Guillain-Barre Syndrome Publications (7318)

Guillain-Barre Syndrome Publications

2014Jul
Vaccine
Vaccine 2014 Jul 18. Epub 2014 Jul 18.
Center for Health Research Northwest, Kaiser Permanente Northwest 3800 N. Interstate Avenue, Portland, OR 97227 United States. Electronic address: Allison.naleway@kpchr.org.

To assess risks for acute adverse events and pregnancy complications in pregnant women following monovalent 2009 H1N1 inactivated influenza (MIV) vaccination.
Within the Vaccine Safety Datalink, we compared rates of pre-specified medically attended events (MAE) occurring within 42 days of MIV vaccination to those occurring in matched cohorts that at the same gestational age were either unvaccinated or received seasonal trivalent inactivated influenza (TIV) vaccine. Using generalized estimating equation method, with a Poisson distribution and log link, we calculated adjusted incident rate ratios (AIRR).Read More


Among 9349 women receiving MIV in any trimester, only one MAE occurred 0-3 days following MIV, an allergic reaction. No cases of Guillain-Barré syndrome, Bell's palsy, or transverse myelitis occurred 1-42 days after MIV. Compared to women receiving TIV and to unvaccinated women, risks for acute MAEs were not increased following MIV for any outcome. Hyperemesis was the most common adverse event in the MIV, TIV, and unvaccinated groups, occurring at a rate of about 4% over a 42-day period in all groups. Over a 42-day window, among all groups, incident gestational diabetes occurred at a rate of 3% and thrombocytopenia occurred at a rate of approximately 0.3%. Among women receiving MIV during pregnancy, increased risks for these and other less common obstetric events were not detected.
In this large cohort of pregnant women no acute safety signals were identified within 6 weeks of receipt of MIV.

2014Jul
J. Peripher. Nerv. Syst.
J Peripher Nerv Syst 2014 Jul 7. Epub 2014 Jul 7.
The University of Queensland Centre for Clinical Research, Queensland, Australia.

Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are thought to be autoimmune diseases. There have been many attempts to find an HLA association with GBS and CIDP with little success. There have been studies of other plausible genes in GBS and CIDP and the role of these genes in GBS and CIDP and the data from these genetic studies is reviewed.Read More

Some of the genes that have been studied are immune related and some others have nervous system effects. The studies are limited by small numbers. Some of the genes show association with disease severity rather than disease susceptibility. The need for more detailed molecular studies of the role of HLA molecules and the need for modern genetic approaches to GBS and CIDP is explained.

2014Jul
Intern. Med.
Intern Med 2014 15;53(14):1569-73. Epub 2014 Jul 15.
Department of Medicine, Mito Kyodo General Hospital, University of Tsukuba, Japan.

A 67-year-old man was admitted due to weakness, coughing, shortness of breath and fever. He had decreased breath sounds in the left lung and muscle weakness in the lower and upper extremities. Chest imaging showed a mass in the left lung, and a biopsy revealed small cell lung cancer.Read More

The nerve conduction velocity was decreased, and anti-GM1 IgG antibodies were positive. The patient showed a temporary neurologic recovery following the administration of cancer chemotherapy, although he eventually died of progression of lung cancer. As a result of the almost simultaneous symptomatic development of lung cancer and Guillain-Barré syndrome, this case may be considered to involve a paraneoplastic neurologic syndrome.

2014Apr
Ann Indian Acad Neurol
Ann Indian Acad Neurol 2014 Apr;17(2):199-201
Department of Neurology, KLE University's Jawaharlal Nehru Medical College, Belgaum, Karnataka, India.

Areflexia is one of the cardinal clinical features for the diagnosis of Guillain Barré syndrome. However, some patients may have sluggish proximal muscle stretch reflexes. Presence of thumb reflex, a distal stretch muscle reflex has not been documented in Guillain Barré syndrome.Read More


We prospectively evaluated thumb reflex in Guillain Barré syndrome patients and age matched controls from April to September 2013.
There were 31 patients with Guillain Barré syndrome in whom thumb reflex could be elicited in all (24 brisk, 7 sluggish), whereas all the other muscle stretch reflexes were absent in 29 patients at presentation and the remaining two had sluggish biceps and quadriceps reflexes (P = 0.001). Serial examination revealed gradual diminution of the thumb reflex (P < 0.001). Rapid progression of weakness was associated with early loss of the thumb reflex.
Thumb reflex, a distal stretch reflex is preserved in the early phase of Guillain Barré syndrome.

