GBS Cases Research Articles

Stabbing Yourself in the Heart: A Case of Autoimmunity Gone Awry.

Within internal medicine, cardiac and neurologic pathology comprises a vast majority of patient complaints. Physicians and advanced-care practitioners must be highly educated and comfortable in the evaluation, diagnosis, and management of these entities. Chest pain accounts for millions of annual visits to the emergency room with pericarditis diagnosed in approximately four percent of patients with nonischemic chest pain. Guillain-Barre Syndrome is autoimmune polyneuropathy that often results in transient paralysis. Simultaneous diagnosis of both entities is a rare but described phenomenon. Here, we present a clinical case of GBS associated pericarditis. A fifty-five-year-old man with history of renal transplant presented with lower extremity weakness and urinary incontinence. Physical exam and diagnostic studies confirmed Guillain-Barre Syndrome. Patient subsequently developed stabbing chest pain with clinical presentation and electrocardiogram consistent with pericarditis. The patient was successfully treated for both diseases. This case highlights that although infrequent, internal medicine care providers must be cognizant of this correlation to ensure timely diagnosis and treatment.

Antibodies to Heteromeric Glycolipid Complexes in Guillain-Barré Syndrome

Autoantibodies are infrequently detected in the sera of patients with the demyelinating form of Guillain-Barré syndrome most commonly encountered in the Western world, despite abundant circumstantial evidence suggesting their existence. We hypothesised that antibody specificities reliant on the cis interactions of neighbouring membrane glycolipids could explain this discrepancy, and would not have been detected by traditional serological assays using highly purified preparations of single gangliosides. To assess the frequency of glycolipid complex antibodies in a Western European cohort of patients GBS we used a newly developed combinatorial glycoarray methodology to screen against large range of antigens (11 gangliosides, 8 other single glycolipids and 162 heterodimeric glycolipid complexes). Serum samples of 181 patients from a geographically defined, Western European cohort of GBS cases were analysed, along with 161 control sera. Serum IgG binding to single gangliosides was observed in 80.0% of axonal GBS cases, but in only 11.8% of cases with demyelinating electrophysiology. The inclusion of glycolipid complexes increased the positivity rate in demyelinating disease to 62.4%. There were 40 antigens with statistically significantly increased binding intensities in GBS as compared to healthy control sera. Of these, 7 complex antigens and 1 single ganglioside also produced statistically significantly increased binding intensities in GBS versus neurological disease controls. The detection of antibodies against specific complexes was associated with particular clinical features including disease severity, requirement for mechanical ventilation, and axonal electrophysiology. This study demonstrates that while antibodies against single gangliosides are often found in cases with axonal-type electrophysiology, antibodies against glycolipid complexes predominate in cases with demyelinating electrophysiology, providing a more robust serum biomarker than has ever been previously available for such cases. This work confirms the activation of the humoral immune system in the dysimmune disease process in GBS, and correlates patterns of antigen recognition with different clinical features.

Twelfth cranial nerve involvement in Guillian Barre syndrome

Guillian Barre Syndrome (GBS) is associated with cranial nerve involvement. Commonest cranial nerves involved were the facial and bulbar (IXth and Xth). Involvement of twelfth cranial nerve is rare in GBS. We present a case of GBS in a thirteen years old boy who developed severe tongue weakness and wasting at two weeks after the onset of GBS. The wasting and weakness of tongue improved at three months of follow up. Brief review of the literature about XIIth cranial nerve involvement in GBS is discussed.

Therapeutic plasma exchange in treatment of neuroimmunologic disorders: Review of 92 cases

Therapeutic plasma exchange (TPE) is a procedure that reduces amount of circulating antibodies in patients through filtration for the treatment of neurologic diseases in which autoimmunity plays a major role. We reviewed the medical records of 92 neurologic patients who had been consecutively treated by TPE between June 2000 and April 2011 at Ankara University School of Medicine, Neurology Department and The Apaheresis Center. Neurological indications included myastehia gravis (MG, 16 patients), Guillain–Barre syndrome (GBS, 37 patients) and miscellaneous diseases (39 patients). The median TPE session number was 5 with a range of 1–8; total number of TPE procedures in all cases was 454. All MG patients improved with TPE during their hospitalization time. Regarding GBS, nearly 67% of the patients improved early, during their hospitalization time, either. In our series, 25% of GBS cases died because of dysautonomia. TPE was not effective in the treatment of the patients with Lambert-Eaton myasthenic syndrome, paraneoplastic polineuropathy, toxic polineuropathy, mononeuropathy multiplex in the case series. During the TPE procedures, 4 patients had hypotension and total number of the procedures was 21 in those patients. One patient had urticaria in only one session of total 5 TPE procedures. Two patients had septicemia; the first one had 3 and the second had 5 TPE procedures; both septic cases died. In conclusion, TPE is an effective treatment in neurologic diseases that autoimmunity plays an important role in the pathogenesis.

