scholarly journals Demyelinating disease in patients with myasthenia gravis

2008 ◽  
Vol 66 (1) ◽  
pp. 5-7 ◽  
Author(s):  
Denis Bernardi Bichuetti ◽  
Tatiane Martins de Barros ◽  
Enedina Maria Lobato Oliveira ◽  
Marcelo Annes ◽  
Alberto Alain Gabbai
Keyword(s):  
Autoimmune Disease ◽  
Autoimmune Diseases ◽  
Myasthenia Gravis ◽  
Muscle Weakness ◽  
Neuromyelitis Optica ◽  
Neuromuscular Transmission ◽  
Demyelinating Disease ◽  
Demyelinating Diseases

Myasthenia gravis (MG) is an autoimmune disease characterized by fluctuating muscle weakness, caused by impaired neuromuscular transmission. Patients with MG can present other autoimmune diseases in association, commonly hypo or hyperthyroidism. The association of MG to demyelinating disease is rare and has been described before. We report on three Brazilian patients with MG that presented distinct demyelinating diseases, two monophasic and one recurrent neuromyelitis optica, several years after the diagnosis of MG, and discuss their clinical courses.

scholarly journals Multiple Sclerosis in Malaysia: Demographics, Clinical Features, and Neuroimaging Characteristics

2013 ◽  
Vol 2013 ◽  
pp. 1-10 ◽  
Author(s):  
S. Viswanathan ◽  
N. Rose ◽  
A. Masita ◽  
J. S. Dhaliwal ◽  
S. D. Puvanarajah ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Neuromyelitis Optica ◽  
Demyelinating Disease ◽  
Age At Onset ◽  
Positive Family History ◽  
Disease Onset ◽  
Demyelinating Diseases ◽  
Lesion Length ◽  
Relapsing Remitting ◽  
Spectrum Disorders

Background. Multiple sclerosis (MS) is an uncommon disease in multiracial Malaysia. Diagnosing patients with idiopathic inflammatory demyelinating diseases has been greatly aided by the evolution in diagnostic criterion, the identification of new biomarkers, and improved accessibility to neuroimaging in the country.Objectives. To investigate the spectrum of multiple sclerosis in Malaysia.Methods. Retrospective analysis with longitudinal follow-up of patients referred to a single tertiary medical center with neurology services in Malaysia.Results. Out of 245 patients with idiopathic inflammatory demyelinating disease, 104 patients had multiple sclerosis. Female to male ratio was 5 : 1. Mean age at onset was 28.6 ± 9.9 years. The Malays were the predominant racial group affected followed by the Chinese, Indians, and other indigenous groups. Subgroup analysis revealed more Chinese having neuromyelitis optica and its spectrum disorders rather than multiple sclerosis. Positive family history was reported in 5%. Optic neuritis and myelitis were the commonest presentations at onset of disease, and relapsing remitting course was the commonest disease pattern observed. Oligoclonal band positivity was 57.6%. At disease onset, 61.5% and 66.4% fulfilled the 2005 and 2010 McDonald’s criteria for dissemination in space. Mean cord lesion length was 1.86 ± 1.65 vertebral segments in the relapsing remitting group as opposed to 6.25 ± 5.18 vertebral segments in patients with neuromyelitis optica and its spectrum disorders.Conclusion. The spectrum of multiple sclerosis in Malaysia has changed over the years. Further advancement in diagnostic criteria will no doubt continue to contribute to the evolution of this disease here.

Clinical and laboratory features of neuromyelitis optica with oligoclonal IgG bands

2007 ◽  
Vol 13 (3) ◽  
pp. 332-335 ◽  
Author(s):  
M. Nakamura ◽  
I. Nakashima ◽  
S. Sato ◽  
I. Miyazawa ◽  
K. Fujihara ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Autoimmune Diseases ◽  
Myasthenia Gravis ◽  
Neuromyelitis Optica ◽  
Disease Duration ◽  
Sicca Syndrome ◽  
Common Features ◽  
Laboratory Features ◽  
Oligoclonal Igg ◽  
Oligoclonal Igg Bands

Of 23 neuromyelitis optica (NMO) cases, we found two cases with oligoclonal IgG bands (OBs). Both patients were positive for NMO-IgG. Their common features were long disease duration and co-existing autoimmune diseases (myasthenia gravis and sicca syndrome). Although OBs are mostly negative in NMO, which distinguishes it from multiple sclerosis (MS), they can be positive by long-standing autoimmunity, which may not be directly related to NMO. Multiple Sclerosis 2007; 13: 332-335. http://msj.sagepub.com

scholarly journals Functional monovalency amplifies the pathogenicity of anti-MuSK IgG4 in myasthenia gravis

