A5060181895- Peripheral Neuropathies and Disorders
- Hereditary Neurological Disorders
- Nerve injury and regeneration
- Myasthenia Gravis and Thymoma
- Peripheral Nerve Disorders
- Monoclonal and Polyclonal Antibodies Research
- Pain Mechanisms and Treatments
- Long-Term Effects of COVID-19
- Advanced Neuroimaging Techniques and Applications
- Botulinum Toxin and Related Neurological Disorders
- Cerebral Palsy and Movement Disorders
- Axon Guidance and Neuronal Signaling
- Autoimmune Neurological Disorders and Treatments
- Toxin Mechanisms and Immunotoxins
- RNA Research and Splicing
- Glycosylation and Glycoproteins Research
- Cancer Treatment and Pharmacology
- Complement system in diseases
- Amyotrophic Lateral Sclerosis Research
- Viral Infections and Immunology Research
- Pain Management and Treatment
- Multiple Sclerosis Research Studies
- Spinal Dysraphism and Malformations
- T-cell and B-cell Immunology
- Neurogenetic and Muscular Disorders Research
The University of Texas Health Science Center at Houston
2016-2025
Erasmus MC
2021-2023
University of Michigan
2023
Aarhus University Hospital
2023
Chiba University Hospital
2022
Erasmus University Rotterdam
2021
University of Houston
2011-2019
Chiba University
2018
Neurology, Inc
2014-2015
The University of Texas at Austin
2013-2015
The acute motor axonal neuropathy (AMAN) form of the Guillain-Barre syndrome is a paralytic disorder abrupt onset characterized pathologically by nerve fiber degeneration variable severity and sparing sensory fibers. There little demyelination or lymphocytic inflammation. Most cases have antecedent infection with Campylobacter jejuni many antibodies directed toward GM1 ganglioside-like epitopes, but mechanism nerve-fiber injury has not been defined. In 7 fatal AMAN, immunocytochemistry...
Abstract The localization, mode of action, and roles complement in the Guillain‐Barré syndrome have been controversial. We used high‐resolution immunocytochemistry to localize activation products early stages acute inflammatory demyelinating polyneuropathy (AIDP) pattern syndrome. Three AIDP subjects who were autopsied had symptoms for 3 9 days at time death. Immunocytochemistry was performed on etched, epoxy resin‐embedded sections, next thin section compared by electron microscopy...
Gangliosides are a family of sialic acid-containing glycosphingolipids highly enriched in the mammalian nervous system. Although they major sialoglycoconjugates brain, their neurobiological functions remain poorly defined. By disrupting gene for key enzyme complex ganglioside biosynthesis (GM2/GD2 synthase; EC 2.4.1.92 ) we generated mice that express only simple gangliosides (GM3/GD3) and examined central peripheral systems. The knockout display decreased myelination, axonal degeneration...
Antibodies targeting major gangliosides that are broadly distributed in the nervous system sometimes associated with clinical symptoms imply selective nerve damage. For example, anti-GD1a antibodies acute motor axonal neuropathy (AMAN), a form of Guillain-Barré syndrome selectively affects nerves, despite reports GD1a is present human axons and myelin not expressed differentially versus sensory roots. We used series high-affinity monoclonal (mAbs) against GM1, GD1a, GD1b GT1b to test whether...
We investigated the possible mechanisms of paralysis and recovery in a patient with acute motor axonal neuropathy (AMAN) pattern Guillain-Barré syndrome. The AMAN GBS is characterized clinically by without sensory involvement electrodiagnostically low compound action potential amplitudes, suggesting damage, evidence demyelination. Many patients have serologic or culture recent Campylobacter jejuni infection. Pathologically, most severe cases are wallerian-like degeneration axons affecting...
Diffusion tensor imaging (DTI) and immunohistochemistry were used to examine axon injury in the rat spinal cord after unilateral L 2 –L 4 dorsal root axotomy at multiple time points (from 16 h 30 d surgery). Three days axotomy, DTI revealed a lesion ipsilateral column extending from lumbar cervical cord. The showed significantly reduced parallel diffusivity increased perpendicular day 3 compared with contralateral unlesioned column. These findings coincided loss of phosphorylated...
GM1 ganglioside has been implicated as a target of immune attack in some diseases the peripheral nervous system. Anti-GM1 antibodies are associated with certain acquired immune-mediated neuropathies. It is not clear how anti-GM1 cause nerve dysfunction and injury; however, sodium and/or potassium ion channel at node Ranvier implicated. To gain insight into pathogenesis these neuropathies, we examined distribution Gal(β1–3)GalNAc moieties fibres their relationship to voltage-gated (Kv1.1,...
Objective: This study was designed to determine if the presence of specific ganglioside-like moieties in Campylobacter lipopolysaccharides(LPSs) is related development Guillain-Barré syndrome (GBS), and discover how frequently such moieties, including GM1, are present these LPSs. Methods: We studied isolates sera from seven patients with GBS (five acute motor axonal neuropathy, one inflammatory demyelinating polyneuropathy, Fisher's syndrome), compared them similar specimens enteritis alone....