Choriodecidual infection downregulates angiogenesis and morphogenesis pathways in fetal lungs from Macaca nemestrina.

BackgroundIntrauterine exposure to amniotic fluid (AF) cytokines is thought to predispose to bronchopulmonary dysplasia (BPD). We evaluated the effects of GBS exposure on RNA expression in fetal lung tissue to determine early molecular pathways associated with fetal lung injury that may progress to BPD.MethodsTen chronically catheterized pregnant monkeys (Macaca nemestrina) at 118–125 days gestation (term = 172 days) received choriodecidual inoculation of either: 1) Group B Streptococcus (n = 5) or 2) saline (n = 5). Cesarean section and fetal necropsy was performed in the first week after GBS or saline inoculation regardless of labor. RNA was extracted from fetal lungs and profiled by microarray. Results were analyzed using single gene, Gene Set, and Ingenuity Pathway Analysis. Validation was by RT-PCR and immunohistochemistry.ResultsDespite uterine quiescence in most cases, fetal lung injury occurred in four GBS cases (intra-alveolar neutrophils, interstitial thickening) and one control (peri-mortem hemorrhage). Significant elevations of AF cytokines (TNF-α, IL-8, IL-1β, IL-6) were detected in GBS versus controls (p<0.05). Lung injury was not directly caused by GBS, because GBS was undetectable by culture and PCR in the AF and fetal lungs. A total of 335 genes were differentially expressed greater than 1.5 fold (p<0.05) with GBS exposure associated with a striking upregulation of genes in innate and adaptive immunity and downregulation of pathways for angiogenesis, morphogenesis, and cellular growth and development.ConclusionsA transient choriodecidual infection may induce fetal lung injury with profound alterations in the genetic program of the fetal lung before signs of preterm labor. Our results provide a window for the first time into early molecular pathways disrupting fetal lung angiogenesis and morphogenesis before preterm labor occurs, which may set the stage for BPD. A strategy to prevent BPD should target the fetus in utero to attenuate alterations in the fetal lung genetic program.

Is guillain barré syndrome/fisher syndrome associated with swine ‘flu vaccination in children? results from a UK-wide epidemiological study

AimsTo report results after 13 months of prospective UK surveillance for Guillain-Barré/Fisher (GBS/FS) syndrome via the British Paediatric Surveillance Unit (BPSU) system.Methods‘Fast-track’ surveillance commenced in September 2009 using the BPSU...

Association of Human Herpesvirus 6 and 8 in Relapsing Remitting Multiple Sclerosis Type during Exacerbation

Multiple Sclerosis (MS) is a chronic demyelinating disease of the CNS with assumed autoimmune etiology. Human herpes viruses have probable effects on relapsing-remitting MS pathogenesis presumably through molecular mimicry and/or bystander mechanisms. In this study we probed the possible contribution of the two herpes viruses, human herpesvirus 6 (HHV-6) and human herpesvirus 8 (HHV-8), in clinically definite multiple sclerosis (CDMS) pa-tients-relapsing remitting type (RRMS) during clinical exacerbations. All patients had no history of immune modulating or suppressing drugs intake in the last 6 months. The peripheral blood samples, from CDMS patients (n = 20) (13F/7M, age (y) = 30.3 ± 3.21) and other immune mediated neurological disorders (OIND) (11F/9M, age = 25.2 ± 12.1), (My-asthenia Gravis, Guillain Barré Syndrome, ischemic stroke in adolescent and young adult with no clear risk factors), as a control group, had been enrolled within 15 months (January-2007-- March -2008). We investigated the existence of specific deoxyribonucleic acid (DNA) sequences belonging to HHV-6 and HHV-8, using polymerase chain reaction (PCR) in the isolated peripheral blood mononuclear cells (PBMCs) and in plasma. PCR demonstrated HHV-6 DNA in 7 cases (35%), HHV-8 sequences in only one cases (5%) in PBMCs from 20 relapsed CDMS patients; all HHV-6 posi-tive cases showed positive plasma results, while the blood samples from 20 OIND patients showed negative results ex-cept one case (5%) out of 9 cases of GBS was positive for HHV-8 in PBMCs. We consequently concluded that there is considerable evidence in this study that proposed the roles of HHV-6 and HHV-8 in MS pathogenesis and clinical ex-acerbation.