2021 ◽  
Vol 118 (13) ◽  
pp. e2020635118
Author(s):  
Dana L. E. Vergoossen ◽  
Jaap J. Plomp ◽  
Christoph Gstöttner ◽  
Yvonne E. Fillié-Grijpma ◽  
Roy Augustinus ◽  
...  
Keyword(s):  
Stochastic Process ◽  
Autoimmune Disease ◽  
Myasthenia Gravis ◽  
Muscle Weakness ◽  
Binding Sites ◽  
Antiinflammatory Activity ◽  
Autoimmune Response ◽  
Cross Link ◽  
Muscle Specific Kinase ◽  
Opposing Effects

Human immunoglobulin (Ig) G4 usually displays antiinflammatory activity, and observations of IgG4 autoantibodies causing severe autoimmune disorders are therefore poorly understood. In blood, IgG4 naturally engages in a stochastic process termed “Fab-arm exchange” in which unrelated IgG4s exchange half-molecules continuously. The resulting IgG4 antibodies are composed of two different binding sites, thereby acquiring monovalent binding and inability to cross-link for each antigen recognized. Here, we demonstrate that this process amplifies autoantibody pathogenicity in a classic IgG4-mediated autoimmune disease: muscle-specific kinase (MuSK) myasthenia gravis. In mice, monovalent anti-MuSK IgG4s caused rapid and severe myasthenic muscle weakness, whereas the same antibodies in their parental bivalent form were less potent or did not induce a phenotype. Mechanistically this could be explained by opposing effects on MuSK signaling. Isotype switching to IgG4 in an autoimmune response thereby may be a critical step in the development of disease. Our study establishes functional monovalency as a pathogenic mechanism in IgG4-mediated autoimmune disease and potentially other disorders.

scholarly journals Invalidization in patients with myasthenia, factors affecting disability

2016 ◽  
Vol 19 (3) ◽  
pp. 129-132
Author(s):  
Darya Yu. Konkova ◽  
V. N Karnaukh
Keyword(s):  
Myasthenia Gravis ◽  
Muscle Weakness ◽  
Neuromuscular Transmission ◽  
Factors Affecting ◽  
Factors Influencing ◽  
Inadequate Therapy ◽  
Disability Group ◽  
Positive Effect

Myasthenia gravis, as a disease associated with impaired neuromuscular transmission and manifested by muscle weakness and fatigue, necessarily leads to the disability of patients. There were analyzed terms and the factors influencing upon disability in 52 patients with myasthenia, 67 per cent of whom had a disability group. The duration of the disease from the beginning to the referral to the examination in the Bureau of the Medical and Social Expertise was revealed to amount in average of 2.68 ± 0.44 years. Out of factors influencing upon more earlier disability there were noted primary or early generalization of the process, severe crisis course and pronounced severity according to MGFA, poor compensation on anticholinesterase drugs, inadequate therapy. There was noted the positive effect of thymectomy on the consequent course of the disease, only 2 out of the 18 operated patients showed its further progression.

scholarly journals Old Dog Encephalitis and Demyelinating Diseases in Man

1975 ◽  
Vol 12 (3) ◽  
pp. 220-226 ◽  
Author(s):  
J. M. Adams ◽  
W. J. Brown ◽  
H. D. Snow ◽  
S. D. Lincoln ◽  
A. W. Sears ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Neuromyelitis Optica ◽  
Plasma Cells ◽  
Demyelinating Disease ◽  
Subacute Sclerosing Panencephalitis ◽  
Fluorescent Antibody ◽  
Optic Tract ◽  
Demyelinating Diseases ◽  
Relationship Of ◽  
Perivascular Infiltration

Pathologic findings in mature dogs with old dog encephalitis were compared with the findings in multiple sclerosis, subacute sclerosing panencephalitis and neuromyelitis optica in man. Fluorescent antibody studies in animal and human tissues were compared. Optic neuritis in dogs with chronic distemper shows changes similar to those in the optic tract of human patients with severe demyelinating disease. The pathologic changes in multiple sclerosis, such as perivascular infiltration with lymphocytes, plasma cells and demyelination are similar to those seen in old dog encephalitis. Demyelination in old dog encephalitis is usually diffuse. The findings strongly support a possible relationship of old dog encephalitis to multiple sclerosis, subacute sclerosing panencephalitis, and neuromyelitis optica.

