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1.
J Neuroinflammation ; 18(1): 251, 2021 Nov 01.
Article in English | MEDLINE | ID: mdl-34719386

ABSTRACT

BACKGROUND: Guillain-Barré syndrome (GBS) is an acute inflammatory neuropathy with a heterogeneous presentation. Although some evidences support the role of autoantibodies in its pathogenesis, the target antigens remain unknown in a substantial proportion of GBS patients. The objective of this study is to screen for autoantibodies targeting peripheral nerve components in Guillain-Barré syndrome. METHODS: Autoantibody screening was performed in serum samples from all GBS patients included in the International GBS Outcome study by 11 different Spanish centres. The screening included testing for anti-ganglioside antibodies, anti-nodo/paranodal antibodies, immunocytochemistry on neuroblastoma-derived human motor neurons and murine dorsal root ganglia (DRG) neurons, and immunohistochemistry on monkey peripheral nerve sections. We analysed the staining patterns of patients and controls. The prognostic value of anti-ganglioside antibodies was also analysed. RESULTS: None of the GBS patients (n = 100) reacted against the nodo/paranodal proteins tested, and 61 (61%) were positive for, at least, one anti-ganglioside antibody. GBS sera reacted strongly against DRG neurons more frequently than controls both with IgG (6% vs 0%; p = 0.03) and IgM (11% vs 2.2%; p = 0.02) immunodetection. No differences were observed in the proportion of patients reacting against neuroblastoma-derived human motor neurons. Reactivity against monkey nerve tissue was frequently detected both in patients and controls, but specific patterns were only detected in GBS patients: IgG from 13 (13%) patients reacted strongly against Schwann cells. Finally, we confirmed that IgG anti-GM1 antibodies are associated with poorer outcomes independently of other known prognostic factors. CONCLUSION: Our study confirms that (1) GBS patients display a heterogeneous repertoire of autoantibodies targeting nerve cells and structures; (2) gangliosides are the most frequent antigens in GBS patients and have a prognostic value; (3) further antigen-discovery experiments may elucidate other potential antigens in GBS.


Subject(s)
Autoantibodies/blood , Guillain-Barre Syndrome/blood , Guillain-Barre Syndrome/diagnosis , Aged , Animals , Cell Line, Tumor , Cohort Studies , Female , Ganglia, Spinal/metabolism , Ganglia, Spinal/pathology , Guillain-Barre Syndrome/epidemiology , Humans , Macaca , Male , Mass Screening/methods , Middle Aged , Prospective Studies , Rats , Spain/epidemiology
2.
Neurol Res ; 44(2): 121-127, 2022 Feb.
Article in English | MEDLINE | ID: mdl-34382919

ABSTRACT

OBJECTIVE: This study aimed to explore the correlation of elevated glucose levels in the blood and cerebrospinal fluid with the progression and short-term prognosis of Guillain-Barré syndrome (GBS). METHODS: The medical records of 982 patients who were diagnosed with GBS in 31 representative tertiary hospitals, located in 14 provinces in southern China, were collected and retrospectively reviewed. Patients were grouped according to the levels of fasting plasma glucose (FPG) and cerebrospinal fluid (CSF) glucose, as well as the concentration of blood hemoglobinAlc (HbA1c). The Hughes grade scale was used to quantify functional outcomes. RESULTS: Compared to patients with normal FPG and CSF glucose levels, those in the high FPG and high CSF glucose groups were characterized by a higher proportion of severe patients (HFGS ≥ 3) at admission (58.8 vs. 73.1, P = 0.000; 57.6 vs. 71.2, P = 0.000), at nadir (67.4 vs. 83.0, P = 0.000; 66.2 vs. 80.4, P = 0.000), and at discharge (29.8 vs. 46.3, P = 0.000; 26.4 vs. 45.0, P = 0.000). Patients in the high HbA1c group also had more severe disability at admission (74.6 vs. 56.1, P = 0.005) and at nadir (80.3 vs. 64.3, P = 0.012) compared to the normal HbA1c group. Moreover, elevated levels of FPG and CSF glucose were significantly correlated with more severe disability at admission, at nadir, and at discharge. CONCLUSIONS: The present study showed that elevated glucose levels in the blood and cerebrospinal fluid were associated with the severity and short-term prognosis of GBS. TRIAL REGISTRATION: chicTR-RRc-17,014,152. ABBREVIATIONS: GBS, Guillain-Barré syndrome; FPG, fasting plasma glucose; CSF, cerebrospinal fluid; HFGS, Hughes Functional Grading Scale; HbA1c, hemoglobin A1c. DM, diabetes mellitus; NCS, nerve conduction study; AIDP, acute inflammatory demyelinating polyneuropathy; AMAN, acute motor axonal neuropathy; AMSAN, acute motor sensory axonal neuropathy; MV, mechanical ventilation.