A Case of Guillain-Barre Syndrome (GBS) Associated with Peginterferon Alfa-2a and Weight Based Ribavirin for Chronic Hepatitis C (HCV)

Purpose: Almost all patients treated with Peginterferon and ribavirin experience one or more adverse events during the course of therapy. However, significant neurological side effects are rare. A 55 year old Caucasian male was referred to us for treatment-naive chronic HCV infection. The laboratory findings showed AST 60 and ALT 62. HCV RNA viral load was 1,620,000 IU/ml and it was a genotype la infection. Liver biopsy revealed stage 3 bridging fibrosis with mild macro vesicular steatosis and severe inflammation. Patient was subsequently started on Peginterferon alfa-2a 180 mcg/ml per week and Ribavirin 1000 mg/day in divided doses. He was tolerating the treatment fairly well with a more than 2 log drop in HCV RNA at week 8. At that time, the patient went to a local hospital with the complaint of numbness of the face, facial asymmetry, difficulty eating and loss of taste. A brain MRI and carotid ultrasound were normal. Patient was diagnosed with Bell's Palsy, started on oral steroids and interferon was held. Next week, the patient noted gradual onset of progressive weakness of bilateral lower extremities and facial diplegia. Patient also started having back pain and neuropathic pain in bilateral lower extremities. All these symptoms progressed over a course of two weeks and patient became wheelchair bound. Examination revealed decreased deep tendon reflexes (DTRs) in bilateral upper extremities, absent DTRs at the knees bilaterally and decreased at the ankles. Patient was found to have 4/5 motor strength in both lower extremities and bilateral weakness involving both upper and lower facial muscles. Patient underwent an electromyography study (EMG) and cerebrospinal fluid (CSF) analysis by lumbar puncture. The EMG revealed uniformly increased latency with uniformly decreased amplitude and decreased velocity across all nerves tested. The CSF showed markedly elevated protein levels (405.8 mg/dl, normal 15.0-45.0 mg/dl) with a normal glucose and 3 leukocytes/μl. Based upon these findings, the patient was diagnosed with acute inflammatory demyelinating polyneuropathy (GBS) and started on Intravenous Immunoglulin (IVIG). Thereafter, patient was transferred to rehab and started improving. The neuropathic pain has improved significantly. Patient is able to walk short distances with a walker. As per knowledge, this is the first case of typical GBS reported with chronic HCV therapy. We think it is important for clinicians to keep this association in mind when prescribing Peginterferon alfa-2a and Ribavirin for HCV treatment. Close attention should be paid to any neurological symptoms developing during the course of treatment. Prompt referral to a neurologist is warranted if these symptoms develop.

Prevalence and mechanism of bladder dysfunction in Guillain–Barré Syndrome

To examine the prevalence and mechanism of urinary dysfunction in GBS. Urinary symptoms were observed and neurological examinations made repeatedly during hospitalization of 65 consecutive patients with clinico-neurophysiologically definite GBS. The patients included 41 men, 24 women; mean age, 41 years old; mean Hughes motor grade, 3; AIDP, 28, AMAN, 37. Urodynamic studies consisted of uroflowmetry, measurement of post-micturition residuals, medium-fill water cystometry, and external anal sphincter electromyography. Urinary dysfunction was observed in 27.7% of GBS cases (urinary retention, 9.2%). Urinary dysfunction was related to the Hughes motor grade (P < 0.05), defecatory dysfunction (P < 0.05), age (P < 0.05), and negatively related to serum IgG class anti-ganglioside antibody GalNAc-GD1a (P < 0.05). Urinary dysfunction was more common in AIDP (39%) than in AMAN (19%). No association was found between antibody titer against neuronal nicotinic acetylcholine receptors and urinary dysfunction. Urodynamic studies in nine patients, mostly performed within 8 weeks after disease onset, revealed post-void residual in 3 (mean 195 ml), among those who were able to urinate; decreased bladder sensation in 1; detrusor overactivity in 8; low compliance in 1; underactive detrusor in 7 (both overactive and underactive detrusor in 5); and nonrelaxing sphincter in 2. In our series of GBS cases, 27.7% of the patients had urinary dysfunction, including urinary retention in 9.2%. Underactive detrusor, overactive detrusor, and to a lesser extent, hyperactive sphincter are the major urodynamic abnormalities. The underlying mechanisms of urinary dysfunction appear to involve both hypo- and hyperactive lumbosacral nerves. Neurourol. Urodynam. 28:432-437, 2009. (c) 2009 Wiley-Liss, Inc.