2014Jul
J. Alzheimers Dis.
J Alzheimers Dis 2014 Jul 11. Epub 2014 Jul 11.
Departments of Medicine, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore Phsyiology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore, Singapore.

A few studies have reported the association of autoantibodies to GM1 or GQ1bα with Alzheimer's disease (AD) or vascular dementia. Here we investigated whether patients with AD or vascular dementia had high titers of the anti-ganglioside antibodies. Sera were obtained from patients with AD (n = 22), vascular dementia (n = 14), Guillain-Barré syndrome, and multifocal motor neuropathy as well as normal controls.Read More

Enzyme-linked immunosorbent assay showed titers of IgG and IgM anti-GM1, anti-GQ1bα, and anti-GT1aα antibodies did not differ among AD, vascular dementia, and normal controls, and being remarkably lower than those in Guilllain-Barré syndrome and multifocal motor neuropathy. The anti-ganglioside antibodies are not biological markers of AD.

2014Jul
Nat Rev Neurol
Nat Rev Neurol 2014 Jul 15. Epub 2014 Jul 15.
Department of Neurology, Erasmus MC University Medical Centre Rotterdam, PO Box 2040, 3000 CA Rotterdam, Netherlands.

Guillain-Barré syndrome (GBS) is a potentially life-threatening postinfectious disease characterized by rapidly progressive, symmetrical weakness of the extremities. About 25% of patients develop respiratory insufficiency and many show signs of autonomic dysfunction. Diagnosis can usually be made on clinical grounds, but lumbar puncture and electrophysiological studies can help to substantiate the diagnosis and to differentiate demyelinating from axonal subtypes of GBS.Read More

Molecular mimicry of pathogen-borne antigens, leading to generation of crossreactive antibodies that also target gangliosides, is part of the pathogenesis of GBS; the subtype and severity of the syndrome are partly determined by the nature of the antecedent infection and specificity of such antibodies. Intravenous immunoglobulin and plasma exchange are proven effective treatments but many patients have considerable residual deficits. Discrimination of patients with treatment-related fluctuations from those with acute-onset chronic inflammatory demyelinating polyneuropathy is important, as these conditions may require different treatments. Novel prognostic models can accurately predict outcome and the need for artificial ventilation, which could aid the selection of patients with a poor prognosis for more-individualized care. This Review summarizes the clinical features of and diagnostic criteria for GBS, and discusses its pathogenesis, treatment and prognosis.

2014Jul
Case Rep Oncol Med
Case Rep Oncol Med 2014 11;2014:712040. Epub 2014 Jun 11.
Department of Internal Medicine, Crittenton Hospital Medical Center, Wayne State University, Rochester, MI 48307, USA.

Sunitinib malate (Sutent, SU011248) is an oral multitargeted tyrosine kinase inhibitor (TKI) used for the treatment of metastatic renal cell carcinoma and imatinib (Gleevec)-resistant gastrointestinal stromal tumor (GIST) with few reported side effects including asthenia, myelosuppression, diarrhea, and mucositis. Scarce literature exists regarding the rare but often serious toxicities of sunitinib. Autoimmune and neurological side effects have been linked to sunitinib's inhibition of VEGF receptors with a corresponding increase in VEGF levels, which is associated with development of different neuropathies.Read More

We hereby report an interesting case of Guillain-Barré syndrome in a middle-aged patient with metastatic renal cell carcinoma following sunitinib treatment.

2014Jul
Handb Clin Neurol
Handb Clin Neurol 2014 ;123:619-45
American Type Culture Collection, Manassas, VA, USA.

Although influenza A and B viruses are primarily known as respiratory viruses and mainly infected only the upper respiratory tract in humans, patients with influenza often develop signs and symptoms that are not due to the respiratory system. Frequently individuals with influenza develop headaches, meningismus, and even seizures in addition to their typical respiratory symptoms. In the past decades, influenza viruses have also been associated with serious non-respiratory signs.Read More

The famous 1918 strain of influenza was associated with von Economo's encephalitis lethargica and postencephalitic parkinsonism. In the 1960s influenza virus infections in children were associated with Reye's syndrome characterized often by fatty non-inflammatory hepatic disease and an encephalopathy with marked non-inflammatory cerebral edema. Intermittently children with influenza develop focal myalgia and myositis. Guillain-Barré syndrome was epidemiologically associated with the 1978 killed influenza vaccine but not subsequent vaccines. Although occasional children with influenza have developed encephalopathy, from 2000 through 2004 there was an increase in the number of serious cases of acute necrotizing encephalopathy accompanying infection with the influenza A 2009 strain. The current H5N1 strain of bird influenza occasionally infects humans with a high mortality rate and some appear to have central nervous signs. This chapter explores what is known about these influenza neurologic associations.