Subject(s)
Disease Progression , Glucose/metabolism , Guillain-Barre Syndrome , Adult , Blood Glucose , Female , Glucose/cerebrospinal fluid , Guillain-Barre Syndrome/blood , Guillain-Barre Syndrome/cerebrospinal fluid , Guillain-Barre Syndrome/diagnosis , Guillain-Barre Syndrome/physiopathology , Humans , Male , Middle Aged , Patient Acuity , Prognosis , Retrospective Studies , Time Factors
3.
BMC Neurol ; 21(1): 275, 2021 Jul 12.
Article in English | MEDLINE | ID: mdl-34253174

ABSTRACT

BACKGROUND: Guillain-Barre syndrome (GBS) is an inflammatory polyradiculoneuropathy characterized by rapidly evolving weakness and areflexia, reaching nadir within 4 weeks. Data on the characteristic of GBS in Saudi Arabia are limited. This study aimed to describe the clinical, electrophysiological, and laboratory characteristics and outcome of a multicenter cohort of patients with GBS. METHODS: This is a retrospective multicenter nationwide study. Patients who had GBS, identified through Brighton Criteria, between January 2015 and December 2019 were included. Data collected included demographics, clinical features, cerebrospinal fluid profile, reported electrophysiological patterns, treatment, and outcome. Reported GBS subtypes were compared using chi-square, Fisher's exact, or Mann-Whitney U tests, as appropriate. RESULTS: A total of 156 patients with GBS were included (men, 61.5%), with a median age of 38 (interquartile range, 26.25-53.5) years. The most commonly reported antecedent illnesses were upper respiratory tract infection (39.1%) and diarrhea (27.8%). All but two patients (98.7%) had weakness, 64.1% had sensory symptoms, 43.1% had facial diplegia, 33.8% had oropharyngeal weakness, 12.4% had ophthalmoplegia, and 26.3% needed mechanical ventilation. Cytoalbuminological dissociation was observed in 69.1% of the patients. GBS-specific therapy was administered in 96.8% of the patients, of whom 88.1% had intravenous immunoglobulin, and 11.9% had plasmapheresis. Approximately half of the patients were able to walk independently within 9 months after discharge, and a third regained the ability to walk independently thereafter. Death of one patient was caused by septicemia. Acute inflammatory demyelinating polyradiculoneuropathy was the most commonly reported GBS subtype (37.7%), followed by acute motor axonal neuropathy (29.5%), and acute motor-sensory axonal neuropathy (19.2%). CONCLUSION: The clinical and laboratory characteristics and outcome of GBS in the Arab population of Saudi Arabia are similar to the international cohorts. The overall prognosis is favorable.


Subject(s)
Guillain-Barre Syndrome/diagnosis , Guillain-Barre Syndrome/therapy , Adolescent , Adult , Aged , Female , Guillain-Barre Syndrome/blood , Guillain-Barre Syndrome/epidemiology , Humans , Immunoglobulins, Intravenous/therapeutic use , Male , Middle Aged , Plasmapheresis/methods , Prognosis , Respiration, Artificial/methods , Retrospective Studies , Saudi Arabia/epidemiology , Treatment Outcome , Young Adult
4.
J Neuroimmunol ; 356: 577580, 2021 07 15.
Article in English | MEDLINE | ID: mdl-33933819

ABSTRACT

We analysed the effect of adding cholesterol to glycolipid antigens on antibody activity with enzyme-linked immunosorbent assay in 123 subjects consisting of 96 patients with Guillain-Barré syndrome, 25 Miller Fisher syndrome, and two Bickerstaff brainstem encephalitis. The use of cholesterol-added GM1 antigens increased anti-GM1 activity in 11 out of 23 anti-GM1-positive patients and resulted in six out of 100 anti-GM1-negative patients becoming anti-GM1-positive. Enhancement of anti-GM1 activity by cholesterol addition was significantly associated with antecedent gastrointestinal infection. The use of cholesterol-added glycolipid antigens can increase the detection rate of anti-glycolipid antibodies and accurately evaluate the anti-glycolipid antibody activity in vivo.


