scholarly journals Serum neurofilament light chain predicts long-term prognosis in Guillain-Barré syndrome patients

2020 ◽  
Vol 92 (1) ◽  
pp. 70-77
Author(s):  
Lorena Martín-Aguilar ◽  
Pol Camps-Renom ◽  
Cinta Lleixà ◽  
Elba Pascual-Goñi ◽  
Jordi Díaz-Manera ◽  
...  
Keyword(s):  
Single Molecule ◽  
Light Chain ◽  
Axonal Neuropathy ◽  
Guillain Barre Syndrome ◽  
Neurofilament Light Chain ◽  
Neurofilament Light ◽  
Fisher Syndrome ◽  
Baseline Serum ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

ObjectiveTo study baseline serum neurofilament light chain (sNfL) levels as a prognostic biomarker in Guillain-Barré syndrome (GBS).MethodsWe measured NfL in serum (98 samples) and cerebrospinal fluid (CSF) (24 samples) of patients with GBS prospectively included in the International GBS Outcome Study (IGOS) in Spain using single-molecule array (SiMoA) and compared them with 53 healthy controls (HCs). We performed multivariable regression to analyse the association between sNfL levels and functional outcome at 1 year.ResultsPatients with GBS had higher NfL levels than HC in serum (55.49 pg/mL vs 9.83 pg/mL, p<0.0001) and CSF (1308.5 pg/mL vs 440.24 pg/mL, p=0.034). Patients with preceding diarrhoea had higher sNfL than patients with respiratory symptoms or no preceding infection (134.90 pg/mL vs 47.86 pg/mL vs 38.02 pg/mL, p=0.016). sNfL levels correlated with Guillain-Barré Syndrome Disability Score and Inflammatory Rasch-built Overall Disability Scale (I-RODS) at every timepoint. Patients with pure motor variant and Miller Fisher syndrome showed higher sNfL levels than patients with sensorimotor GBS (162.18 pg/mL vs 95.50 pg/mL vs 38.02 pg/mL, p=0.025). Patients with acute motor axonal neuropathy cute motor axonal neuropathy had higher sNfL levels than other variants (190.55 pg/mL vs 46.79 pg/mL, p=0.013). sNfL returned to normal levels at 1 year. High baseline sNfL levels were associated with inability to run (OR=1.65, 95% CI 1.14 to 2.40, p=0.009) and lower I-RODS (β −2.60, 95% CI −4.66 to −0.54, p=0.014) at 1 year. Cut-off points predicting clinically relevant outcomes at 1 year with high specificity were calculated: inability to walk independently (>319 pg/mL), inability to run (>248 pg/mL) and ability to run (<34 pg/mL).ConclusionBaseline sNfL levels are increased in patients with GBS, are associated with disease severity and axonal variants and have an independent prognostic value in patients with GBS.

scholarly journals Serum neurofilament light chain predicts long-term prognosis in Guillain-Barré syndrome patients

2020 ◽  
Author(s):  
Lorena Martín-Aguilar ◽  
Pol Camps-Renom ◽  
Cinta Lleixà ◽  
Elba Pascual-Goñi ◽  
Jordi Diaz-Manera ◽  
...  
Keyword(s):  
Light Chain ◽  
High Specificity ◽  
Guillain Barre Syndrome ◽  
Neurofilament Light Chain ◽  
Neurofilament Light ◽  
Fisher Syndrome ◽  
Baseline Serum ◽  
Guillain Barré Syndrome ◽  
One Year ◽  
Guillain Barré