2014Jul
Handb Clin Neurol
Handb Clin Neurol 2014 ;123:285-305
Department of Neurology, School of Medicine, Wayne State University, Detroit, MI, USA. Electronic address: atselis@med.wayne.edu.

Epstein-Barr virus (EBV) is a human herpesvirus that is highly prevalent in all human populations studied. The initial infection is either asymptomatic or a self-limited febrile illness, but occasionally may be more severe and cause neurologic and systemic disease. After resolution of the initial illness, the virus persists in a "latent" asymptomatic form, kept in check by the immune system.Read More

In the modern era of immune modulation and suppression (cancer chemotherapy, transplants, monoclonal antibody therapy, acquired immunodeficiency syndrome (AIDS)), EBV can become "reactivated," and result in a number of illnesses, some affecting the nervous system. The systemic and neurologic effects of EBV can be difficult to diagnose and manage. In part, this is due to the unusual mix of pathogenetic mechanisms of EBV, which infects and immortalizes B lymphocytes. Immune insufficiency can unleash a lymphoproliferative state. Treatment of EBV disease may require consideration of a neoplastic treatment paradigm, in addition to regular antiviral medications.

2014Jul
BMJ Case Rep
BMJ Case Rep 2014 11;2014. Epub 2014 Jul 11.
Department of Medicine and Geriatrics, Tuen Mun Hospital, Hong Kong, Hong Kong.
2014Jun
J. Neurol. Sci.
J Neurol Sci 2014 Jun 19. Epub 2014 Jun 19.
Departments of Medicine and Physiology, Yong Loo Lin School of Medicine, National University of Singapore, Singapore. Electronic address: mdcyuki@nus.edu.sg.

To study the clinical profile of Guillain-Barré syndrome (GBS) patients who died in 4 Asian countries in order to understand factors underlying any variation in mortality.
Retrospectively reviewed medical records of GBS patients who died in 7 hospitals from 4 Asian countries between 2001 and 2012. Baseline characteristics, timing and causes of death were recorded.Read More


A total of 16 out of 261 GBS patients died. The overall mortality rate was 6%, with a range of 0 to 13%. The leading causes of death were respiratory infections, followed by myocardial infarction. The median age of our patients was 77years. Half of the patients required mechanical ventilation and almost all had significant concomitant illnesses. A disproportionate number of patients in the Hong Kong cohort died (13%). Patients with advanced age, fewer antecedent respiratory infections and need for mechanical ventilation were at most risk. Most deaths occurred during the plateau phase of GBS and on the general ward after having initially received intensive care.
There is considerable variability in mortality of GBS among different Asian cohorts. Although the risks factors for mortality were similar to Western cohorts, the timing and site of death differed. This allows specific measures to be implemented to improve GBS care in countries with higher mortality.

2014Jun
Brain
Brain 2014 Jun 19. Epub 2014 Jun 19.
5 School of Medical Sciences, Department of Physiology, Faculty of Medicine, University of New South Wales, Sydney, Australia c.lin@unsw.edu.au.

Distinguishing patients with acute-onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathy prior to relapse is often challenging at the onset of their clinical presentation. In the present study, nerve excitability tests were used in conjunction with the clinical phenotype and disease staging, to differentiate between patients with acute-onset chronic inflammatory demyelinating polyneuropathy and patients with acute inflammatory demyelinating polyneuropathy at an early stage, with the aim to better guide treatment. Clinical assessment, staging and nerve excitability tests were undertaken on patients initially fulfilling the diagnostic criteria of acute inflammatory demyelinating polyneuropathy soon after symptom onset and their initial presentation.Read More

