scholarly journals Heterogeneity of Polyneuropathy Associated with Anti-MAG Antibodies

2015 ◽  
Vol 2015 ◽  
pp. 1-9 ◽  
Author(s):  
Laurent Magy ◽  
Raphaël Kaboré ◽  
Stéphane Mathis ◽  
Prisca Lebeau ◽  
Karima Ghorab ◽  
...  
Keyword(s):  
Peripheral Nerves ◽  
Monoclonal Gammopathy ◽  
Demyelinating Neuropathy ◽  
Laboratory Findings ◽  
Electrodiagnostic Testing ◽  
Immune Mediated ◽  
Chronic Inflammatory Demyelinating Neuropathy ◽  
Myelin Associated Glycoprotein ◽  
Demyelinating Peripheral Neuropathy ◽  
Self Antigen

Polyneuropathy associated with IgM monoclonal gammopathy and anti-myelin associated glycoprotein (MAG) antibodies is an immune-mediated demyelinating neuropathy. The pathophysiology of this condition is likely to involve anti-MAG antibody deposition on myelin sheaths of the peripheral nerves and it is supposed to be distinct from chronic inflammatory demyelinating neuropathy (CIDP), another immune-mediated demyelinating peripheral neuropathy. In this series, we have retrospectively reviewed clinical and laboratory findings from 60 patients with polyneuropathy, IgM gammopathy, and anti-MAG antibodies. We found that the clinical picture in these patients is highly variable suggesting a direct link between the monoclonal gammopathy and the neuropathy. Conversely, one-third of patients had a CIDP-like phenotype on electrodiagnostic testing and this was correlated with a low titer of anti-MAG antibodies and the absence of widening of myelin lamellae. Our data suggest that polyneuropathy associated with anti-MAG antibodies is less homogeneous than previously said and that the pathophysiology of the condition is likely to be heterogeneous as well with the self-antigen being MAG in most of the patients but possibly being another component of myelin in the others.

scholarly journals Comparison of Monoclonal Gammopathies Linked to Poliovirus or Coxsackievirus vs. Other Infectious Pathogens

Cells ◽  
2021 ◽  
Vol 10 (2) ◽  
pp. 438
Author(s):  
Jean Harb ◽  
Nicolas Mennesson ◽  
Cassandra Lepetit ◽  
Maeva Fourny ◽  
Margaux Louvois ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
B Cell ◽  
Monoclonal Gammopathy ◽  
Coat Proteins ◽  
Chronic Stimulation ◽  
B Cell Epitopes ◽  
Monoclonal Immunoglobulins ◽  
Self Antigen ◽  
Coxsackievirus B1 ◽  
Undetermined Significance

Chronic stimulation by infectious pathogens or self-antigen glucosylsphingosine (GlcSph) can lead to monoclonal gammopathy of undetermined significance (MGUS) and multiple myeloma (MM). Novel assays such as the multiplex infectious antigen microarray (MIAA) and GlcSph assays, permit identification of targets for >60% purified monoclonal immunoglobulins (Igs). Searching for additional targets, we selected 28 purified monoclonal Igs whose antigen was not represented on the MIAA and GlcSph assays; their specificity of recognition was then analyzed using microarrays consisting of 3760 B-cell epitopes from 196 pathogens. The peptide sequences PALTAVETG and PALTAAETG of the VP1 coat proteins of human poliovirus 1/3 and coxsackievirus B1/B3, respectively, were specifically recognized by 6/28 monoclonal Igs. Re-analysis of patient cohorts showed that purified monoclonal Igs from 10/155 MGUS/SM (6.5%) and 3/147 MM (2.0%) bound to the PALTAVETG or PALTAAETG epitopes. Altogether, PALTAV/AETG-initiated MGUS are not rare and few seem to evolve toward myeloma.

scholarly journals An Unusual Case of “Conjugal” Polymyalgia Rheumatica after SARS-CoV-2 Vaccination

Rheumato ◽  
2021 ◽  
Vol 1 (1) ◽  
pp. 17-21
Author(s):  
Elena Vanni ◽  
Jacopo Ciaffi ◽  
Luana Mancarella ◽  
Francesco Ursini
Keyword(s):  
Environmental Factors ◽  
Polymyalgia Rheumatica ◽  
Temporal Correlation ◽  
Married Couples ◽  
Laboratory Findings ◽  
Immune Mediated ◽  
Asia Syndrome ◽  
Causative Effect ◽  
Genetic And Environmental Factors