ABSTRACTObjectiveTo study baseline serum neurofilament light chain (sNfL) levels as a prognostic biomarker in Guillain-Barré syndrome (GBS).MethodsWe measured NfL using SiMoA in serum (98 samples) and CSF (24 samples) of GBS patients prospectively included in the International GBS Outcome Study (IGOS) in Spain and compared them with controls (HC). We performed multivariable regression to analyze the association between sNfL levels and functional outcome at one year.ResultsGBS patients had higher NfL levels than HC in serum (55.49pg/mL vs 9.13pg/mL, p<0,0001) and CSF (1308.5pg/mL vs 440.24pg/mL, p=0.034). Patients with preceding diarrhea had higher sNfL than patients with respiratory symptoms or no preceding infection (134.90pg/mL vs 47.86pg/mL vs 38.02pg/mL, p=0.016). sNfL levels correlated with GDS and R-ODS scales. Patients with pure motor variant and Miller- Fisher syndrome showed higher sNfL levels than patients with sensory-motor GBS (162.18pg/mL vs 95.50pg/mL vs 38.02pg/mL; p=0.025). AMAN patients had higher sNfL levels than other variants (190.55pg/mL vs 46.79pg/mL, p=0.013). sNfL returned to normal levels at one year. High baseline sNfL levels were associated with inability to run (OR=1.65, 95% CI 1.14-2.40, p=0.009) and lower R-ODS (β −2.60, 95% β −4.66-(−0.54), p=0.014) at one year. Cut-off points predicting clinically relevant outcomes at one year with high specificity were calculated: inability to walk independently (>319pg/mL), inability to run (>248pg/mL) and ability to run (<34pg/mL).ConclusionBaseline sNfL levels are increased in patients with GBS, they are associated with disease severity and axonal variants and they have an independent prognostic value in GBS patients.

scholarly journals Increased serum neurofilament light chain concentration indicates poor outcome in Guillain-Barré syndrome

2020 ◽  
Vol 17 (1) ◽  
Author(s):  
Patrick Altmann ◽  
Desiree De Simoni ◽  
Alexandra Kaider ◽  
Birgit Ludwig ◽  
Jakob Rath ◽  
...  
Keyword(s):  
Light Chain ◽  
Guillain Barre Syndrome ◽  
Neurofilament Light Chain ◽  
Neurofilament Light ◽  
Poor Outcome ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

scholarly journals Ratio and index of Neurofilament light chain indicate its origin in Guillain‐Barré Syndrome

2020 ◽  
Vol 7 (11) ◽  
pp. 2213-2220
Author(s):  
Peter Körtvelyessy ◽  
Jens Kuhle ◽  
Emrah Düzel ◽  
Stefan Vielhaber ◽  
Christian Schmidt ◽  
...  
Keyword(s):  
Light Chain ◽  
Guillain Barre Syndrome ◽  
Neurofilament Light Chain ◽  
Neurofilament Light ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

Acute Motor Axonal Neuropathy in a 5-Month-Old Child

2019 ◽  
Vol 18 (03) ◽  
pp. 171-174
Author(s):  
Federica Sullo ◽  
Milena Motta ◽  
Pierluigi Smilari ◽  
Luigi Rampello ◽  
Filippo Greco ◽  
...  
Keyword(s):  
Axonal Neuropathy ◽  
Male Infant ◽  
Guillain Barre Syndrome ◽  
Acute Motor Axonal Neuropathy ◽  
Fisher Syndrome ◽  
Acute Inflammatory Demyelinating Polyneuropathy ◽  
Guillain Barré Syndrome ◽  
Guillain Barré ◽  
Prior Infection ◽  
Prevalent Form

AbstractGuillain–Barré syndrome (GBS) is an acute inflammatory polyneuropathy characterized by rapidly progressive, essentially symmetric weakness and areflexia in a previously otherwise healthy child. It is the most common cause of acute flaccid paralysis in children, and its reported incidence is 1 to 2/100,000 population. Prior infection is a well-established predating event in GBS. The commonly recognized variants of GBS are acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy, and Miller–Fisher syndrome. AIDP is the most prevalent form. As Guillain–Barrè syndrome represents an important differential diagnosis in infancy with pronounced and progressive hypotonia, we herein report a case of AMAN in a 5-month-old male infant without known exposure to immunomodulating factors or infections.