Anti-GD1a ganglioside antibodies (Abs) are the serological hallmark of acute motor axonal form post-infectious paralysis, Guillain-Barré syndrome. Development a disease model in mice has been impeded by weak immunogenicity gangliosides and apparent resistance GD1a-containing neural membranes to anti-GD1a antibody-mediated injury. Here we used with altered biosynthesis generate such at nerve terminals. First, bypassed immunological tolerance immunizing GD1a-deficient, β-1,4- N...
Electrodiagnostic features of demyelination are essential for establishing the diagnosis in demyelinating subtypes Guillain-Barré syndrome (GBS), but they may also occur disorders that mimic GBS clinically. Information about their frequency mimics is sparse.Evaluation electrodiagnostic from 38 patients with suspected whom was later refuted (GBS mimics). Their diagnostic accuracy analyzed by comparison nerve conduction studies (NCS) 73 confirmed patients.Disorders mimicked clinically at time...
To investigate CSF findings in relation to clinical and electrodiagnostic subtypes, severity, outcome of Guillain-Barré syndrome (GBS) based on 1,500 patients the International GBS Outcome Study.
ABSTRACT GM 1 - and GD 1a -like ganglioside mimicry in Campylobacter jejuni lipooligosaccharide (LOS) is considered to be involved the pathogenesis of -induced Guillain-Barré syndrome (GBS). Compared with gastroenteritis-related isolates, GBS-related C. isolates were strongly associated expression mimicry. The presence a few genes LOS mimicry, cst-II , cgtA cgtB was also strains. epitope may an important virulence phenotype risk developing GBS following campylobacter infection.
Recent studies have proposed that neurite outgrowth is influenced by specific nerve cell surface gangliosides, which are sialic acid-containing glycosphingolipids highly enriched in the mammalian nervous system. For example, endogenous lectin, myelin-associated glycoprotein (MAG), reported to bind axonal gangliosides (GD1a and GT1b) inhibit outgrowth. Clustering of absence inhibitors such as MAG also shown culture. In some human autoimmune PNS CNS disorders, autoantibodies against GD1a or...
<h3>Background and Objectives</h3> The clinical course outcome of the Guillain-Barré syndrome (GBS) are diverse vary among regions. modified Erasmus GBS Outcome Score (mEGOS), developed with data from Dutch patients, is a model that predicts risk walking inability in patients GBS. study objective was to validate mEGOS International Study (IGOS) cohort improve its performance region specificity. <h3>Methods</h3> We used prospective first 1,500 included IGOS, aged ≥6 years unable walk...
Abstract : Gangliosides, sialic acid‐bearing glycosphingolipids, are highly enriched in the vertebrate nervous system. Anti‐ganglioside antibodies associated with various human neuropathies, although pathogenicity of these remains unproven. Testing pathogenic role anti‐ganglioside will be facilitated by developing high‐affinity IgG‐class complement‐fixing monoclonal anti‐bodies against major brain gangliosides, a goal that has been difficult to achieve. In this study, mice lacking complex...
Axon calibre is crucial to efficient impulse transmission in the peripheral nervous system. Neurofilament numbers determine gross axonal diameter, but intra‐axonal distribution depends on phosphorylation status of neurofilament sidearms. Myelin‐associated glycoprotein (MAG) has been implicated signalling cascade controlling and hence control axon calibre. In an electron microscopic morphometric study we measured nearest neighbour distances (NNND) axons sural nerves from patients with...
ABSTRACT Introduction : We investigated the utility of diffusion tensor imaging (DTI) for detecting neuropathic changes in proximal nerve segments patients with peripheral neuropathy. Methods Twenty‐one individuals ( n = 11) and without 10) neuropathy underwent DTI a defined sciatic segment. Patients controls were evaluated by clinical examination conduction studies at baseline 6 months after initial scan. Results The mean fractional anisotropy (FA) value was significantly lower nerves from...
Guillain-Barré syndrome (GBS) is a postinfectious autoimmune neuropathy and anti-ganglioside antibodies (Abs) are strongly associated with this disorder. Several studies have implied that specific Abs induce in patients axonal forms of GBS. To study the mechanisms Abs-induced neuropathy, we established new passive transfer mouse model by L5 spinal nerve transection (L5SNT; modified Chung's model) systemic administration Abs. L5SNT causes degeneration small proportion fibers constitute...
Myasthenia gravis (MG) is an autoimmune, neuromuscular condition and patients with MG are vulnerable due to immunosuppressant use disease manifestations of dyspnea dysphagia during the coronavirus 2019 (COVID-19) pandemic.We conducted a retrospective cohort study using Optum® de-identified COVID-19 Electronic Health Record (EHR) dataset. Primary outcomes, such as hospitalization, ventilator use, intensive care unit (ICU) admission, death in MG, were compared those without MG: subgroups...