The rare occurrence of polymyalgia rheumatica (PMR) in married couples has been reported in the literature. Susceptibility to PMR is contributed by genetic and environmental factors and cases of PMR developing after influenza vaccine have also been described, in a debated phenomenon known as ‘ASIA’ syndrome. We report the case of two cohabitating married patients developing PMR few weeks after the first dose of ChAdOx1-S SARS-CoV-2 vaccine. Both patients presented with typical symptoms suggestive of PMR. Laboratory findings and ultrasound examination confirmed the diagnosis. Glucocorticoid therapy led to rapid improvment of symptoms. Anti-receptor-binding domain IgG titre was tested and, eight weeks after vaccination, both patients showed no antibody response. It has been suggested that vaccines might trigger autoimmune or inflammatory states in predisposed individuals and various hypotheses have been made regarding the pathogenesis of PMR. Although the causative effect of vaccines cannot be determined, the close temporal correlation observed in our case supports the potential role of environmental factors in triggering the onset of PMR. However, the literature indicates that post-COVID19 vaccination immune-mediated or inflammatory adverse events are extremely rare and vaccination should be encouraged since the benefit largely outweighs possible risks.

scholarly journals Membranoproliferative Glomerulonephritis Type 1 Secondary to an Infected Ventriculoperitoneal Shunt: a Case Report

2014 ◽  
Vol 12 (2) ◽  
pp. 119-121
Author(s):  
Christos Paliouras ◽  
Foteini Lamprianou ◽  
Georgios Ntetskas ◽  
Georgios Mattas ◽  
Nikolaos Karvouniaris ◽  
...  
Keyword(s):  
Ventriculoperitoneal Shunt ◽  
Membranoproliferative Glomerulonephritis ◽  
Classical Pathway ◽  
Glomerular Injury ◽  
Laboratory Findings ◽  
Immune Mediated ◽  
Histologic Pattern ◽  
Life Threatening ◽  
Subsequent Activation

AbstractVentricular shunting is the usual method for treatment of congenital or acquired hydrocephalus. Immune-mediated glomerulonephritis (shunt nephritis) is a rare but life-threatening complication of this neurosurgical technique. Intraglomerular deposition of circulating immune complexes and the subsequent activation of the classical pathway of serum complement’s cascade result in glomerular inflammation. Membranoproliferative gomerulonephritis is the most common histologic pattern observed in renal biopsy. The diagnosis needs high suspicion and is based on clinical and laboratory findings. Deterioration of renal function in association with signs of infection and low levels of serum complement’s proteins C3 and C4 make the diagnosis possible. The prognosis is variable and depends on the time of diagnosis after the onset of glomerular injury. The optimal treatment includes timely removal of the infected shunt in combination with aggressive antibiotic therapy. In this paper we present the case of a membranoproliferative glomerulonephritis type 1 in a patient with a ventriculoperitoneal shunt. Although this type of shunting is considered safer than the ventriculoatrial one, the risk of complications such as an immune-mediated glomerulonephritis still exists.

Brachial Plexitis—Parsonage-Turner Syndrome

2018 ◽  
Author(s):  
Sandra Hearn
Keyword(s):  
Turner Syndrome ◽  
Impression Management ◽  
Peripheral Nerves ◽  
Surgical Intervention ◽  
Sudden Onset ◽  
Accurate Diagnosis ◽  
Advanced Imaging ◽  
Neuralgic Amyotrophy ◽  
Arm Pain ◽  
Immune Mediated

The chapter provides a case-based review of Parsonage-Turner syndrome. Parsonage-Turner syndrome, also known as neuralgic amyotrophy and idiopathic brachial plexitis, represents immune-mediated inflammation of neural structures within the brachial plexus and peripheral nerves of the upper limb. Classic features include sudden-onset shoulder or upper arm pain, followed by weakness in the forequarter or limb. This pattern, in addition to nonmechanical and nonradicular clinical features, should prompt consideration of Parsonage-Turner syndrome diagnosis. The diagnosis is established on clinical grounds, although electrodiagnostic data and advanced imaging can support the impression. Management is conservative. For neurosurgeons, accurate diagnosis of Parsonage-Turner syndrome can avoid unnecessary and inappropriate surgical intervention when symptoms are erroneously ascribed to another cause.

scholarly journals Presence of the myelin-associated glycoprotein correlates with alterations in the periodicity of peripheral myelin.