Subject(s)
Autoantibodies/blood , Cholesterol/administration & dosage , G(M1) Ganglioside/blood , Glycolipids/blood , Guillain-Barre Syndrome/blood , Miller Fisher Syndrome/blood , Encephalitis/blood , Encephalitis/drug therapy , Enzyme-Linked Immunosorbent Assay/methods , Female , Guillain-Barre Syndrome/drug therapy , Humans , Male , Miller Fisher Syndrome/drug therapy , Retrospective Studies
5.
J Neuroimmunol ; 355: 577572, 2021 06 15.
Article in English | MEDLINE | ID: mdl-33862418

ABSTRACT

INTRODUCTION: While children of all ages may be affected by Guillain-Barre-Syndrome (GBS), there are no reports of Dengue Fever (DF) as the preceding or concurrent infection in this age group. In addition, the presence of anti-GM1 IgM antibody, commonly seen in Multifocal Motor Neuropathy, is rarely encountered in both axonal and demyelinating variants of GBS. Moreover, only few neuromuscular ultrasound findings of the axonal variant in children were reported in the literature. CASE: Here we present a nine-year-old female who developed the classic signs, symptoms and neurophysiologic findings of axonal type of GBS during DF. She had elevated anti-GM1 IgM antibody atypical of this variant and diffusely enlarged nerves via neuromuscular ultrasound. CONCLUSION: In a pediatric patient with DF and acute flaccid paralysis, GBS should always be one of the considerations. Although rare, anti-ganglioside GM1 IgM antibody can still be found in axonal variant of GBS.


Subject(s)
Antibodies, Anti-Idiotypic/blood , Autoantibodies/blood , Axons/pathology , Dengue/blood , G(M1) Ganglioside/blood , Guillain-Barre Syndrome/blood , Child , Dengue/complications , Dengue/diagnostic imaging , Female , Guillain-Barre Syndrome/complications , Guillain-Barre Syndrome/diagnostic imaging , Humans
6.
J Peripher Nerv Syst ; 26(1): 83-89, 2021 Mar.
Article in English | MEDLINE | ID: mdl-33555098

ABSTRACT

Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in children. The objective of this study was to investigate the preceding infections, clinical, serological and electrophysiological characteristics and outcome of childhood GBS in Bangladesh. We included 174 patients with GBS aged <18 years from a prospective cohort in Bangladesh between 2010 and 2018. We performed multivariate logistic regression to determine the risk factors for poor outcome. Among 174 children with GBS, 74% (n = 129) were male. Around half of the patients (49%, n = 86) had severe muscle weakness, 65% (n = 113) were bedbound (GBS disability score 4) and 17% (n = 29) patients required mechanical ventilation at admission. Campylobacter jejuni serology and anti-GM1 IgG antibody were positive in 66% and 21% of the patients respectively. One hundred and forty-three (82%) patients did not receive standard treatment and half of them recovered fully or with minor deficits at 6-month. Twenty patients (11%) died throughout the study period. At 3-month of onset of weakness, complete recovery or recovery with minor deficit was significantly higher in demyelinating GBS patients compared to axonal GBS patients (86% vs 51%, P = .001). Cranial nerve palsy (OR = 4.00, 95%CI = 1.55-10.30, P = .004) and severe muscle weakness (OR = 0.16, 95%CI = 0.06-0.45, P = .001) were the important risk factors of poor outcome in children with GBS. Further large-scale studies are required for better understanding of factors associated with mortality and morbidity in childhood GBS.


