Journal articles: 'Neuromuscular autoimmune disease'

1

Parry, Garth, and Terry Heiman-Patterson. "Pregnancy and Autoimmune Neuromuscular Disease." Seminars in Neurology 8, no. 03 (September 1988): 197–204. http://dx.doi.org/10.1055/s-2008-1041378.

Full text

APA, Harvard, Vancouver, ISO, and other styles

2

Tanovska, Nikolina, Gabriela Novotni, Slobodanka Sazdova-Burneska, Igor Kuzmanovski, Bojan Boshkovski, Goran Kondov, Marija Jovanovski-Srceva, Anita Kokareva, and Rozalinda Isjanovska. "Myasthenia Gravis and Associated Diseases." Open Access Macedonian Journal of Medical Sciences 6, no. 3 (March 5, 2018): 472–78. http://dx.doi.org/10.3889/oamjms.2018.110.

Full text

Abstract:

BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease caused by the action of specific antibodies to the postsynaptic membrane of the neuromuscular junction, leading to impaired neuromuscular transmission. Patients with MG have an increased incidence of other autoimmune diseases.AIM: to determine the presence of other associated diseases in patients with MG.METHOD: A group of 127 patients with MG followed in 10 years period, in which the presence of other associated diseases has been analysed.RESULTS: The sex ratio is in favour of the female sex, the average age of the initial manifestation of the disease is less than 50 years, 65.4% of the patients with MG have another disease. 15.0% patients have associated another autoimmune disease. Thyroid disease is the most common associated with MG, rarely rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and other autoimmune diseases. Other diseases include hypertension, heart disease, diabetes, respiratory diseases, dyslipidemia. 10.2% of the patients are diagnosed with extrathymic tumours of various origins.CONCLUSION: Associated diseases are common in patients with MG, drawing attention to the possible common basis for their coexistence, as well as their impact on the intensity and treatment of the disease.

APA, Harvard, Vancouver, ISO, and other styles

3

Bichuetti, Denis Bernardi, Tatiane Martins de Barros, Enedina Maria Lobato Oliveira, Marcelo Annes, and Alberto Alain Gabbai. "Demyelinating disease in patients with myasthenia gravis." Arquivos de Neuro-Psiquiatria 66, no. 1 (March 2008): 5–7. http://dx.doi.org/10.1590/s0004-282x2008000100002.

Full text

Abstract:

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.

APA, Harvard, Vancouver, ISO, and other styles

4

Levinson, Arnold I., Decheng Song, Glen Gaulton, and Yi Zheng. "The Intrathymic Pathogenesis of Myasthenia Gravis." Clinical and Developmental Immunology 11, no. 3-4 (2004): 215–20. http://dx.doi.org/10.1080/17402520400001769.

Full text

Abstract:

The thymus is considered to play an important role in the pathogenesis of Myasthenia gravis, an autoimmune disease characterized by antibody-mediated skeletal muscle weakness. However, its role is yet to be defined. The studies described herein summarize our efforts to determine how intrathymic expression of the neuromuscular type of acetylcholine (ACh) receptors is involved in the immunopathogenesis of this autoimmune disease. We review the work characterizing the expression of neuromuscular ACh receptors in the thymus and advance a new hypothesis that examines the intrathymic expression of this autoantigen in disease pathogenesis.

APA, Harvard, Vancouver, ISO, and other styles

5

Kopishinskaya, S. V., N. O. Zharinova, I. A. Velichko, N. G. Zhukova, V. V. Bucev, I. V. Korobejnikov, A. A. Gasanova, A. S. Arakelyan, O. V. Petruchik, and A. N. Payudis. "Basic principles for the management of neurological patients during the COVID-19 pandemic." Neuromuscular Diseases 10, no. 1 (June 3, 2020): 31–42. http://dx.doi.org/10.17650/2222-8721-2020-10-1-31-42.

Full text

Abstract:

In a COVID-19 pandemic, a neurologist needs to be able to assess the risks of virus infection in patients with individual neurological diseases. The review presents categories of risk groups from the Association of British Neurologists for neuromuscular diseases, multiple sclerosis and other autoimmune diseases of the central nervous system, stroke, epilepsy and Parkinson’s disease. The risk of infection and the management of patients with neuromuscular diseases are analyzed in detail. The use of multiple sclerosis disease modifying drugs, the treatment of stroke patients are discussed. The data from the international guidelines for the management of patients with epilepsy and Parkinson’s disease are presented.

APA, Harvard, Vancouver, ISO, and other styles

6

Jacob, Saiju. "Myasthenia Gravis – A Review of Current Therapeutic Options." European Neurological Review 13, no. 2 (2018): 86. http://dx.doi.org/10.17925/enr.2018.13.2.86.

Full text

Abstract:

Myasthenia gravis (MG) is an autoimmune disorder that leads to skeletal muscle weakness and fatigue. The autoimmune attack is caused by autoantibodies against the acetylcholine postsynaptic receptors at the neuromuscular junction of skeletal muscles. However, other antigenic targets that are components of the neuromuscular junction have also been implicated in the pathogenesis of MG. The current standard of care is immunosuppressive therapy; however, many existing therapeutic options have not been validated for use in MG in large randomised controlled trials. Furthermore, around 10% of patients with generalised MG are refractory to treatment. The complement system is involved in numerous inflammatory, neurodegenerative and autoimmune diseases, and is a key factor in the pathogenesis of acetylcholine receptor antibody-related MG. Targeting complement and other components involved in the underlying pathogenesis of the disease may provide useful treatment options, particularly for refractory patients.

APA, Harvard, Vancouver, ISO, and other styles

7

Herbert, Megan K., Judith Stammen-Vogelzangs, Marcel M. Verbeek, Anke Rietveld, Ingrid E. Lundberg, Hector Chinoy, Janine A. Lamb, et al. "Disease specificity of autoantibodies to cytosolic 5′-nucleotidase 1A in sporadic inclusion body myositis versus known autoimmune diseases." Annals of the Rheumatic Diseases 75, no. 4 (February 24, 2015): 696–701. http://dx.doi.org/10.1136/annrheumdis-2014-206691.

