglioside treatment was reported in Europe several decades ago. We identified 7 patients who developed GBS after intravenous use of gangliosides (ganglioside+ group) and compared their clinical data with those of 77 non-ganglioside-associated GBS patients (ganglioside- group) in 2013, aiming at gaining the distinct features of ganglioside-associated GBS. Although the mean age, protein levels in cerebrospinal fluid (CSF) and frequency of cranial nerve involvement were similar between the two groups, the Hughes Functional Grading Scale (HFGS) score and the Medical Research Council (MRC) sum score at nadir significantly differed (4.Read More

9±0.4 vs 3.6±1.0; 7.7±5.5 vs 36.9±14.5, both p<0.001), indicating a higher disease severity of ganglioside-associated GBS. A higher ratio of patients with ganglioside-associated GBS required mechanical ventilation (85.7% vs 15.6%, p<0.01). The short-term prognosis of ganglioside-associated GBS, as measured by the HFGS score and the MRC sum score at discharge, was poorer (4.3±0.5 vs 2.8±1.1; 17.3±12.9 vs 46.0±13.9, both p<0.001). All the patients in the ganglioside+ group presented an axonal form of GBS, namely acute motor axonal neuropathy (AMAN). When compared with the AMAN patients in the ganglioside- group, more severe functional deficits at nadir and poorer recovery after standard treatment were still prominent in ganglioside-associated GBS. Anti-GM1 and anti-GT1a antibodies were detectable in patients with AMAN while not in patients with the demyelinating subtype of GBS. The concentrations of these antibodies in patients with AMAN were insignificantly different between the ganglioside+ and ganglioside- groups. In sum, ganglioside-associated GBS may be a devastating side effect of intravenous use of gangliosides, which usually manifests a more severe clinical course and poorer outcome.