Subject(s)
Antibodies, Bacterial/blood , Autoantibodies/blood , Cranial Nerve Diseases , Guillain-Barre Syndrome , Muscle Weakness , Adolescent , Bangladesh , Campylobacter jejuni/immunology , Child , Child, Preschool , Cranial Nerve Diseases/diagnosis , Cranial Nerve Diseases/etiology , Cranial Nerve Diseases/physiopathology , Female , Gangliosides/immunology , Guillain-Barre Syndrome/blood , Guillain-Barre Syndrome/complications , Guillain-Barre Syndrome/diagnosis , Guillain-Barre Syndrome/physiopathology , Humans , Infant , Male , Muscle Weakness/diagnosis , Muscle Weakness/etiology , Muscle Weakness/physiopathology , Prognosis , Prospective Studies , Risk Factors , Survival Analysis
7.
Neurol Neuroimmunol Neuroinflamm ; 8(2)2021 03.
Article in English | MEDLINE | ID: mdl-33547152

ABSTRACT

OBJECTIVE: To identify the clinical phenotypes and infectious triggers in the 2019 Peruvian Guillain-Barré syndrome (GBS) outbreak. METHODS: We prospectively collected clinical and neurophysiologic data of patients with GBS admitted to a tertiary hospital in Lima, Peru, between May and August 2019. Molecular, immunologic, and microbiological methods were used to identify causative infectious agents. Sera from 41 controls were compared with cases for antibodies to Campylobacter jejuni and gangliosides. Genomic analysis was performed on 4 C jejuni isolates. RESULTS: The 49 included patients had a median age of 44 years (interquartile range [IQR] 30-54 years), and 28 (57%) were male. Thirty-two (65%) had symptoms of a preceding infection: 24 (49%) diarrhea and 13 (27%) upper respiratory tract infection. The median time between infectious to neurologic symptoms was 3 days (IQR 2-9 days). Eighty percent had a pure motor form of GBS, 21 (43%) had the axonal electrophysiologic subtype, and 18% the demyelinating subtype. Evidence of recent C jejuni infection was found in 28/43 (65%). No evidence of recent arbovirus infection was found. Twenty-three cases vs 11 controls (OR 3.3, confidence interval [CI] 95% 1.2-9.2, p < 0.01) had IgM and/or IgA antibodies against C jejuni. Anti-GM1:phosphatidylserine and/or anti-GT1a:GM1 heteromeric complex antibodies were strongly positive in cases (92.9% sensitivity and 68.3% specificity). Genomic analysis showed that the C jejuni strains were closely related and had the Asn51 polymorphism at cstII gene. CONCLUSIONS: Our study indicates that the 2019 Peruvian GBS outbreak was associated with C jejuni infection and that the C jejuni strains linked to GBS circulate widely in different parts of the world.


Subject(s)
Campylobacter Infections/diagnosis , Campylobacter Infections/epidemiology , Campylobacter jejuni/isolation & purification , Disease Outbreaks , Guillain-Barre Syndrome/diagnosis , Guillain-Barre Syndrome/epidemiology , Adult , Campylobacter Infections/blood , Case-Control Studies , Female , Guillain-Barre Syndrome/blood , Humans , Male , Middle Aged , Peru/epidemiology
8.
J Neuroimmunol ; 353: 577504, 2021 04 15.
Article in English | MEDLINE | ID: mdl-33548620

ABSTRACT

PURPOSE: Up to 20-30% of patients with Guillain-Barré syndrome (GBS) suffer serious clinical manifestations such as respiratory failure. We aim to determine whether two new prognostic biomarkers, the neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR), could reliably predict respiratory failure in GBS.we MATERIALS AND METHODS: Data from 426 patients diagnosed at our center with GBS between January 2015 and July 2019 were retrospectively analyzed. Data were collected from the hospital database. Logistic regression and receiver operating characteristic curves were used to examine whether NLR alone, PLR alone or the combination, as measured at admission, could predict respiratory failure during hospitalization. Nomograms for predicting respiratory failure in GBS individuals were established, and predictive accuracy was evaluated using Harrell's concordance index (C-index). RESULTS: A total of 74 (17%) patients developed respiratory failure during hospitalization, and this was predicted independently by neutrophil count, NLR, PLR, and a combined "NLR-PLR" index, with the combined index performing best. The C-index of nomograms was 0.952 (95%CI 0.930-0.974) when NLR-PLR was included, or 0.933 (95%CI 0.911-0.955) when it was excluded. CONCLUSIONS: The prognostic biomarkers NLR and PLR may be independent predictors of respiratory failure in GBS. Combining the two indices may be more effective than either one on its own.