The acute motor-sensory axonal neuropathy variant of Guillain-Barré syndrome after thoracic spine surgery

2011 ◽  
Vol 15 (6) ◽  
pp. 605-609 ◽  
Author(s):  
Jocelyn Cheng ◽  
D. Ethan Kahn ◽  
Michael Y. Wang
Keyword(s):  
Spine Surgery ◽  
Thoracic Spine ◽  
Axonal Neuropathy ◽  
Guillain Barre Syndrome ◽  
Acute Motor Axonal Neuropathy ◽  
Fisher Syndrome ◽  
Guillain Barré Syndrome ◽  
Classic Form ◽  
Guillain Barré ◽  
New Onset

Guillain-Barré syndrome (GBS) is the eponym used to describe acute inflammatory polyradiculoneuropathies, which manifest with weakness and diminished reflexes. Although the classic form of GBS is considered to be an ascending demyelinating polyneuropathy, several variants have been described in the literature, including the Miller-Fisher syndrome, acute panautonomic neuropathy, acute motor axonal neuropathy, and acute motor-sensory axonal neuropathy (AMSAN). Few cases of postoperative GBS have been documented, particularly for the AMSAN variant. The authors describe the case of a patient who developed AMSAN after thoracic spine surgery and highlight the importance of investigating new-onset weakness in the postoperative period.

Case 23

2018 ◽  
Author(s):  
Bashar Katirji
Keyword(s):  
Axonal Neuropathy ◽  
Recovery Phase ◽  
Clinical Findings ◽  
Guillain Barre Syndrome ◽  
Acute Motor Axonal Neuropathy ◽  
Fisher Syndrome ◽  
Immune Mediated ◽  
Guillain Barré Syndrome ◽  
Diagnostic Sensitivity And Specificity ◽  
Guillain Barré

Guillain-Barré syndrome is the prototype of acute immune-mediated neuropathies. Guillain-Barré syndrome has several subtypes including acute inflammatory demyelinating polyneuropathy, acute motor axonal neuropathy, and acute motor sensory axonal neuropathy. Guillain-Barré syndrome has also several variants including Miller Fisher syndrome, ataxic form, and pharyngeal–cervical–brachial form. This case highlights the clinical findings in Guillain-Barré syndrome and discusses in details the diagnostic criteria that are essential in confirming the diagnosis and excluding mimickers of the disorder. This is followed by a detailed discussion on the electrodiagnostic findings in Guillain-Barré syndrome during the acute presentation and recovery phase. The diagnostic sensitivity and specificity of the various findings seen on nerve conduction studies are included.

scholarly journals A Predictive Model for Guillain-Barré Syndrome Based on Single Learning Algorithms

2017 ◽  
Vol 2017 ◽  
pp. 1-9 ◽  
Author(s):  
Juana Canul-Reich ◽  
Juan Frausto-Solís ◽  
José Hernández-Torruco
Keyword(s):  
Predictive Model ◽  
Axonal Neuropathy ◽  
Classification Performance ◽  
Guillain Barre Syndrome ◽  
Acute Motor Axonal Neuropathy ◽  
Fisher Syndrome ◽  
Adequate Treatment ◽  
Guillain Barré Syndrome ◽  
Subtype Identification ◽  
Guillain Barré

Background. Guillain-Barré Syndrome (GBS) is a potentially fatal autoimmune neurological disorder. The severity varies among the four main subtypes, named as Acute Inflammatory Demyelinating Polyneuropathy (AIDP), Acute Motor Axonal Neuropathy (AMAN), Acute Motor Sensory Axonal Neuropathy (AMSAN), and Miller-Fisher Syndrome (MF). A proper subtype identification may help to promptly carry out adequate treatment in patients. Method. We perform experiments with 15 single classifiers in two scenarios: four subtypes’ classification and One versus All (OvA) classification. We used a dataset with the 16 relevant features identified in a previous phase. Performance evaluation is made by 10-fold cross validation (10-FCV). Typical classification performance measures are used. A statistical test is conducted in order to identify the top five classifiers for each case. Results. In four GBS subtypes’ classification, half of the classifiers investigated in this study obtained an average accuracy above 0.90. In OvA classification, the two subtypes with the largest number of instances resulted in the best classification results. Conclusions. This study represents a comprehensive effort on creating a predictive model for Guillain-Barré Syndrome subtypes. Also, the analysis performed in this work provides insight about the best single classifiers for each classification case.