Patients were subsequently followed up for minimum of 12 months to determine if their clinical presentations were more consistent with acute-onset chronic inflammatory demyelinating polyneuropathy. Clinical severity as evaluated by Medical Research Council sum score and Hughes functional grading scale were not significantly different between the two cohorts. There was no difference between the time of onset of initial symptoms and nerve excitability test assessment between the two cohorts nor were there significant differences in conventional nerve conduction study parameters. However, nerve excitability test profiles obtained from patients with acute inflammatory demyelinating polyneuropathy demonstrated abnormalities in the recovery cycle of excitability, including significantly reduced superexcitability (P < 0.001) and prolonged relative refractory period (P < 0.01), without changes in threshold electrotonus. In contrast, in patients with acute-onset chronic inflammatory demyelinating polyneuropathy, a different pattern occurred with the recovery cycle shifted downward (increased superexcitability, P < 0.05; decreased subexcitability, P < 0.05) and increased threshold change in threshold electrotonus in both hyperpolarizing and depolarizing directions [depolarizing threshold electrotonus (90-100 ms) P < 0.005, hyperpolarizing threshold electrotonus (10-20 ms), P < 0.01, hyperpolarizing threshold electrotonus (90-100 ms), P < 0.05], perhaps suggesting early hyperpolarization. In addition, using excitability parameters superexcitability, subexcitability and hyperpolarizing threshold electrotonus (10-20 ms), the patients with acute inflammatory demyelinating polyneuropathy and acute-onset chronic inflammatory demyelinating polyneuropathy could be clearly separated into two non-overlapping groups. Studies of nerve excitability may be able to differentiate acute from acute-onset chronic inflammatory demyelinating polyneuropathy at an early stage. Characteristic nerve excitability parameter changes occur in early acute-onset chronic inflammatory demyelinating polyneuropathy, to match the clinical phenotype. Importantly, this pattern of change was strikingly different to that shown by patients with acute inflammatory demyelinating polyneuropathy, suggesting that nerve excitability techniques may be useful in distinguishing acute-onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathy at the initial stage.

2014Apr
J Neurosci Rural Pract
J Neurosci Rural Pract 2014 Apr;5(2):204-5
Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.
2014Jun
Eur. J. Clin. Microbiol. Infect. Dis.
Eur J Clin Microbiol Infect Dis 2014 Jun 26. Epub 2014 Jun 26.
Emerging Diseases and Immunobiology Research Group, Centre for Food and Waterborne Diseases (CFWD), International Centre for Diarrheal Diseases Research (ICDDR,B), GPO Box 128, Dhaka, 1000, Bangladesh, zislam@icddrb.org.

Campylobacter jejuni is the most important cause of antecedent infections leading to Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS). The objective of the present study was to define the genetic diversity, population structure, and potential role of poultry in the transmission of Campylobacter to humans in Bangladesh. We determined the population structure of C.Read More

jejuni isolated from poultry (n = 66) and patients with enteritis (n = 39) or GBS (n = 10). Lipooligosaccharide (LOS) typing showed that 50/66 (76 %) C. jejuni strains isolated from poultry could be assigned to one of five LOS locus classes (A-E). The distribution of neuropathy-associated LOS locus classes A, B, and C were 30/50 (60 %) among the typable strains isolated from poultry. The LOS locus classes A, B, and C were significantly associated with GBS and enteritis-related C. jejuni strains more than for the poultry strains [(31/38 (82 %) vs. 30/50 (60 %), p < 0.05]. Multilocus sequence typing (MLST) defined 15 sequence types (STs) and six clonal complexes (CCs) among poultry isolates, including one ST-3740 not previously documented. The most commonly identified type, ST-5 (13/66), in chicken was seen only once among human isolates (1/49) (p < 0.001). Amplified fragment length polymorphism (AFLP) revealed three major clusters (A, B, and C) among C. jejuni isolated from humans and poultry. There seems to be a lack of overlap between the major human and chicken clones, which suggests that there may be additional sources for campylobacteriosis other than poultry in Bangladesh.

2014Aug
J. Neurol. Sci.
J Neurol Sci 2014 Aug 6;343(1-2):221-3. Epub 2014 Jun 6.
Department of Biomedical Molecular Biology, Ghent University, 927 Technologiepark, 9052 Ghent, Belgium; VIB Inflammation Research Center, 927 Technologiepark, 9052 Ghent, Belgium.