Subject(s)
Biomarkers/blood , Guillain-Barre Syndrome/complications , Lymphocyte Count , Respiratory Insufficiency/etiology , Adult , Female , Guillain-Barre Syndrome/blood , Guillain-Barre Syndrome/immunology , Humans , Male , Middle Aged , Neutrophils , Platelet Count , Retrospective Studies
9.
Ann Clin Transl Neurol ; 7(11): 2213-2220, 2020 11.
Article in English | MEDLINE | ID: mdl-33030817

ABSTRACT

OBJECTIVE: Neurofilament light chain (NfL) has been established as a biomarker of axonal damage in many diseases of the central nervous system (CNS). Increased levels of serum NfL (sNfL) can derive as well from damage in the peripheral nervous system (PNS) as from CNS, but little is known about the quantities contributing to sNfL. Peripheral nerve damage may be reflected by an increase in sNfL levels, while the NfL CSF/serum ratio and NfL index decreases. METHODS: We collected serum and cerebrospinal fluid (CSF) from 21 Guillain-Barré Syndrome (GBS) patients and measured NfL in serum and CSF and compared them with 19 neurologically healthy controls. RESULTS: In general, NfL in CSF and serum was significantly higher in GBS patients. Serum NfL was higher in GBS patients admitted to the intensive care unit (P = 0.02). Controls had a mean CSF/serum NfL ratio of 26.7 (ranging from 5.8 to 69.5) indicating a central origin of NfL. Three GBS patients had a similar range (23.9 to 42.7, mean 33.3) all of them with demyelinating pathology in the PNS. Eighteen GBS patients with axonal or mixed axonal-demyelinating pathology showed significantly lower CSF/serum ratios (0.02-12.2, mean 4.4), indicative of a peripheral origin of NfL. When applying the NfL index subdivisions remain the same. INTERPRETATION: These results demonstrate that the PNS is a relevant contributor to sNfL levels and that the distribution can be identified by a lowered NfL CSF/serum ratio of NfL index. Furthermore, acute or subacute polyneuropathies are likely confounding factors in interpreting sNfL levels in CNS diseases.


Subject(s)
Guillain-Barre Syndrome/blood , Guillain-Barre Syndrome/cerebrospinal fluid , Neurofilament Proteins/blood , Neurofilament Proteins/cerebrospinal fluid , Aged , Biomarkers/blood , Biomarkers/cerebrospinal fluid , Female , Guillain-Barre Syndrome/diagnosis , Humans , Male , Middle Aged , Retrospective Studies , Severity of Illness Index
11.
Eur Neurol ; 83(4): 415-420, 2020.
Article in English | MEDLINE | ID: mdl-32784293

ABSTRACT

The aim of the present study was to investigate the frequency and clinical features of Guillain-Barré syndrome (GBS) with hyperCKemia. We retrospectively identified 139 patients with GBS at 2 teaching hospitals in South Korea. We excluded patients with Miller-Fisher syndrome (n = 19), acute bulbar palsy (n = 3), and those whose serum creatine kinase (CK) levels were not measured (n = 45). Twelve of 72 patients (16.7%) had transient hyperCKemia, defined as serum CK ≥300 IU/L. The frequency of male sex and non-demyelinating electrodiagnostic features were higher in patients with hyperCKemia than those without. Transient hyperCKemia, occasionally seen in patients with GBS may be associated with the non-demyelinating subtype.


Subject(s)
Creatine Kinase/blood , Guillain-Barre Syndrome/blood , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Republic of Korea , Retrospective Studies , Young Adult
12.
Neurol Neuroimmunol Neuroinflamm ; 7(5)2020 09.
Article in English | MEDLINE | ID: mdl-32736337