scholarly journals A rare case of Miller Fisher variant of Guillain-Barré Syndrome (GBS) induced by a checkpoint inhibitor

2019 ◽  
Vol 12 (8) ◽  
pp. e229443 ◽  
Author(s):  
Caitlin Jane McNeill ◽  
Janev Fehmi ◽  
James Gladwin ◽  
Christopher Price
Keyword(s):  
Checkpoint Inhibitor ◽  
Checkpoint Inhibitors ◽  
Axonal Neuropathy ◽  
Sudden Onset ◽  
Guillain Barre Syndrome ◽  
High Dose ◽  
Fisher Syndrome ◽  
Guillain Barré Syndrome ◽  
Neurotoxic Effects ◽  
Guillain Barré

With the recent development of novel, more potent cancer treatment, in particular, immune ‘checkpoint inhibitors’, cases of neurological immune-related adverse events are on the rise. Although rare, this includes Guillain-Barré Syndrome (GBS). We present the case of a 68-year-old male who was admitted with sudden onset of worsening neurological symptoms following immunotherapy treatment. These symptoms progressed quickly to respiratory failure requiring intubation and admission to the intensive care unit. He was thoroughly investigated and is believed to have an axonal neuropathy in the form of Miller Fisher Syndrome (MFS) variant of GBS, secondary to immunotherapy treatment. He was initially treated with intravenous immunoglobulin, and later, perhaps more effectively, with high dose steroids which significantly improved his symptoms. This case of checkpoint inhibitor-induced MFS is one of few in the literature and is an important reminder of the potential for new immunotherapeutic agents to cause significant neurotoxic effects. These should be promptly and thoroughly investigated, in particular, as the management of these patients can differ from standard treatments used in these conditions.

scholarly journals Guillain–Barré Syndrome in COVID-19—The Potential Role of NCAM-1 and Immunotherapy

BioMed ◽  
2021 ◽  
Vol 1 (1) ◽  
pp. 80-92
Author(s):  
Krzysztof Laudanski ◽  
Aleksandra Yakhkind ◽  
Mariana Restrepo ◽  
Lindsay Draham ◽  
Adam Edward Lang
Keyword(s):  
Immune System ◽  
Guillain Barre Syndrome ◽  
System Response ◽  
Healthy Individuals ◽  
Neurofilament Light Chain ◽  
Neurofilament Light ◽  
Immune System Activation ◽  
Guillain Barré Syndrome ◽  
Acute Polyneuropathy ◽  
Guillain Barré

Coronavirus disease 2019 (COVID-19) interacts with the nervous system directly and indirectly by affecting the activation of the immune system. Guillain–Barré syndrome (GBS) is triggered by an inappropriate immune system activation that overlaps with the neurotoxic mechanism of an invading pathogen. Here, we discuss the complexity of an abnormal immune system response leading to the generation of autoimmunity in the setting of acute viral infection. A 67-year-old male patient with COVID-19 developed a sensory motor acute polyneuropathy with respiratory failure. Several serum inflammatory and neurodegeneration markers were collected during hospital days 1, 3, 8, and 67 and compared to healthy individuals. Neural cell adhesion molecule 1 (NCAM-1) and neurofilament light chain (NfL) values were highly variable when compared to healthy individuals, but not to the reference COVID-19 group. We focused our attention on NCAM-1 as a possible target for antibodies directed at COVID-19 in silico.

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