Full text

Abstract:

ObjectivesThe diagnosis of inclusion body myositis (IBM) can be challenging as it can be difficult to clinically distinguish from other forms of myositis, particularly polymyositis (PM). Recent studies have shown frequent presence of autoantibodies directed against cytosolic 5′-nucleotidase 1A (cN-1A) in patients with IBM. We therefore, examined the autoantigenicity and disease specificity of major epitopes of cN-1A in patients with sporadic IBM compared with healthy and disease controls.MethodsSerum samples obtained from patients with IBM (n=238), PM and dermatomyositis (DM) (n=185), other autoimmune diseases (n=246), other neuromuscular diseases (n=93) and healthy controls (n=35) were analysed for the presence of autoantibodies using immunodominant cN-1A peptide ELISAs.ResultsAutoantibodies directed against major epitopes of cN-1A were frequent in patients with IBM (37%) but not in PM, DM or non-autoimmune neuromuscular diseases (<5%). Anti-cN-1A reactivity was also observed in some other autoimmune diseases, particularly Sjögren's syndrome (SjS; 36%) and systemic lupus erythematosus (SLE; 20%).ConclusionsIn summary, we found frequent anti-cN-1A autoantibodies in sera from patients with IBM. Heterogeneity in reactivity with the three immunodominant epitopes indicates that serological assays should not be limited to a distinct epitope region. The similar reactivities observed for SjS and SLE demonstrate the need to further investigate whether distinct IBM-specific epitopes exist.

APA, Harvard, Vancouver, ISO, and other styles

8

Gajdos, P. "Contribution of intravenous immunoglobulins to the treatment of myasthenia." Neurology Bulletin XXXIII, no. 1-2 (May 15, 2001): 114–15. http://dx.doi.org/10.17816/nb79794.

Full text

APA, Harvard, Vancouver, ISO, and other styles

9

Mizrachi, Tehila, Livnat Brill, Malcolm Rabie, Yoram Nevo, Yakov Fellig, Mayan Zur, Dimitrios Karussis, Oded Abramsky, Talma Brenner, and Adi Vaknin-Dembinsky. "NMO-IgG and AQP4 Peptide Can Induce Aggravation of EAMG and Immune-Mediated Muscle Weakness." Journal of Immunology Research 2018 (2018): 1–10. http://dx.doi.org/10.1155/2018/5389282.

Full text

Abstract:

Neuromyelitis optica (NMO) and myasthenia gravis (MG) are autoimmune diseases mediated by autoantibodies against either aquaporin 4 (AQP4) or acetylcholine receptor (AChR), respectively. Recently, we and others have reported an increased prevalence of NMO in patients with MG. To verify whether coexisting autoimmune disease may exacerbate experimental autoimmune MG, we tested whether active immunization with AQP4 peptides or passive transfer of NMO-Ig can affect the severity of EAMG. Injection of either AQP4 peptide or NMO-Ig to EAMG or to naive mice caused increased fatigability and aggravation of EAMG symptoms as expressed by augmented muscle weakness (but not paralysis), decremental response to repetitive nerve stimulation, increased neuromuscular jitter, and aberration of immune responses. Thus, our study shows increased disease severity in EAMG mice following immunization with the NMO autoantigen AQP4 or by NMO-Ig, mediated by augmented inflammatory response. This can explain exacerbation or increased susceptibility of patients with one autoimmune disease to develop additional autoimmune syndrome.

APA, Harvard, Vancouver, ISO, and other styles

10

Chirileanu, Ruxanda Dana, Mihaela Simu, Cecilia Rosca, Raluca Tudor, and Patricia Jurca. "TRANSVERSE MYELITIS IN A MYASTHENIA GRAVIS PATIENT. A CASE PRESENTATION." Romanian Journal of Neurology 15, no. 1 (March 31, 2016): 44–47. http://dx.doi.org/10.37897/rjn.2016.1.7.

Full text

Abstract:

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.

APA, Harvard, Vancouver, ISO, and other styles

11

Vincent, Angela, and Maria Isabel Leite. "Neuromuscular junction autoimmune disease: muscle specific kinase antibodies and treatments for myasthenia gravis." Current Opinion in Neurology 18, no. 5 (October 2005): 519–25. http://dx.doi.org/10.1097/01.wco.0000180660.57801.3f.

Full text

APA, Harvard, Vancouver, ISO, and other styles

12

Asaduzzaman, Mohammad, Uzzwal Kumar Mallick, Shemu Sultana, Silfat Azam, Md Shamsul Hoque, Masud Kabir, M. Sakhawat Hossain, Kazi Ikramul Haque, and Farhana Mumtaz. "A case of Myasthenia Gravis masquerading as GBS." Bangladesh Critical Care Journal 6, no. 1 (May 9, 2018): 54–56. http://dx.doi.org/10.3329/bccj.v6i1.36614.

Full text

Abstract:

Myasthenia gravis (MG) is an autoimmune neurologic disease that affects the post synaptic portion of the neuromuscular junction. Usually MG occur in young adults or in the elderly characterized by weakness of fatigue of skeletal muscles due to repetitive use.1-2 It represents a challenge for clinicians due to the diversity of disease manifestations. It is therefore important to monitor the neuromuscular blockade (NMB) due to the multiple presentations of MG. In our case the patient presented with clinical features suggestive of Guillen Barre Syndrome (GBS) which is an autoimmune disease against myelin nerve sheath. Initially he was planned to be treated as GBS because of it’s unusual descending paralysis like presentation. Nerve conduction studies were done a few times and were found to be normal. Anti acetylcholine receptor antibody test was found positive. Plane X Ray of chest and CT scan of Chest showed a mediastinal mass suggestive of Thymoma. Patient recovered dramatically after receiving treatment for Myasthenia Gravis.Bangladesh Crit Care J March 2018; 6(1): 54-56

APA, Harvard, Vancouver, ISO, and other styles

13

Teodorescu, Andreea, Petru Ifteni, Paula Petric, Sebastian Toma, Adrian Baracan, Claudia Gavris, Gheorghe G. Balan, Vladimir Poroch, and Alina Mihaela Pascu. "Acetylcholinesterase Inhibitors Test Confirmed Myasthenia Gravis in Psychosis Remitted by Aripiprazole." Revista de Chimie 68, no. 12 (January 15, 2018): 2952–54. http://dx.doi.org/10.37358/rc.17.12.6014.