ABSTRACT

OBJECTIVE: To determine whether IgG subclasses of antiparanodal autoantibodies are related to disease course and treatment response in acute- to subacute-onset neuropathies, we retrospectively screened 161 baseline serum/CSF samples and 66 follow-up serum/CSF samples. METHODS: We used ELISA and immunofluorescence assays to detect antiparanodal IgG and their subclasses and titers in serum/CSF of patients with Guillain-Barré syndrome (GBS), recurrent GBS (R-GBS), Miller-Fisher syndrome, and acute- to subacute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP). We evaluated clinical data retrospectively. RESULTS: We detected antiparanodal autoantibodies with a prevalence of 4.3% (7/161), more often in A-CIDP (4/23, 17.4%) compared with GBS (3/114, 2.6%). Longitudinal subclass analysis in the patients with GBS revealed IgG2/3 autoantibodies against Caspr-1 and against anti-contactin-1/Caspr-1, which disappeared at remission. At disease onset, patients with A-CIDP had IgG2/3 anti-Caspr-1 and anti-contactin-1/Caspr-1 or IgG4 anti-contactin-1 antibodies, IgG3 being associated with good response to IV immunoglobulins (IVIg). In the chronic phase of disease, IgG subclass of one patient with A-CIDP switched from IgG3 to IgG4. CONCLUSION: Our data (1) confirm and extend previous observations that antiparanodal IgG2/3 but not IgG4 antibodies can occur in acute-onset neuropathies manifesting as monophasic GBS, (2) suggest association of IgG3 to a favorable response to IVIg, and (3) lend support to the hypothesis that in some patients, an IgG subclass switch from IgG3 to IgG4 may be the correlate of a secondary progressive or relapsing course following a GBS-like onset.


Subject(s)
Autoantibodies , Cell Adhesion Molecules, Neuronal/immunology , Contactin 1/immunology , Guillain-Barre Syndrome , Immunoglobulin G , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating , Adult , Autoantibodies/blood , Autoantibodies/cerebrospinal fluid , Autoantibodies/immunology , Follow-Up Studies , Guillain-Barre Syndrome/blood , Guillain-Barre Syndrome/cerebrospinal fluid , Guillain-Barre Syndrome/immunology , Humans , Immunoglobulin G/blood , Immunoglobulin G/cerebrospinal fluid , Immunoglobulin G/immunology , Miller Fisher Syndrome/blood , Miller Fisher Syndrome/cerebrospinal fluid , Miller Fisher Syndrome/immunology , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/blood , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/cerebrospinal fluid , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/immunology , Retrospective Studies
13.
J Mol Neurosci ; 71(1): 137-141, 2021 Jan.
Article in English | MEDLINE | ID: mdl-32588399

ABSTRACT

Interleukin (IL)-34 is ligand for the colony-stimulating factor (CSF)-1 receptor. This cytokine has fundamental roles the pathogenesis of a number of autoimmune and neurologic disorders. However, its role in the pathogenesis of acute and chronic inflammatory demyelinating polyneuropathies (AIDP and CIDP) has not been assessed yet. We measured serum levels of IL-34 33 CIDP cases, 16 AIDP cases, and 33 control subjects using commercial ELISA kits. IL-34 levels were significantly higher in both AIDP (44.87 ± 4.38) and CIDP (44.87 ± 4.38) groups compared with healthy subjects (30.10 ± 1.05) (P = 0.046 and P = 0.01, respectively). Differences between female subgroups were insignificant. However, levels of this cytokine were significantly higher in male subjects with CIDP compared with male controls (P = 0.042). Thus, levels of this cytokine might be regarded as biomarkers for these kinds of autoimmune disorders. Future studies are needed to verify these results and find the molecular mechanism of participation of IL-34 in the pathogenesis of AIDP/CIDP.


Subject(s)
Guillain-Barre Syndrome/blood , Interleukins/blood , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/blood , Biomarkers , Case-Control Studies , Female , Guillain-Barre Syndrome/immunology , Humans , Interleukins/physiology , Male , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/immunology , Sex Characteristics
14.
BMC Neurol ; 20(1): 214, 2020 May 27.
Article in English | MEDLINE | ID: mdl-32460711

ABSTRACT

BACKGROUND: It is not well defined whether Guillain-Barré syndrome (GBS) patients with elevated serum creatine kinase (CK) levels have characteristic clinical features and are related to the subgroups of GBS. METHODS: We retrospectively studied 51 consecutive patients with GBS, who visited our hospital, and compared clinical, laboratory and electrophysiological findings between patients with and without elevated CK levels. RESULTS: Of 51 patients, 14 patients (27%) showed an elevation of serum CK levels. When compared with patients with the normal CK levels, the ratios of male, antecedent infections, and anti-GM1 antibody positivity were significantly higher in patients with elevated CK levels. The ratios of hypoesthesia, cranial nerve involvement, and urinary retention were significantly less in patients with elevated CK levels. There were no significant differences in disability at peak between two groups. In the electrophysiological examination, sensory nerve abnormalities were not observed. Although some patients with elevated CK levels showed prolongation of distal motor latencies (DMLs) and increase of durations in the initial examination, development of the prolongation of DMLs and increase of durations was not observed in the follow-up examinations. The findings were consistent with acute motor axonal neuropathy (AMAN) with reversible conduction failure (RCF) but not acute inflammatory demyelinating polyneuropathy (AIDP). CONCLUSIONS: The results suggest that the GBS patients with elevated CK levels represent not a group of AIDP but a group of AMAN with axonal degeneration or RCF even though the initial electrophysiological examination shows AIDP pattern.