scholarly journals TRANSVERSE MYELITIS IN A MYASTHENIA GRAVIS PATIENT. A CASE PRESENTATION

2016 ◽  
Vol 15 (1) ◽  
pp. 44-47
Author(s):  
Ruxanda Dana Chirileanu ◽  
◽  
Mihaela Simu ◽  
Cecilia Rosca ◽  
Raluca Tudor ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Neuromuscular Transmission ◽  
Transverse Myelitis ◽  
Acute Disseminated Encephalomyelitis ◽  
Demyelinating Diseases ◽  
Nicotinic Acetylcholine ◽  
Case Presentation ◽  
Autoimmune Syndrome ◽  
Immunoglobulin Treatment ◽  
Recurrent Transverse Myelitis

Myasthenia gravis (MG) is an autoimmune disease caused by the presence of immunoglobulin G (IgG)1 and IgG3 complement activating antibodies against the nicotinic acetylcholine receptor, which affects the neuromuscular junction leading to fluctuating muscle weakness due to impaired neuromuscular transmission. The association of MG and demyelinating diseases is rare, but it has been described before and it could be part of an unspecific immune activation, due to genetic susceptibility or it could just happen randomly. Demyelinating diseases (DD) in MG patients can occur as monophasic events (myelitis, optic neuritis, acute disseminated encephalomyelitis) or recurrent diseases (multiple sclerosis, recurrent transverse myelitis) and since the incidence of DD is higher in MG patients than in general population this association could be part of an autoimmune syndrome or genetically induced. We present the case of a 30 year old woman who presented with an unspecific onset of MG and after 5 months was readmitted to our unit with transverse myelitis (with negative aquaporin 4 antibodies) which regressed after 5 days of combined intravenous corticotherapy and immunoglobulin treatment.

Diagnosis, assessment, and management of myasthenia gravis and paramyasthenic syndromes

2016 ◽  
Author(s):  
Ugan Reddy ◽  
Nicholas Hirsch
Keyword(s):  
Autoimmune Disease ◽  
Myasthenia Gravis ◽  
Muscle Weakness ◽  
Motor Nerve ◽  
Neuromuscular Blocking ◽  
Myasthenic Crisis ◽  
Igg Antibodies ◽  
Limb Weakness ◽  
Myasthenic Syndrome ◽  
Long Acting

Diseases that affect the neuromuscular junction (NMJ) interfere with normal nerve transmission and cause weakness of voluntary muscles. The two most commonly encountered are acquired myasthenia gravis (MG) and the Lambert–Eaton myasthenic syndrome (LEMS). Acquired MG is an autoimmune disease in which antibodies are directed towards receptors at the NMJ. In 85% of patients, IgG antibodies against the postsynaptic acetylcholine receptor (AChR) are found (seropositive MG). The thymus gland appears to be involved in the production of these which cause an increase rate of degradation of AChR resulting in a decreased receptor density resulting in a reduced postsynaptic end-plate potential following motor nerve stimulation and leading to muscle weakness. Although all voluntary muscles can be affected, ocular, bulbar, respiratory, and proximal limb weakness predominates. In the majority of seronegative patients, an antibody directed towards a NMJ protein called muscle specific tyrosine kinase (MUSK) is found. Anti-MUSK MG is characterized by severe bulbar and respiratory muscle weakness. Diagnosis of MG requires a high degree of clinical suspicion coupled with pharmacological and electrophysiological testing, and detection of the various causative antibodies. Treatment of MG involves enhancing neuromuscular transmission with long-acting anticholinesterase agents and immunosuppression. Acute exacerbations are treated with either plasma exchange or intravenous immunoglobulin. Myasthenic crisis is associated with severe muscle weakness that necessitates tracheal intubation and mechanical ventilation. LEMS is an autoimmune disease in which IgG antibodies are directed towards the pre-synaptic voltage-gated calcium channels at the NMJ. It is often associated with malignant disease (usually small cell carcinoma of the lung). Autonomic dysfunction is prominent and patients show abnormal responses to neuromuscular blocking drugs.

scholarly journals Surgical Treatment is Effective in Treating Thymoma Accompanied with Non-Myasthenia Gravis Autoimmune Diseases: A Single-Center Clinical Experience

2020 ◽  
pp. 145749692096433
Author(s):  
L. Liu ◽  
J. Zhang ◽  
G. Wang ◽  
C. Guo ◽  
Y. Chen ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Autoimmune Disease ◽  
Autoimmune Diseases ◽  
Myasthenia Gravis ◽  
Complete Recovery ◽  
Treatment Method ◽  
Disease Symptoms ◽  
Organ Specific ◽  
The Mean