Full text

Abstract:

Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disease affecting the neuromuscular junction and causes weakness in the skeletal muscles. The acetylcholine receptor is usually attacked in skeletal muscles, but other components of neuromuscular junction, such as muscle-specific receptor tyrosine kinase, may be affected. MG can be life-threatening when the respiratory muscles are involved. The first symptom in about 2 out of 3 cases is the damage of the extrinsic eye muscles. The condition is treatable, so an early recognition is needed. Although there have been reports of associations between psychosis and myasthenia gravis it is unclear if psychotic symptoms in MG are an integral part of the various manifestations of this disease, or are due to another co-occurring distinct disorder. Sometimes psychotic episodes could disguise the simptoms of myastenia gravis, and delay the diagnosis.

APA, Harvard, Vancouver, ISO, and other styles

14

Samuel, Hanock Unni, N. V. Jayachandran, and N. K. Thulaseedharan. "A case of juvenile polymyositis post viral hepatitis A." International Journal of Advances in Medicine 4, no. 3 (May 23, 2017): 864. http://dx.doi.org/10.18203/2349-3933.ijam20172288.

Full text

Abstract:

Polymyositis is rare as a stand-alone entity and is often misdiagnosed; most patients whose condition has been diagnosed as polymyositis have inclusion-body myositis, necrotizing autoimmune myositis, or inflammatory dystrophy. Polymyositis remains a diagnosis of exclusion and is best defined as a subacute proximal myopathy in adults who do not have rash, a family history of neuromuscular disease, exposure to myotoxic drugs (e.g. statins, penicillamine, and zidovudine), involvement of facial and extraocular muscles, endocrinopathy, or the clinical phenotype of inclusion body myositis. The etiology of polymyositis may be due to underlying systemic autoimmune diseases, viral, parasitic, bacterial infections or drug induced. Here we describe a case of juvenile polymyositis post viral infection with hepatitis A.

APA, Harvard, Vancouver, ISO, and other styles

15

Shahrizaila, Nortina, and Nobuhiro Yuki. "Guillain-Barré Syndrome Animal Model: The First Proof of Molecular Mimicry in Human Autoimmune Disorder." Journal of Biomedicine and Biotechnology 2011 (2011): 1–5. http://dx.doi.org/10.1155/2011/829129.

Full text

Abstract:

Molecular mimicry between self and microbial components has been proposed as the pathogenic mechanism of autoimmune diseases, and this hypothesis is proven in Guillain-Barré syndrome. Guillain-Barré syndrome, the most frequent cause of acute neuromuscular paralysis, sometimes occurs afterCampylobacter jejunienteritis. Gangliosides are predominantly cell-surface glycolipids highly expressed in nervous tissue, whilst lipo-oligosaccharides are major components of the Gram-negative bacteriumC. jejuniouter membrane. IgG autoantibodies to GM1 ganglioside were found in the sera from patients with Guillain-Barré syndrome. Molecular mimicry was demonstrated between GM1 and lipo-oligosaccharide ofC. jejuniisolated from the patients. Disease models by sensitization of rabbits with GM1 andC. jejunilipo-oligosaccharide were established. Guillain-Barré syndrome provided the first verification that an autoimmune disease is triggered by molecular mimicry. Its disease models are helpful to further understand the molecular pathogenesis as well as to develop new treatments in Guillain-Barré syndrome.

APA, Harvard, Vancouver, ISO, and other styles

16

Phillips, William D., and Angela Vincent. "Pathogenesis of myasthenia gravis: update on disease types, models, and mechanisms." F1000Research 5 (June 27, 2016): 1513. http://dx.doi.org/10.12688/f1000research.8206.1.

Full text

Abstract:

Myasthenia gravis is an autoimmune disease of the neuromuscular junction (NMJ) caused by antibodies that attack components of the postsynaptic membrane, impair neuromuscular transmission, and lead to weakness and fatigue of skeletal muscle. This can be generalised or localised to certain muscle groups, and involvement of the bulbar and respiratory muscles can be life threatening. The pathogenesis of myasthenia gravis depends upon the target and isotype of the autoantibodies. Most cases are caused by immunoglobulin (Ig)G1 and IgG3 antibodies to the acetylcholine receptor (AChR). They produce complement-mediated damage and increase the rate of AChR turnover, both mechanisms causing loss of AChR from the postsynaptic membrane. The thymus gland is involved in many patients, and there are experimental and genetic approaches to understand the failure of immune tolerance to the AChR. In a proportion of those patients without AChR antibodies, antibodies to muscle-specific kinase (MuSK), or related proteins such as agrin and low-density lipoprotein receptor-related protein 4 (LRP4), are present. MuSK antibodies are predominantly IgG4 and cause disassembly of the neuromuscular junction by disrupting the physiological function of MuSK in synapse maintenance and adaptation. Here we discuss how knowledge of neuromuscular junction structure and function has fed into understanding the mechanisms of AChR and MuSK antibodies. Myasthenia gravis remains a paradigm for autoantibody-mediated conditions and these observations show how much there is still to learn about synaptic function and pathological mechanisms.

APA, Harvard, Vancouver, ISO, and other styles

17

Zagoriti, Zoi, Manousos E. Kambouris, George P. Patrinos, Socrates J. Tzartos, and Konstantinos Poulas. "Recent Advances in Genetic Predisposition of Myasthenia Gravis." BioMed Research International 2013 (2013): 1–12. http://dx.doi.org/10.1155/2013/404053.

Full text

Abstract:

Myasthenia gravis (MG) is an autoimmune disease mediated by the presence of autoantibodies that bind to components of the neuromuscular junction, causing the symptoms of muscular weakness and fatigability. Like most autoimmune disorders, MG is a multifactorial, noninherited disease, though with an established genetic constituent. The heterogeneity observed in MG perplexes genetic analysis even more, as it occurs in various levels, including diverse autoantigens, thymus histopathology, and age at onset. In this context of distinct subgroups, a plethora of association studies, discussed in this review, have assessed the involvement of various HLA and non-HLA related loci in MG susceptibility, over the past five years. As expected, certain HLA alleles were strongly associated with MG. Many of the non-HLA genes, such asPTPN22andCTLA-4, have been previously studied in MG and other autoimmune diseases and their association with MG has been reevaluated in more cohesive groups of patients. Moreover, novel risk or protective loci have been revealed, as in the case ofTNIP1andFOXP3. Although the majority of these results have been derived from candidate gene studies, the focal point of all recent genetic studies is the first genome-wide association study (GWAS) conducted on early-onset MG patients.