Subject(s)
Creatine Kinase/blood , Guillain-Barre Syndrome , Female , Guillain-Barre Syndrome/blood , Guillain-Barre Syndrome/epidemiology , Guillain-Barre Syndrome/physiopathology , Humans , Male , Retrospective Studies
16.
Neurol Sci ; 41(6): 1351-1354, 2020 Jun.
Article in English | MEDLINE | ID: mdl-32399950

ABSTRACT

Guillain-Barré syndrome (GBS) is an acute polyradiculoneuropathy associated with dysimmune processes, often related to a previous infectious exposure. During Italian severe acute respiratory syndrome coronavirus-2 outbreak, a woman presented with a rapidly progressive flaccid paralysis with unilateral facial neuropathy after a few days of mild respiratory symptoms. Coronavirus was detected by nasopharyngeal swab, but there was no evidence of its presence in her cerebrospinal fluid, which confirmed the typical albumin-cytological dissociation of GBS, along with consistent neurophysiological data. Despite immunoglobulin infusions and intensive supportive care, her clinical picture worsened simultaneously both from the respiratory and neurological point of view, as if reflecting different aspects of the same systemic inflammatory response. Similar early complications have already been observed in patients with para-infectious GBS related to Zika virus, but pathological mechanisms have yet to be established.


Subject(s)
Betacoronavirus , Coronavirus Infections/complications , Coronavirus Infections/diagnosis , Guillain-Barre Syndrome/diagnosis , Guillain-Barre Syndrome/etiology , Hospitalization , Pneumonia, Viral/complications , Pneumonia, Viral/diagnosis , Aged , Betacoronavirus/isolation & purification , COVID-19 , Coronavirus Infections/blood , Female , Guillain-Barre Syndrome/blood , Humans , Italy , Pandemics , Pneumonia, Viral/blood , SARS-CoV-2
18.
J Neuroinflammation ; 17(1): 86, 2020 Mar 17.
Article in English | MEDLINE | ID: mdl-32183837

ABSTRACT

BACKGROUND: Guillain-Barré syndrome (GBS) is an autoimmune disease that results in demyelination and axonal damage. Five percent of patients die and 20% remain significantly disabled on recovery. Recovery is slow in most cases and eventual disability is difficult to predict, especially early in the disease. Blood or cerebrospinal fluid (CSF) biomarkers that could help identify patients at risk of poor outcome are required. We measured serum neurofilament light chain (sNfL) concentrations from blood taken upon admission and investigated a correlation between sNfL and clinical outcome. METHODS: Baseline sNfL levels in 27 GBS patients were compared with a control group of 22 patients with diagnoses not suggestive of any axonal damage. Clinical outcome parameters for GBS patients included (i) the Hughes Functional Score (HFS) at admission, nadir, and discharge; (ii) the number of days hospitalised; and (iii) whether intensive care was necessary. RESULTS: The median sNfL concentration in our GBS sample on admission was 85.5 pg/ml versus 9.1 pg/ml in controls. A twofold increase in sNfL concentration at baseline was associated with an HFS increase of 0.6 at nadir and reduced the likelihood of discharge with favourable outcome by a factor of almost three. Higher sNfL levels upon admission correlated well with hospitalisation time (rs = 0.69, p < 0.0001), during which transfer to intensive care occurred more frequently at an odds ratio of 2.4. Patients with baseline sNfL levels below 85.5 pg/ml had a 93% chance of being discharged with an unimpaired walking ability. CONCLUSIONS: sNfL levels measured at hospital admission correlated with clinical outcome in GBS patients. These results represent amounts of acute axonal damage and reflect mechanisms resulting in disability in GBS. Thus, sNfL may serve as a convenient blood-borne biomarker to personalise patient care by identifying those at higher risk of poor outcome.