Systematic review of the frequency and clinical pattern of Guillain-Barré syndrome (GBS) in Arab countries was initiated by a keyword search of PubMed, Medline and Embase and examination of references in all relevant papers. Seven articles were included from Iraq (n=1), Kuwait (n=1), Libya (n=2), and Saudi Arabia (n=3). The only incidence report from the Arab world, a 1987 study from Libya, gives an incidence of 1.Read More

7 per 100,000 person-years. Some studies reported that GBS was more common in males and in people in their twenties and thirties. Five studies showed that GBS occurred more frequently during the colder months, and antecedent infection was reported in 26-76% of cases. Most patients had an ascending pattern of weakness and almost all patients had lower limb weakness and reduced or absent reflexes. Facial weakness was the most common cranial nerve involvement. Only one study classified the GBS patients according to electrophysiological findings and reported that 68% of the patients had demyelination type and 15% axonal type. Protein level in cerebrospinal fluid is elevated in most of the GBS patients. Mortality rate from GBS was up to 8%. This systematic review shows an immense deficit in epidemiological data on GBS in Arab countries. The limited data show that clinical pattern, sex and age distribution, reported antecedent infection, and GBS subtypes are similar to those in Western countries. However, further well-designed epidemiological studies on GBS in the Arab world are needed.

2014Jun
Biochim. Biophys. Acta
Biochim Biophys Acta 2014 Jun 17. Epub 2014 Jun 17.
University of Athens Medical School, Athens, Greece; Thomas Jefferson University, Philadelphia, PA, USA. Electronic address: mdalakas@med.uoa.gr.

Autoimmune neuropathies occur when immunologic tolerance to myelin or axonal antigens is lost. Even though the triggering factors and the underling immunopathology have not been fully elucidated in all neuropathy subsets, immunological studies on the patients' nerves, transfer experiments with the patients' serum or intraneural injections, and molecular fingerprinting on circulating autoantibodies or autoreactive T cells, indicate that cellular and humoral factors, either independently or in concert with each other, play a fundamental role in their cause. The review is focused on the main subtypes of autoimmune neuropathies, mainly the Guillain-Barré syndrome(s), the Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), the Multifocal Motor Neuropathy (MMN), and the IgM anti-MAG-antibody mediated neuropathy.Read More

It addresses the factors associated with breaking tolerance, examines the T cell activation process including co-stimulatory molecules and key cytokines, and discusses the role of antibodies against peripheral nerve glycolipids or glycoproteins. Special attention is given to the newly identified proteins in the nodal, paranodal and juxtaparanodal regions as potential antigenic targets that could best explain conduction failure and rapid recovery. New biological agents against T cells, cytokines, B cells, transmigration and transduction molecules involved in their immunopathologic network, are discussed as future therapeutic options in difficult cases. This article is part of a Special Issue entitled: Neuromuscular Diseases: Pathology and Molecular Pathogenesis.

2014Jun
Iran J Child Neurol
Iran J Child Neurol 2014 ;8(2):70-2
1.Pediatrics growth disorders research center, 17 Shahrivar Hospital, School of Medicine, Guilan University of Medical Sciences, Rasht, Iran.

We report a rare case that revealed severe myalgia as the chief complaint that is not mentioned in the list of frequent symptoms of Guillain Barré. Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy (AIDP).Required features for diagnosis of GBS are progressive motor weakness of more than one limb and areflexia.Read More

We report an 11-yearold boy who was referred to the emergency department with complaints of generalized body pain and gate problem. It seems that if myalgias are the chief complaint and weakness is mentioned as a less important symptom, clinicians should consider GBS after ruling out other reasons for myalgia especially inflammatory myositis.

2014Jun
Neurol. Sci.
Neurol Sci 2014 Jun 20. Epub 2014 Jun 20.
Neuroscience Center, Department of Neurology, The First Hospital of Jilin University, Xinmin Street 71#, Changchun, 130021, China.
2014Jun
Neurologia
Neurologia 2014 Jun 11. Epub 2014 Jun 11.
Servicio de Neurología, Hospital Santa Bárbara, Soria, España. Electronic address: angelam.gutierrez@gmail.com.

Pain is a common symptom in patients with Guillain-Barre syndrome. Intensity is moderate to severe in most cases and pain may persist after resolution of the disease.
Identify the most appropriate analgesic therapy for pain management in patients with Guillain-Barre syndrome.Read More


Systematic review and selection of scientific articles on treatment of pain in Guillain-Barre syndrome patients, published between January 1985 and December 2012. We included only randomised, double-blind, controlled trials assessing the effectiveness of drugs for pain management in these patients.
Four articles met the inclusion criteria. One evaluated the use of gabapentin, another evaluated carbamazepine, a third compared gabapentin to carbamazepine, and the last evaluated use of methylprednisolone. Both carbamazepine and gabapentin were useful for pain management. Patients experienced lower-intensity pain with gabapentin treatment in the study comparing that drug to carbamazepine. Methylprednisolone was not shown to be effective for reducing pain. The published data did not permit completion of a meta-analysis.
There is no robust evidence at present that would point to a single treatment option for this disorder. Further clinical studies of larger patient samples and with a longer duration are needed to characterise types of pain for each patient and measure pain intensity in an objective way.