APA, Harvard, Vancouver, ISO, and other styles

18

Greenberg, Steven A., Jack L. Pinkus, Sek Won Kong, Clare Baecher-Allan, Anthony A. Amato, and David M. Dorfman. "Highly differentiated cytotoxic T cells in inclusion body myositis." Brain 142, no. 9 (July 20, 2019): 2590–604. http://dx.doi.org/10.1093/brain/awz207.

Full text

Abstract:

Abstract Inclusion body myositis is a late onset treatment-refractory autoimmune disease of skeletal muscle associated with a blood autoantibody (anti-cN1A), an HLA autoimmune haplotype, and muscle pathology characterized by cytotoxic CD8+ T cell destruction of myofibres. Here, we report on translational studies of inclusion body myositis patient muscle compared with a diverse set of other muscle disease samples. Using available microarray data on 411 muscle samples from patients with inclusion body myositis (n = 40), other muscle diseases (n = 265), and without neuromuscular disease (normal, n = 106), we identified a signature of T-cell cytotoxicity in inclusion body myositis muscle coupled with a signature of highly differentiated CD8 T-cell effector memory and terminally differentiated effector cells. Further, we examined killer cell lectin-like receptor G1 (KLRG1) as a marker of this population of cells, demonstrated the correlation of KLRG1 gene expression with lymphocyte cytotoxicity across 28 870 human tissue samples, and identified the presence of KLRG1 on pathogenic inclusion body myositis muscle invading T cells and an increase in KLRG1 expressing T cells in inclusion body myositis blood. We examined inclusion body myositis muscle T-cell proliferation by Ki67 immunohistochemistry demonstrating that diseased muscle-invading T cells are minimally or non-proliferative, in accordance with known properties of highly differentiated or terminally differentiated T cells. We found low expression of KLRG1 on infection-protective human lymphoid tissue central memory T cells and autoimmune-protective human blood regulatory T cells. Targeting highly differentiated cytotoxic T cells could be a favourable approach to treatment of inclusion body myositis.

APA, Harvard, Vancouver, ISO, and other styles

19

Wu, Yufan, Boleslaw Lach, John P. Provias, Mark A. Tarnopolsky, and Steven K. Baker. "Statin-associated Autoimmune Myopathies: A Pathophysiologic Spectrum." Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 41, no. 5 (September 2014): 638–47. http://dx.doi.org/10.1017/cjn.2014.22.

Full text

Abstract:

AbstractBackgroundStatins have recently been reported to cause a rare autoimmune inflammatory and/or necrotic myopathy that begins or persists after drug cessation.MethodsWe report on 26 patients seen at a neuromuscular centre between 2005 and 2011 who demonstrated muscle weakness/myalgias and creatine kinase elevations during or after statin treatment with continuation of signs and symptoms despite statin withdrawal.ResultsAll patients were treated with immunosuppressive therapy with good response; all improved biochemically and 86% improved clinically. Sixty-five percent of patients who attempted to taper off immunosuppressive therapy relapsed. We report on a novel finding whereby five of the seven patients who underwent multiple biopsies throughout their disease demonstrated a transformation of their histological diagnosis, with four progressing from having myofibre necrosis with minimal or no inflammation to a diagnosis of polymyositis.ConclusionsThis study offers preliminary evidence that statin-associated necrotizing myopathy and statin-associated polymyositis may not be separate entities but are part of the same pathophysiological spectrum. Both entities respond well to immunosuppression.

APA, Harvard, Vancouver, ISO, and other styles

20

Koneczny, Inga, and Ruth Herbst. "Myasthenia Gravis: Pathogenic Effects of Autoantibodies on Neuromuscular Architecture." Cells 8, no. 7 (July 2, 2019): 671. http://dx.doi.org/10.3390/cells8070671.

Full text

Abstract:

Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction (NMJ). Autoantibodies target key molecules at the NMJ, such as the nicotinic acetylcholine receptor (AChR), muscle-specific kinase (MuSK), and low-density lipoprotein receptor-related protein 4 (Lrp4), that lead by a range of different pathogenic mechanisms to altered tissue architecture and reduced densities or functionality of AChRs, reduced neuromuscular transmission, and therefore a severe fatigable skeletal muscle weakness. In this review, we give an overview of the history and clinical aspects of MG, with a focus on the structure and function of myasthenic autoantigens at the NMJ and how they are affected by the autoantibodies’ pathogenic mechanisms. Furthermore, we give a short overview of the cells that are implicated in the production of the autoantibodies and briefly discuss diagnostic challenges and treatment strategies.

APA, Harvard, Vancouver, ISO, and other styles

21

Sinaei, Farnaz, Farzad Fatehi, Shahram Oveis Gharan, Soroush Ehsan, Koorosh Kamali, Aliakbar Amirzargar, Mahdi Mahmoudi, Alaleh Vaghefifar, and Shahriar Nafissi. "Association of HLA Class II Alleles with Disease Severity and Treatment Response in Iranian Patients with Myasthenia Gravis." Journal of Neuromuscular Diseases 8, no. 5 (September 14, 2021): 827–29. http://dx.doi.org/10.3233/jnd-210700.

Full text

Abstract:

Myasthenia gravis is an autoimmune neuromuscular disease with a multifactorial etiology. A major part of the genetic susceptibility belongs to the HLA encoding genes. In this study, we investigated the role of HLA class II polymorphism in disease severity, and treatment response. In our 146 patients, 15 DRB1, 7 DQA1, and 9 DQB1 alleles, and 19 haplotypes were found. Adjusted p-values did not show any significant associations between these loci, disease severity and treatment outcome. Further studies in different populations with a larger number of patients are needed to determine the exact contribution of HLA class II alleles to MG prognosis.

APA, Harvard, Vancouver, ISO, and other styles

22

Arnon, Roee, Tal Yahalomi, Irit Rozen-Knisbacher, Joseph Pikkel, and Dina Mostovoy. "Ocular Myasthenia Gravis with Severe Blepharitis and Ocular Surface Disease: A Case Report." Case Reports in Ophthalmology 11, no. 2 (July 13, 2020): 322–29. http://dx.doi.org/10.1159/000508815.