Background and Aims: Although thymoma is inextricably linked to autoimmune disease, its best treatment method remains unclear. In this study, we sought to evaluate therapeutic effect of surgical resection of thymoma on non-myasthenia gravis autoimmune diseases. Materials and Methods: This was a retrospective study covering 32 patients with thymoma accompanied with non-myasthenia gravis autoimmune disease. The relationships between surgical treatment, thymoma pathological type, and prognosis of autoimmune diseases were analyzed from postoperative follow-up data. Results: In total, 32 patients in this study underwent surgical treatment. The mean age of the patients was 51.7 years. By the last follow-up, 2 patients had died, while the other 30 patients showed no sign of tumor recurrence and metastasis. According to the postoperative follow-up data, 22 patients (68.75%) showed improvement or even complete recovery of autoimmune disease symptoms, 9 patients (28.13%) showed no significant change, and only 1 patient’s (3.12%) postoperative symptom was aggravated. Female patients and patients aged 50 and older were more likely to combine with non-organ-specific autoimmune diseases (p = 0.036, p = 0.017). Conclusion: In conclusion, this study presents that surgical treatment achieves a satisfactory prognosis for thymoma combined with non-myasthenia gravis autoimmune disease.

scholarly journals Myelin insulation as a risk factor for axonal degeneration in autoimmune demyelinating disease

2021 ◽  
Author(s):  
Erik Schaeffner ◽  
Julia Edgar ◽  
Maria Lehning ◽  
Judith Strauss ◽  
Mar Bosch-Queralt ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Risk Factor ◽  
Autoimmune Disease ◽  
Clinical Outcome ◽  
Axonal Degeneration ◽  
Demyelinating Disease ◽  
Demyelinating Diseases ◽  
Extracellular Milieu ◽  
Inflammatory Conditions ◽  
Immune Mediated

Axonal degeneration determines the clinical outcome of multiple sclerosis (MS), and is thought to result from exposure of denuded axons to immune-mediated damage. We challenge this view after finding in MS and its mouse models that myelin itself increases the risk of axons to degenerate under inflammatory conditions. We propose a model for demyelinating diseases in which for axons that remain myelinated, and thus shielded from the extracellular milieu, dependence from oligodendroglial support turns fatal in an autoimmune disease environment.

scholarly journals Systemic lupus erythematosus with the development of neuromyelitis optica (Devic's syndrome) is a rare combination of autoimmune diseases

2019 ◽  
Vol 13 (4) ◽  
pp. 89-95 ◽  
Author(s):  
E. S. Vinogradova ◽  
A. P. Panova ◽  
N. M. Bulanov ◽  
P. I. Novikov ◽  
S. V. Moiseev
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Autoimmune Diseases ◽  
Neuromyelitis Optica ◽  
Lupus Erythematosus ◽  
Demyelinating Disease ◽  
Transverse Myelitis ◽  
Young Female ◽  
Systemic Lupus ◽  
Devic’S Syndrome ◽  
Immune Mediated

Neuromyelitis optica ((NMO), Devic's syndrome) is an immune-mediated inflammatory demyelinating disease characterized by transverse myelitis and optic neuritis. Determination of the level of antibodies to aquaporin 4 (NMO-IgG) is presently one of the key methods for the diagnosis and assessment of the activity of ONM, which allows this disease to be differentiated from multiple sclerosis and other demyelinating CNS lesions. ONM can occur not only as an independent disease, but also as a syndrome in different systemic diseases, such as: systemic lupus erythematosus (SLE), antineutrophilic cytoplasmic antibody-associated vasculitides, Sjögren's disease, etc. (up to 50–70%). In such situations, the clinician is always confronted with a question as whether the patient can have two rare autoimmune diseases or develop ONM as a systemic manifestation of rheumatic disease.The paper describes a clinical case of a young female patient with SLE concurrent with a CNS lesion, the manifestations of which corresponded to ONM. The patient had focal changes in the substance of the brain and spinal cord, as evidenced by magnetic resonance imaging, as well as high NMO-IgG titers. The development of ONM worsens SLE prognosis and requires active immunosuppressive therapy. The patient received three plasmapheresis sessions, ultrahigh-dose glucocorticoid and cyclophosphamide therapy, followed by replacement with azathioprine, causing a stable clinical and laboratory disease remission to be achieved.

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