Subject(s)
Biomarkers/blood , Guillain-Barre Syndrome/blood , Neurofilament Proteins/blood , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Prognosis , Recovery of Function , Retrospective Studies , Young Adult
19.
Medicine (Baltimore) ; 99(4): e18805, 2020 Jan.
Article in English | MEDLINE | ID: mdl-31977873

ABSTRACT

RATIONALE: Anti-GQ1b antibody syndrome refers to a distinct variant of Guillain- Barré syndrome. Involvement of the optic nerve in anti-GQ1b antibody syndrome is extremely rare. PATIENT CONCERNS: Here, we report a case of anti-GQ1b antibody syndrome presenting with visual deterioration as the initial symptom. A 73-year-old man presented with a 5-day history of bilateral blurred vision and ptosis. He had a previous history of diarrhea starting 10 days before admission. Physical examination showed visual deterioration, ophthalmoplegia, and peripheral facial paralysis. Testing of both serum and cerebrospinal fluid was positive for anti-GQ1b immunoglobulin G antibodies and negative for anti-aquaporin 4antibodies. DIAGNOSIS: Anti-GQ1b antibody syndrome. INTERVENTIONS: The patient was treated with intravenous methylprednisolone and human immunoglobulin. OUTCOMES: After a 20-day follow-up, the patient's condition took a favorable turn. LESSONS: This case reminds us that anti-GQ1b antibody syndrome should be suspected in patients with visual deterioration and preceding infection.


Subject(s)
Guillain-Barre Syndrome/diagnosis , Immunoglobulin G/blood , Aged , Autoantibodies/blood , Guillain-Barre Syndrome/blood , Guillain-Barre Syndrome/classification , Guillain-Barre Syndrome/drug therapy , Humans , Immunoglobulins, Intravenous/therapeutic use , Immunologic Factors/therapeutic use , Male , Ophthalmoplegia/diagnosis , Ophthalmoplegia/etiology , Syndrome , Vision Disorders/etiology
20.
Acta Neurol Belg ; 121(3): 661-668, 2021 Jun.
Article in English | MEDLINE | ID: mdl-31970702

ABSTRACT

Guillain-Barré syndrome (GBS) is an acute-onset, immune-mediated polyradiculoneuropathy, often precipitated by an antecedent infection. An association of GBS with vector-borne viral infections has been suggested, with evidence for the involvement of Zika, Dengue, Chikungunya and West Nile virus (WNV). This prospective case-control study was conducted to identify vector-borne viral infections in GBS. Thirteen individuals newly diagnosed as GBS were enrolled. Disease severity, prognostic factors and nerve conduction patterns were assessed. Eleven individuals with non-infectious conditions requiring cerebrospinal fluid (CSF) analysis were included as controls. Plasma, CSF and urine specimens were evaluated via nucleic acid amplification assays aimed to detect a broad spectrum of viruses. WNV and Toscana virus (TOSV) IgM/IgG antibodies were screened using commercial immunofluorescence assays and confirmed via virus neutralization tests (VNT). Partial TOSV nucleocapsid and genotype 1 polymerase sequences were detected in CSF of a patient with normal pressure hydrocephalus. Two control subjects had VNT-confirmed TOSV IgM in plasma. VNT-confirmed WNV and TOSV IgG were detected in 15.4% and 61.5% of GBS patients, respectively. Variations in WNV IgG and TOSV IgM detection rates were not statistically significant among study cohorts. However, TOSV IgG was significantly more frequent in GBS patients. No difference was observed for disease form or prognostic scores for virus markers. Follow-up serological profiles were identical to the initial findings. We have identified TOSV as a potential precipitating agent in GBS, with some rare clinical presentations of symptomatic TOSV infections.


Subject(s)
Guillain-Barre Syndrome/diagnosis , Sandfly fever Naples virus/isolation & purification , Adult , Aged , Antibodies, Viral/blood , Case-Control Studies , Female , Guillain-Barre Syndrome/blood , Guillain-Barre Syndrome/virology , Humans , Immunoglobulin G/blood , Male , Middle Aged , Prospective Studies , Severity of Illness Index , Young Adult
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