Full text

Abstract:

Ocular myasthenia gravis (OMG) is an autoimmune disease of the neuromuscular junction and commonly associated with other immune diseases. We describe a 16-year-old female who presented to our clinic with 1-month complaints of diplopia and strabismus, visual acuity deterioration, and ocular irritation. Her examination showed crossed diplopia and alternating exotropia of 25 prism diopters, severe blepharitis, conjunctival hyperemia, corneal pannus, epithelial irregularities, and subepithelial opacities. Workup included pediatric neurologic examination, laboratory tests, imaging, and electrophysiological tests. Diagnoses of OMG and blepharitis with ocular surface disease were made. Topical treatment included eyelid hygiene, tea tree oil scrubbing, topical steroids, and tacrolimus ointment. Systemic treatment included corticosteroids, pyridostigmine, azathioprine, intravenous immunoglobulins, amitriptyline, and doxycycline. Both diseases were refractory to intensive immunosuppressive treatment and had simultaneous relapses and an intertwined course. Our hypothesis is that a shared immune mechanism may be the cause of both OMG and ocular surface disease in our patient.

APA, Harvard, Vancouver, ISO, and other styles

23

Philippe, Aurélie, Rusan Catar, Björn Hegner, and Duska Dragun. "Autoimmune mediated G-protein receptor activation in cardiovascular and renal pathologies." Thrombosis and Haemostasis 101, no. 04 (2009): 643–48. http://dx.doi.org/10.1160/th08-10-0710.

Full text

Abstract:

SummaryAntibodies directed against G-protein coupled receptors (GPCR) can act as allosteric receptor agonists or antagonists. Prototypic disease for agonistic antibody action is a Graves disease of the thyroid gland where antibodies that stimulate G-protein coupled thyroid-stimulating hormone receptor (TSHR) were first described 50 years ago. Myasthenia gravis is the prototype for antagonistic autoimmune actions, where antibodies directed against the nicotinic acetylcholine receptor (AChR) cause blockade of neuromuscular junctions. Antibodies and B-cells are increasingly recognised as major modulators of various cardiovascular and renal pathologies. We aim to critically review the notion that antibodies targeting other GPCRs may amplify or cause various cardiovascular and renal pathologies and summarise the current state of research, as well as perspectives in diagnostic and therapeutic strategies. In terms of targets we will focus on the α-adrenergic receptor (α1AR), the β-adrenergic receptor (β1AR), and the angiotensin II type 1 receptor (AT1R).

APA, Harvard, Vancouver, ISO, and other styles

24

Grozdanova, Aleksandra, Slobodan Apostolski, and Ljubica Suturkova. "The role of molecular mimicry in the etiology of Guillain Barré Syndrome." Macedonian Pharmaceutical Bulletin 56 (May 2011): 3–12. http://dx.doi.org/10.33320/maced.pharm.bull.2010.56.001.

Full text

Abstract:

Molecular mimicry between host tissue structures and microbial components has been proposed as the pathogenic mechanism for triggering of autoimmune diseases by preceding infection. Recent studies stated that molecular mimicry as the causative mechanism remains unproven for most of the human diseases. Still, in the case of the peripheral neuropathy Guillain-Barré syndrome (GBS) this hypothesis is supported by abundant experimental evidence. GBS is the most frequent cause of acute neuromuscular paralysis and in some cases occurs after infection with Campylobacter jejuni (C. jejuni). Epidemiological studies, showed that more than one third of GBS patients had antecedent C. jejuni infection and that only specific C. jejuni serotypes are associated with development of GBS. The molecular mimicry between the human gangliosides and the core oligosaccharides of bacterial lipopolysaccharides (LPSs) presumably results in production of antiganglioside cross-reactive antibodies which are likely to be a contributory factor in the induction and pathogenesis of GBS. Antiganglioside antibodies were found in the sera from patients with GBS and by sensitization of rabbits with gangliosides and C. jejuni LPSs animal disease models of GBS were established. GBS as prototype of post-infection immune-mediated disease probably will provide the first verification that an autoimmune disease can be triggered by molecular mimicry.

APA, Harvard, Vancouver, ISO, and other styles

25

Silvestri, Nicholas, Shafeeq Ladha, Tahseen Mozaffar, Gretchen Ayer, and Lisa M. Betts. "Patient-reported Outcomes—An Emerging Cornerstone of Effective Intravenous Immunoglobulin Therapy." US Neurology 11, no. 01 (2015): 40. http://dx.doi.org/10.17925/usn.2015.11.01.40.

Full text

Abstract:

Intravenous immunoglobulin (IVIG) therapy is increasingly important in the management of various immune-mediated neuromuscular disorders including chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), myasthenia gravis (MG), and other neuromuscular disorders. Administrative burden, quality of life (QoL) concerns, adverse event prevention, economic pressures, and logistical factors are driving greater IVIG use into the home setting where it is administered by nurses. Patient-reported outcome measures (PROMs) are selfassessment instruments designed to measure a patients disability, QoL, or their perceptions of health status in relation to specific diseases. PROMs may be a valuable means of monitoring disease status and treatment efficacy in patients receiving IVIG at home. Case reports and small clinical studies show that various specific and general purpose PROMs, such as the 15-item MG-specific QOL (MG-QOL15) and the Myasthenia Gravis Activities of Daily Living scale (MG-ADL), can provide valuable information for patient monitoring at home. PROMs may help to alert physicians that earlier follow-up or treatment regimen changes are needed. PROM data recording systems such as Walgreens PartnerPoint Clinical ManagementSMmaintain regular reporting to the physician and enable efficacy and adverse events to be tracked. Pilot studies of patients with neuromuscular disease receiving IVIG at home demonstrate a strong correlation in PROM scores between assessments administered by pharmacy clinical staff and those administered by physicians indicating the reliability and suitability of PROMs for remote patient management. Further work to validate additional commonly used PROMS for autoimmune disease is needed if they are to be useful when administered outside the physician clinic.

APA, Harvard, Vancouver, ISO, and other styles

26

Gu, D., L. Wogensen, N. A. Calcutt, C. Xia, S. Zhu, J. P. Merlie, H. S. Fox, J. Lindstrom, H. C. Powell, and N. Sarvetnick. "Myasthenia gravis-like syndrome induced by expression of interferon gamma in the neuromuscular junction." Journal of Experimental Medicine 181, no. 2 (February 1, 1995): 547–57. http://dx.doi.org/10.1084/jem.181.2.547.