1982 ◽  
Vol 92 (3) ◽  
pp. 877-882 ◽  
Author(s):  
B D Trapp ◽  
R H Quarles
Keyword(s):  
Schwann Cell ◽  
Peripheral Nerves ◽  
Minor Component ◽  
Integral Membrane Protein ◽  
Structural Protein ◽  
Adult Rat ◽  
Pns Myelin ◽  
Myelin Associated Glycoprotein ◽  
A Minor ◽  
Periaxonal Space

The myelin-associated glycoprotein (MAG) is an integral membrane protein (congruent to 100,000 mol wt) which is a minor component of purified peripheral nervus system (PNS) myelin. In the present study, MAG was localized immunocytochemically in 1-micrometer thick Epon sections of 7-d and adult rat peripheral nerves, and its localization was compared to that of the major structural protein (Po) of PNS myelin. To determine more precisely the localization of MAG, immunostained areas in 1 micrometer sections were traced on electron micrographs of identical areas from adjacently cut thin sections.l MAG was localized in periaxonal membranes. Schmidt-Lantermann incisures, paranodal membranes, and the outer mesaxon of PNS myelin sheaths. Compact regions of PNS myelin did not react with MAG antiserum. The results demonstrate MAG's presence in "'semi-compact" Schwann cell or myelin membranes that have a gap of 12-14 nm between extracellular leaflets and a spacing of 5 nm or more between cytoplasmic leaflets. In compact regions of the myelin sheath which do not contain MAG, the cytoplasmic leaflets are "fused" and form the major dense line, whereas the extracellular leaflets are separated by a 2.0 nm gap appearing as paired minor dense lines. Thus, it is proposed that MAG plays a role in maintaining the periaxonal space, Schmidt-Lantermann incisures, paranodal myelin loops, and outer mesaxon by preventing "complete" compaction of Schwann cell and myelin membranes. The presence of MAG in these locations also suggests that MAG may serve a function in regulating myelination in the PNS.

High-dose intravenous immunoglobulins in the treatment of demyelinating neuropathy associated with monoclonal gammopathy

Neurology ◽  
1990 ◽  
Vol 40 (2) ◽  
pp. 212-212 ◽  
Author(s):  
D. Cook ◽  
M. Dalakas ◽  
A. Galdi ◽  
D. Biondi ◽  
H. Porter
Keyword(s):  
Monoclonal Gammopathy ◽  
Intravenous Immunoglobulins ◽  
High Dose ◽  
Demyelinating Neuropathy

scholarly journals Systemic Lupus Erythematosus Presenting as Thrombotic Thrombocytopenia Purpura: How Close Is Close Enough?

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Cesar A. Perez ◽  
Nabil Abdo ◽  
Anuj Shrestha ◽  
Edgardo S. Santos
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Laboratory Findings ◽  
Thrombocytopenic Purpura ◽  
Double Stranded Dna ◽  
Immune Mediated ◽  
Healthy Female ◽  
Life Threatening

Thrombotic thrombocytopenic purpura (TTP) is an uncommon life-threatening disease characterized by microangiopathic hemolytic anemia and thrombocytopenia, commonly associated with infections, malignancy, drugs, and autoimmune diseases. We report a case of 19-year-old previously healthy female that presents with anemia and thrombocytopenia diagnosed with thrombotic thrombocytopenic purpura that was treated successfully with plasmapheresis and corticosteroids. Laboratory findings also revealed antinuclear antibodies and antibodies to double-stranded DNA. Two weeks after presentation developed inflammatory arthritis, fulfilling diagnostic criteria for systemic lupus erythematosus (SLE). Prompt diagnosis and treatment with plasma exchange and corticosteroids should be instituted as soon as the diagnosis of TTP is suspected, even if other diagnoses, including lupus, are possible. When present, the coexistence of these two etiologies can have a higher mortality than either disease alone. An underlying diagnosis of SLE should be considered in all patients presenting TTP and the study of this association may provide a better understanding of their immune-mediated pathophysiology.

Sign in / Sign up

Export Citation Format

Share Document