Improved Serological Diagnosis Stresses the Major Role of Campylobacter jejuni in Triggering Guillain-Barré Syndrome

Guillain-Barré syndrome (GBS) is a postinfectious autoimmune polyradiculoneuropathy. The most frequent antecedent pathogen is Campylobacter jejuni, followed by cytomegalovirus. However, more than 40% of GBS cases currently cannot be attributed to triggering events. This might be due to the shortcomings of the serological assays used for diagnosing infections, in particular for C. jejuni. In our study investigating 36 patients with acute GBS, standard serological methods identified the triggering viral or bacterial etiology in only 25% of cases. However, using a highly specific enzyme-linked immunosorbent assay based on two recombinant outer antigens encoded by C. jejuni genes Cj0017 (P39) and Cj0113 (P18), we found serological evidence of a preceding C. jejuni infection in 80.6% of the patients but in only 3.5% of the controls. We conclude that the role of C. jejuni in triggering GBS has been greatly underestimated.

Efficacy of repeated intravenous immunoglobulin in severe unresponsive Guillain-Barré syndrome

1 and 4 while the two others had demyelinative features. All four had raised cerebrospinal fluid protein concentrations upon admission and patient 4 had a 25 000 GM1 antibody titre. The patients were retreated with IVIg after 14–21 days and substantial or rapid improvement was noted within days (figure). All four patients began to walk within several weeks. Patient 1 is of special interest because he was in a critical condition for 15 days with flaccid quadriplegia, respiratory failure, and severe autonomic instability, with long asystoles necessitating a pacemaker. Electrophysiological studies at this phase showed inexcitable nerves in all his limbs. During the second course of IVIg he began to improve and to make a remarkable recovery. Unlike the patients reported by Irani et al no relapses occurred in all 12 patients after the onset of motor improvement. As the figure suggests, it appears that the second course of IVIg had a favourable impact on the clinical course of these patients. This favourable response is especially striking as two of the patients had features compatible with an adverse prognosis (severe axonal involvement in both and antecedent diarrhoea and high titres of GM1 antibodies in one). The outcome in these patients suggests that in some GBS cases the standard dose of IVIg is not sufficient and that in such cases it is justified to repeat the treatment. This is true even in cases with adverse prognostic features such as early axonal damage. Now that it has been determined that IVIg and plasma exchange are equally effective and that the combination of therapies offers no advantage, a next logical step might be a controlled study designed to determine optimum doses and regimens of current therapies in GBS.

Rare adverse events associated with oral poliovirus vaccine in Brazil.

Oral poliovirus vaccine (OPV) developed by A. Sabin has been effectively used to control poliomyelitis in Brazil, and the last case with the isolation of a wild poliovirus strain occurred in March 1989. Although the vaccine controlled the circulation of wild strains and poliomyelitis cases associated with these strains were not detected during the last eight years, rare cases classified as vaccine-associated paralytic poliomyelitis (VAPP) have been detected. Molecular characterization studies of poliovirus strains isolated from VAPP cases and from healthy contacts have confirmed that the isolates are derived from the Sabin vaccine strains and also detected genomic modifications known or suspected to increase neurovirulence such as mutations and recombination. The molecular characterization of polioviruses isolated during the last eight years from paralysis cases classified as Guillain-Barré (GBS) syndrome and transverse myelitis (TM), and from facial paralysis (FP) cases also confirmed the vaccine origin of the strains and demonstrated mutations known to increase neurovirulence. Analysis of the epidemiologic data of these GBS, TM and FP cases demonstrated that in most of them the last OPV dose was given months or years before the onset of the disease and the isolation of the polioviruses. The temporal association between the isolation of these strains and the GBS, TM and FP suggested that the Sabin vaccine-derived poliovirus strains could also rarely trigger the diseases.

Acute polyradiculitis (Guillain-Barré syndrome): an epidemiological study of 156 cases observed in Beijing.

One hundred fifty-six patients with acute polyradiculoneuritis (Guillain-Barré syndrome, GBS) were admitted to the Neurology Service of the Capital Hospital, Beijing, from 1949 through 1975. There were 105 male and 51 female patients ranging in age from 3 1/2 to 80 years, 118 (75.6%) being below 30 years. Sixty-eight patients were inhabitants of the urban district while 88 were from rural areas. Cases of GBS were observed throughout the year, but 107 patients (68.6%) had their disease onset from July through October. As judged from the seasonal and district predilections in this series, enteric infections may be an important predisposing factor for cases with onset during the summer and fall, particularly in the countryside.