Full text

Abstract:

Abnormal humoral responses toward motor end plate constituents in muscle induce myasthenia gravis (MG). To study the etiology of this disease, and whether it could be induced by host defense molecules, we examined the consequences of interferon (IFN) gamma production within the neuromuscular junction of transgenic mice. The transgenic mice exhibited gradually increasing muscular weakness, flaccid paralysis, and functional disruption of the neuromuscular junction that was reversed after administration of an inhibitor of acetylcholinesterase, features which are strikingly similar to human MG. Furthermore, histological examination revealed infiltration of mononuclear cells and autoantibody deposition at motor end plates. Immunoprecipitation analysis indicated that a previously unidentified 87-kD target antigen was recognized by sera from transgenic mice and also by sera from the majority of human MG patients studied. These results suggest that expression of IFN-gamma at motor end plates provokes an autoimmune humoral response, similar to human MG, thus linking the expression of this factor with development of this disease.

APA, Harvard, Vancouver, ISO, and other styles

27

Dresser, Laura, Richard Wlodarski, Kourosh Rezania, and Betty Soliven. "Myasthenia Gravis: Epidemiology, Pathophysiology and Clinical Manifestations." Journal of Clinical Medicine 10, no. 11 (May 21, 2021): 2235. http://dx.doi.org/10.3390/jcm10112235.

Full text

Abstract:

Myasthenia gravis (MG) is an autoimmune neurological disorder characterized by defective transmission at the neuromuscular junction. The incidence of the disease is 4.1 to 30 cases per million person-years, and the prevalence rate ranges from 150 to 200 cases per million. MG is considered a classic example of antibody-mediated autoimmune disease. Most patients with MG have autoantibodies against the acetylcholine receptors (AChRs). Less commonly identified autoantibodies include those targeted to muscle-specific kinase (MuSK), low-density lipoprotein receptor-related protein 4 (Lrp4), and agrin. These autoantibodies disrupt cholinergic transmission between nerve terminals and muscle fibers by causing downregulation, destruction, functional blocking of AChRs, or disrupting the clustering of AChRs in the postsynaptic membrane. The core clinical manifestation of MG is fatigable muscle weakness, which may affect ocular, bulbar, respiratory and limb muscles. Clinical manifestations vary according to the type of autoantibody, and whether a thymoma is present.

APA, Harvard, Vancouver, ISO, and other styles

28

Basile, Umberto, Mariapaola Marino, Cecilia Napodano, Krizia Pocino, Paolo Emilio Alboini, Francesca Gulli, Amelia Evoli, Carlo Provenzano, and Emanuela Bartoccioni. "Serological Immunoglobulin-Free Light Chain Profile in Myasthenia Gravis Patients." Journal of Immunology Research 2018 (2018): 1–8. http://dx.doi.org/10.1155/2018/9646209.

Full text

Abstract:

Background. Serological levels of free immunoglobulin light chains (FLCs), produced in excess of heavy chains during synthesis of immunoglobulins by plasma cells, can be considered a direct marker of B cell activity in different systemic inflammatory-autoimmune conditions and may represent a useful predictor of rituximab (RTX) therapeutic efficacy, as reported for rheumatoid arthritis and systemic lupus erythematosus. Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction with antibodies (abs) targeting the acetylcholine receptor (AChR) or the muscle-specific tyrosine kinase (MuSK), inducing muscle weakness and excessive fatigability. As MG course may be remarkably variable, we evaluated the possible use of FLCs as biomarkers of disease activity. Subjects and Methods. We assessed FLC levels in 34 sera from 17 AChR-MG and from 13 MuSK-MG patients, in comparison with 20 sera from patients with systemic autoimmune rheumatic diseases and 18 from healthy blood donors, along with titers of specific auto-abs and IgG subclass distribution. Results. We found a statistically significant increase in free κ chains in both AChR- and MuSK-MG patients, while free λ chain levels were increased only in AChR-MG. We also observed a significant reduction of both free κ and λ chains in 1/4 MuSK-MG patients along with specific abs titer, two months after RTX treatment. Conclusions. From our data, FLCs appear to be a sensitive marker of B cell activation in MG. Further investigations are necessary to exploit their potential as reliable biomarkers of disease activity.

APA, Harvard, Vancouver, ISO, and other styles

29

Kuci, Saimir, Alfred Ibrahimi, Shaban Memeti, Marsela Goga, Selman Dumani, and Ali Refatllari. "Perioperative Approach in a Patient with Myasthenia Gravis." Open Access Macedonian Journal of Medical Sciences 9, no. C (August 24, 2021): 136–39. http://dx.doi.org/10.3889/oamjms.2021.7002.

Full text

Abstract:

Background: Myasthenia gravis is an autoimmune neuromuscular disorder that causes the destruction and overall decrease in functional acetylcholine receptors at the neuromuscular junction. The resultant respiratory and cardiovascular implications are a primary cause of mortality; therefore, a complete and comprehensive understandings of this disorder is vital for the anesthesia provider. Anesthesia management in myasthenia gravis is a great challenge for all anesthesiologists. In this disease, even small doses of muscle relaxants could lead to delayed recovery for respiratory muscles. Case report: We present the case of a 38 years old woman (weight 87 kg) diagnosed with Myasthenia Gravis, which symptoms has worsened recently. The case demonstrates the anesthetic challenges involved, with a focus on the overall approach, pharmacologic considerations, physiological changes, and an emphasis on preoperative operative and post-operative optimization. Conclusion: Thymectomy is a common procedure performed in cases of myasthenia gravis (MG) with a thymoma or general MG that does not improve with medical therapy. During anesthesia the use of propofol or sevoflurane with opioids without the use of any neuromuscular blocking agents has been used with success.

APA, Harvard, Vancouver, ISO, and other styles

30

Dwi ariningtyas, Ninuk, and Laily Irfana. "Pregnancy with Myasthenia Gravis." Qanun Medika - Medical Journal Faculty of Medicine Muhammadiyah Surabaya 4, no. 1 (January 27, 2020): 119. http://dx.doi.org/10.30651/jqm.v4i1.3678.