Many stroke and neuromuscular patients with paraplegia or severe hemiparesis cannot control trunk balance.
To support the pelvis/hip of paresis patients, a new pelvic/hip support system was developed bearing a convex pressing member placed over the post-trochanteric groove, a cutaneous landmark sited on the lateral portion of the gluteus maximus muscle and indicating the posterior aspect of the greater trochanter.
Preliminary study.Read More


Stance control differences in two paretic patients (Guillain-Barré syndrome and stroke sequelae) with or without post-trochanteric groove support were examined. The contact pressure on the post-trochanteric groove was examined in eight healthy volunteers using an impact force sensor. The pelvic-lumbar movement was also examined using three-dimensional motion analysis, and the gluteus muscles activity was evaluated using surface electromyography.
Without post-trochanteric groove support, total three-dimensional displacement of the sacral marker was longer in the paresis patients than in normal controls, while post-trochanteric groove support decreased this distance. Post-trochanteric groove support provided compression pressure on the post-trochanteric groove, and all subjects showed a more upright trunk position, providing more anterior pelvic tilting. Six of eight subjects showed increased lumbar lordosis. Five of eight subjects showed gluteus maximus and/or gluteus medius muscle activation.
The mechanisms of post-trochanteric groove support were suggested to be spino-pelvic coordination and gluteal muscle activation.
The post-trochanteric groove is a cutaneous landmark located behind the pelvis/hip joint. Applying pressure to the post-trochanteric groove from behind pushes the trunk to adopt a more upright position, leading to improved stance control. Underlining mechanisms appear to be spino-pelvic coordination and gluteal muscle activation.

2014Jul
Neurology
Neurology 2014 Jul 11;83(2):113-7. Epub 2014 Jun 11.
From the Departments of Molecular Diagnosis (S.S., M.S., K. Sogawa, T.K., M.I., M.B., T.I., K.N., K. Sato, K.M., F.N.) and Neurology (M.M., S.M., M.B., K. Shibuya, Y.S., S.K.), Graduate School of Medicine, Chiba University; and Department of Physics (Y.K.), School of Science, Kitasato University, Kanagawa, Japan.

Previous histochemical studies in the demyelinating form of Guillain-Barré syndrome (GBS), acute inflammatory demyelinating polyneuropathy (AIDP), have shown complement deposition on the surface of Schwann cells, and therefore unknown epitopes would be present on the outer surface of Schwann cells.
We used a proteomic-based approach to search for the target molecules of AIDP in the extracted proteins from schwannoma cells. Sera were obtained from 40 patients with GBS, 31 controls with inflammatory disease, and 46 normal controls.Read More


We found that patients with AIDP after cytomegalovirus (CMV) infection have serum autoantibodies against membrane-organizing extension spike protein (moesin), which is expressed in the Schwann cell processes at the nodes of Ranvier and is crucial for myelination. Of the 40 patients with GBS, 6 had recent CMV infection and 5 of them (83%) had high levels of serum immunoglobulin G antibodies against moesin. The anti-moesin antibodies were found in none of the control subjects with disease including 5 with CMV infection but no neuropathy, and only 2 (4%) of the 46 normal control subjects. Immunocytochemistry showed that moesin was stained at the distal tips of schwannoma cells by sera from the patients with CMV-related AIDP but not by sera from controls.
Moesin is a possible immunologic target molecule of pathogenic autoantibodies in patients with CMV-related AIDP.
This study provides Class II evidence that levels of serum anti-moesin antibodies accurately distinguishes CMV-related AIDP from non-CMV-related AIDP (sensitivity 83%, specificity 93%).

2014Jul
Neurology
Neurology 2014 Jul 11;83(2):118-24. Epub 2014 Jun 11.
From the Faculty of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Dokkyo Medical University (N.K., K.H.), Tochigi, Japan; Graduate School of Medicine (S.S., S.K.), Chiba University, Japan; National Neurosciences Institute (T.U.), Singapore; and National University Singapore (Y.-C.C., N.Y.), Singapore. mdcyuki@nus.edu.sg.