Full text

Abstract:

ABSTRACTMyasthenia Gravis (MG) is a serious autoimmune disease, but now can be treated. Symptoms include weakness and fatigue in voluntary muscles caused by an autoantibody reaction to nicotinic acetylcholine receptor (AChR) at the post synapse of the neuromuscular junction. Pregnancy can affect autoimmune diseases so that pregnancy can aggravate MG disease. On the other hand it is also reported that pregnancy does not affect and can even improve MG disease. In this article, We report a 27-year-old woman who was diagnosed with myasthenia gravis that having a pregnancy. Initially she had no problems with pregnancy. Patients underwent pregnancy by taking the drug Mestinon four times daily and roborant. But entering the 33rd-34th week, the examination results showed that the pregnancy experienced oligohydramnios and Intrauterine Growth Retardation (IUGR), it was probably caused by malnutrition. Then we decided to end the patient's pregnancy with a Caesarean section. The operation went well, born to a baby boy / 2450grams / Apgar Score 5-7. Observation for one week the mother's condition continued to improve. Diplopia and weaknesses also improve. Likewise the baby showed a healthy condition. The patient was discharged while still taking MG drugs that had been previously consumed. This case report showed that pregnancy worsened MG disease, but MG did not affect pregnancy.Keywords : Pregnancy, Myasthenia Gravis

APA, Harvard, Vancouver, ISO, and other styles

31

Haque, Nazmul, Rukhsana Parvin, Naser Ahmed, and AKM Rafique Uddin. "Ice-on-Eyes Test in the Diagnosis of Myasthenia Gravis." Journal of Enam Medical College 2, no. 2 (December 5, 2012): 92–93. http://dx.doi.org/10.3329/jemc.v2i2.12845.

Full text

Abstract:

Myasthenia gravis is the most frequent autoimmune neuromuscular transmission disorder with incidence of 2-20 patients per million. Its pathophysiology is autoimmune, with acetylcholine receptor auto antibodies damaging the post-synaptic fold at the muscle membrane. The diagnostic confirmation of myasthenia gravis is often challenging. Ice-oneyes test can be used to diagnose this disease for its simplicity, safety and cost-effectiveness. Here we report a case of myasthenia gravis in Enam Medical College Hospital, Savar, Dhaka where ice-on-eyes test was done with improvement of ptosis of the patient. Aim of this case report is to make aware our physicians to apply this simple bed side test instead of common traditional edrophonium (tensilon test) test for confirmation of the diagnosis of ocular myasthenia gravis. DOI: http://dx.doi.org/10.3329/jemc.v2i2.12845 J Enam Med Col 2012; 2(2): 92-93

APA, Harvard, Vancouver, ISO, and other styles

32

Zagoriti, Zoi, Marianthi Georgitsi, Olga Giannakopoulou, Fotios Ntellos, Socrates J. Tzartos, George P. Patrinos, and Konstantinos Poulas. "Genetics of Myasthenia Gravis: A Case-Control Association Study in the Hellenic Population." Clinical and Developmental Immunology 2012 (2012): 1–7. http://dx.doi.org/10.1155/2012/484919.

Full text

Abstract:

Myasthenia gravis (MG) is an heterogeneous autoimmune disease characterized by the production of autoantibodies against proteins of the postsynaptic membrane, in the neuromuscular junction. The contribution of genetic factors to MG susceptibility has been evaluated through family and twin studies however, the precise genetic background of the disease remains elusive. We conducted a case-control association study in 101 unrelated MG patients of Hellenic origin and 101 healthy volunteers in order to assess the involvement of common genetic variants in susceptibility to MG. We focused on three candidate genes which have been clearly associated with several autoimmune diseases, aiming to investigate their potential implication in MG pathogenesis. These are interferon regulatory factor 5 (IRF-5), TNFα-induced protein 3 (TNFAIP3), also known as A20, and interleukin-10 (IL-10), key molecules in the regulation of immune function. A statistical trend of association (P=0.068) betweenIL-10promoter single nucleotide polymorphisms (SNPs) and the subgroups of early and late-onset MG patients was revealed. No statistically significant differences were observed in the rest of the variants examined. As far as we are aware, this is the first worldwide attempt to address the possible association betweenIRF-5andTNFAIP3common genetic variants and the genetic basis of MG.

APA, Harvard, Vancouver, ISO, and other styles

33

Ingelfinger, Florian, Sinduya Krishnarajah, Michael Kramer, Sebastian G. Utz, Edoardo Galli, Mirjam Lutz, Pascale Zwicky, et al. "Single-cell profiling of myasthenia gravis identifies a pathogenic T cell signature." Acta Neuropathologica 141, no. 6 (March 28, 2021): 901–15. http://dx.doi.org/10.1007/s00401-021-02299-y.

Full text

Abstract:

AbstractMyasthenia gravis (MG) is an autoimmune disease characterized by impaired neuromuscular signaling due to autoantibodies targeting the acetylcholine receptor. Although its auto-antigens and effector mechanisms are well defined, the cellular and molecular drivers underpinning MG remain elusive. Here, we employed high-dimensional single-cell mass and spectral cytometry of blood and thymus samples from MG patients in combination with supervised and unsupervised machine-learning tools to gain insight into the immune dysregulation underlying MG. By creating a comprehensive immune map, we identified two dysregulated subsets of inflammatory circulating memory T helper (Th) cells. These signature ThCD103 and ThGM cells populated the diseased thymus, were reduced in the blood of MG patients, and were inversely correlated with disease severity. Both signature Th subsets rebounded in the blood of MG patients after surgical thymus removal, indicative of their role as cellular markers of disease activity. Together, this in-depth analysis of the immune landscape of MG provides valuable insight into disease pathogenesis, suggests novel biomarkers and identifies new potential therapeutic targets for treatment.

APA, Harvard, Vancouver, ISO, and other styles

34

Berta, E., P. Confalonieri, O. Simoncini, G. Bernardi, G. Busnach, R. Mantegazza, F. Cornelio, and C. Antozzi. "Removal of antiacetylcholine receptor antibodies by protein-A immunoadsorption in myasthenia gravis." International Journal of Artificial Organs 17, no. 11 (November 1994): 603–8. http://dx.doi.org/10.1177/039139889401701109.