To comprehensively investigate the relationship between antibodies to single glycolipids and their complexes and Guillain-Barré syndrome subtypes and clinical features.
In acute sera from 199 patients with Guillain-Barré syndrome, immunoglobulin G (IgG) antibodies to glycolipids and ganglioside complexes were tested using ELISA against individual antigens from single glycolipids including gangliosides (LM1, GM1, GM1b, GD1a, GalNAc-GD1a, GD1b, GT1a, GT1b, GQ1b) and a neutral glycolipid, asialo-GM1 (GA1), and antigens from the combination of 2 different glycolipids. Based on serial nerve conduction studies, the electrodiagnoses were as follows: 69 demyelinating subtype, 85 axonal subtypes, and 45 unclassified.Read More


Significant associations were detected between acute motor axonal neuropathy subtype and IgG antibodies to GM1, GalNAc-GD1a, GA1, or LM1/GA1 complex. Reversible conduction failure was significantly associated with IgG antibodies to GM1, GalNAc-GD1a, GD1b, or complex of LM1/GA1. No significant association was demonstrated between acute inflammatory demyelinating polyneuropathy and any of the glycolipids or ganglioside complexes. Anti-ganglioside complex antibodies alone were detected in 7 patients (5 axonal subtype).
The current study demonstrates that antibodies to single glycolipids and ganglioside complexes are associated with acute motor axonal neuropathy or acute motor conduction block neuropathy but not acute inflammatory demyelinating polyneuropathy.
This study provides Class II evidence that antibodies to glycolipids are increased in patients with acute motor axonal neuropathy and acute motor conduction block neuropathy but not acute inflammatory demyelinating polyneuropathy.

Background: Some patients with Guillain-Barré syndrome (GBS) also have acquired demyelination of the central nervous system (CNS) (i.e. acquired demyelinating syndrome, ADS).Read More

Often, the overlap of GBS and ADS is overlooked. Therefore, we evaluated case reports of GBS/ADS overlap syndrome. Methods: We mainly performed website-based research based on articles in cases presented with GBS/ADS overlap syndrome. A total of 66 cases were included. Clinical and prognosis data were analyzed. Results: A total of 85% of patients with simultaneous or consecutive occurrence of GBS and ADS were identified within 4 weeks of the initial diagnosis. Transverse myelitis (TM) (32%) was the most common ADS found in GBS/ADS. Patients with Miller Fisher syndrome (MFS)/ADS overlap syndrome had greater female predominance, mean age, frequency of onset at the same time period, or within a short period, and percentage of sole involvement of the subtentorial region. The outcome was favorable based on functional status in 74% of patients. The sensory level (OR  =  0·182, 95% CI  =  0·055-0·598; P  =  0·005) was the best predictor of a poor outcome, while visual deficit (OR  =  4·667, 95% CI  =  1·187-18·352; P  =  0·027) predicted a favorable outcome. Conclusion: The ADS in GBS are diverse, CNS demyelinating may occur at any time, but early in the GBS course (and vice versa). MFS/ADS overlap syndromes is more common. The prognosis is generally good, but patients with sensory level deficit are likely to have a poor prognosis. The features of MFS/other CIS may better reflect involvement of the brainstem in MFS itself, rather than ADS in autoimmune peripheral neuropathies.

2014Feb
Surg Obes Relat Dis
Surg Obes Relat Dis 2014 Feb 26. Epub 2014 Feb 26.
Neurology Department, CHU de Pointe-à-Pitre, University Hospital of Pointe-à-Pitre, Guadeloupe, France. Electronic address: landais-anne@voila.fr.
2014Jun
Small GTPases
Small GTPases 2014 Jun 9;5. Epub 2014 Jun 9.
Research Service; Department of Veterans Affairs; Edward Hines Jr. VA Hospital; Hines, IL USA; Neuroscience Institute; Stritch School of Medicine; Loyola University Chicago; Maywood, IL USA; Department of Ophthalmology; Stritch School of Medicine; Loyola University Chicago; Maywood, IL USA.

Transendothelial migration of autoreactive leukocytes into peripheral nerves is an early pathological hallmark of acute inflammatory demyelinating polyneuropathy (AIDP), a North American and European variant of Guillain-Barré Syndrome. Whereas the clinical management of AIDP is currently limited to non-selective immune modulating therapies, recent experimental studies support selective targeting of leukocyte trafficking as a promising alternative therapeutic strategy. Here, using a combination of targeted siRNA knockdown and pharmacological inhibitors, we report a novel role of both Cdc42 and RalA GTPases in facilitating TNF-α mediated CCL2 trafficking and release from immortalized rat peripheral nerve microvascular endoneurial endothelial cells.Read More

These findings raise interest in Cdc42 and RalA GTPases as potential therapeutic targets for the management of autoimmune inflammatory peripheral nerve disease.

2014Jun
Scand. J. Immunol.
Scand J Immunol 2014 Jun 9. Epub 2014 Jun 9.
Department of Neurology, the First Hospital of Jilin University, Changchun, China; Department of Neurobiology, Care Sciences and Society, Karolinska Institute, Stockholm, Sweden.

Experimental autoimmune neuritis (EAN), an animal model of human Guillain-Barré syndrome, has long been considered as a T helper (Th) 1 cell-mediated autoimmune disorder. However, deficiency of IFN-γ, a signature Th1 cytokine, aggravated EAN, with features of elevated production of IL-17A, despite an alleviated systemic Th1 immune response. We hypothesized that Th17 cells and their cytokines might play a pathogenic role in EAN.Read More

To further clarify the roles of these Th and regulatory T cell (Treg) cytokines in the pathogenesis of EAN and their interrelationship, we investigated the expression of Th1/Th2/Th17/Treg cytokines in EAN in the present study. We found that the levels of Th17 cells and IL-17A in cauda equina (CE) infiltrating cells and splenic mononuclear cells (MNCs) as well as in serum paralleled the disease evolution, which increased progressively during the initiation stage and reached higher value at the peak of EAN. The same pattern was also noticed for the expression of IL-22. The diverse expression profiles of FoxP3, IL-17 receptors A and C were seen in CE infiltrating cells and splenic MNCs in EAN. These findings indicate a major pro-inflammatory role of Th17 cells and IL-17A in the pathogenesis of EAN. Therapeutic interventions may be focused upon inhibiting Th17 cells and their cytokines in the early phase of EAN, so as to delay and suppress clinical signs of the disease, which has relevance for future studies on pathogenesis and treatment of GBS in humans. This article is protected by copyright. All rights reserved.

2014Jun
Braz J Anesthesiol
Braz J Anesthesiol 2014 May-Jun;64(3):195-8. Epub 2013 Oct 11.
Department of Anesthesiology and Reanimation, School of Medicine, Dokuz Eylül University, Konak, Turkey. Electronic address: vhanci@gmail.com.

Guillain-Barré Syndrome is one of the most common causes of acute polyneuropathy in adults. Recently, the occurrence of Guillain-Barré Syndrome after major and minor surgical operations has been increasingly debated. In Guillain-Barré syndrome, syndrome of inappropriate antidiuretic hormone secretion and dysautonomy are generally observed after maximal motor deficit.Read More


A 44-year-old male patient underwent a laparoscopic cholecystectomy for acute cholecystitis. After the development of a severe headache, nausea, diplopia, and attacks of hypertension in the early postoperative period, a computer tomography of the brain was normal. Laboratory tests revealed hyponatremia linked to syndrome of inappropriate antidiuretic hormone secretion, the patient's fluids were restricted, and furosemide and 3% NaCl treatment was initiated. On the second day postoperative, the patient developed numbness moving upward from the hands and feet, loss of strength, difficulty swallowing and respiratory distress. Guillain-Barré syndrome was suspected, and the patient was moved to intensive care. Cerebrospinal fluid examination showed 320 mg/dL protein, and acute motor-sensorial axonal neuropathy was identified by electromyelography. Guillain-Barré syndrome was diagnosed, and intravenous immune globulin treatment (0.4 g/kg/day, 5 days) was initiated. After 10 days in the intensive care unit, at which the respiratory, hemodynamic, neurologic and laboratory results returned to normal, the patient was transferred to the neurology service.
Our case report indicates that although syndrome of inappropriate antidiuretic hormone secretion and autonomic dysfunction are rarely the initial characteristics of Guillain-Barré syndrome, the possibility of postoperative syndrome of inappropriate antidiuretic hormone secretion should be kept in mind. The presence of secondary hyponatremia in this type of clinical presentation may delay diagnosis.

2014Jun
J. Neurol. Neurosurg. Psychiatr.
J Neurol Neurosurg Psychiatry 2014 Jun 5. Epub 2014 Jun 5.
Department of Neuroscience and Imaging, University 'G. d'Annunzio', Chieti-Pescara, Chieti, Italy.