Full text

Abstract:

Myasthenia Gravis is an autoimmune disease in which autoantibodies to the acetylcholine receptor interfere with neuromuscular transmission. Plasma exchange is effective in temporarily relieving the symptoms of the disease, but for repeated use the lack of selectivity and need for replacement fluids (which increases the risk of contracting viral diseases) are important drawbacks. Staphylococcal protein A, a potent ligand for immunoglobulins, that interacts negligibly with other plasma proteins, appears to be an optimal candidate for removing antiacetylcholine receptor antibodies, which are mostly IgG. We treated three patients with severe immunosuppression-resistant myasthenia gravis with protein A immunoadsorption. Neurological impairment significantly improved in all patients. After immunoadsorption of 1.5-2 plasma volumes per session, the mean percentage reductions for serum IgG and specific autoantibodies were 71% and 82% respectively. No major side effects occurred. Protein A immunoadsorption appears to be a safe, efficient and effective alternative to plasmaexchange for selected myasthenic patients requiring prolonged apheresis.

APA, Harvard, Vancouver, ISO, and other styles

35

Jain, Angita, Nadine Norton, Katelyn A. Bruno, Leslie T. Cooper, Paldeep S. Atwal, and DeLisa Fairweather. "Sex Differences, Genetic and Environmental Influences on Dilated Cardiomyopathy." Journal of Clinical Medicine 10, no. 11 (May 25, 2021): 2289. http://dx.doi.org/10.3390/jcm10112289.

Full text

Abstract:

Dilated cardiomyopathy (DCM) is characterized by dilatation of the left ventricle and impaired systolic function and is the second most common cause of heart failure after coronary heart disease. The etiology of DCM is diverse including genetic pathogenic variants, infection, inflammation, autoimmune diseases, exposure to chemicals/toxins as well as endocrine and neuromuscular causes. DCM is inherited in 20–50% of cases where more than 30 genes have been implicated in the development of DCM with pathogenic variants in TTN (Titin) most frequently associated with disease. Even though male sex is a risk factor for heart failure, few studies have examined sex differences in the pathogenesis of DCM. We searched the literature for studies examining idiopathic or familial/genetic DCM that reported data by sex in order to determine the sex ratio of disease. We found 31 studies that reported data by sex for non-genetic DCM with an average overall sex ratio of 2.5:1 male to female and 7 studies for familial/genetic DCM with an overall average sex ratio of 1.7:1 male to female. No manuscripts that we found had more females than males in their studies. We describe basic and clinical research findings that may explain the increase in DCM in males over females based on sex differences in basic physiology and the immune and fibrotic response to damage caused by mutations, infections, chemotherapy agents and autoimmune responses.

APA, Harvard, Vancouver, ISO, and other styles

36

Finnis, Maria F., and Sandeep Jayawant. "Juvenile Myasthenia Gravis: A Paediatric Perspective." Autoimmune Diseases 2011 (2011): 1–7. http://dx.doi.org/10.4061/2011/404101.

Full text

Abstract:

Myasthenia gravis (MG) is an autoimmune disease in which antibodies are directed against the postsynaptic membrane of the neuromuscular junction, resulting in muscle weakness and fatigability. Juvenile myasthenia gravis (JMG) is a rare condition of childhood and has many clinical features that are distinct from adult MG. Prepubertal children in particular have a higher prevalence of isolated ocular symptoms, lower frequency of acetylcholine receptor antibodies, and a higher probability of achieving remission. Diagnosis in young children can be complicated by the need to differentiate from congenital myasthenic syndromes, which do not have an autoimmune basis. Treatment commonly includes anticholinesterases, corticosteroids with or without steroid-sparing agents, and newer immune modulating agents. Plasma exchange and intravenous immunoglobulin (IVIG) are effective in preparation for surgery and in treatment of myasthenic crisis. Thymectomy increases remission rates. Diagnosis and management of children with JMG should take account of their developmental needs, natural history of the condition, and side-effect profiles of treatment options.

APA, Harvard, Vancouver, ISO, and other styles

37

Krisnamurti, Desak Gede Budi, Rani Wardani Hakim, Radiana Dhewayani Antarianto, Siti Farida, Erni Hernawati Purwaningsih, and Jan Sudir Purba. "THE ROLE OF ACALYPHA INDICA LINN. EXTRACT ON HEART RATES WITH MYASTHENIA GRAVIS RAT MODEL." Asian Journal of Pharmaceutical and Clinical Research 11, no. 1 (January 1, 2018): 257. http://dx.doi.org/10.22159/ajpcr.2017.v11i1.18616.

Full text

Abstract:

Objective: Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction (NMJ) caused by antibodies that attack components of the postsynaptic membrane, impair neuromuscular transmission, and lead to weakness and fatigue of skeletal muscle. Acetylcholine is also used as a neurotransmitter in the autonomic nervous system. Striated cardiac muscle can be a target for immune attack manifesting as heart failure, arrhythmia, and sudden death. Involvement of the heart rate (HR) has been claimed and reported, but a causal connection between MG and altered cardiac function has not been found.Methods: For this study of experimental autoimmune MG (EAMG) is used rocuronium, prostigmine, and Acalypha indica (AI) Linn. compared with HR.Results: From the results, the study found that sympathetic activity of HR variability in EAMG injected with rocuronium 10 mg/kg body weight (BW) in 10 min significantly found increasing in measures of short-term variations in HR variability, indicating parasympathetic impairment.Conclusion: We conclude that in MG, cholinergic transmission is affected more diffusely than previously thought. Furthermore, AI was given orally 30 mg/kg BW has an effect similar to the injecting of prostigmine 10 mg/kg BW that can reduce HR. Driven by the fact that the pharmacological treatment of MG is unsatisfied, it needs the therapeutic development for MG using herbal ingredients of AI. This means that the AI compositions containing anti-MG whose composition should be investigated for the next research.

APA, Harvard, Vancouver, ISO, and other styles

38

Krisnamurti, Desak Gede Budi, Rani Wardani Hakim, Radiana Dhewayani Antarianto, Siti Farida, Erni Hernawati Purwaningsih, and Jan Sudir Purba. "THE ROLE OF ACALYPHA INDICA LINN. EXTRACT ON HEART RATES WITH MYASTHENIA GRAVIS RAT MODEL." Asian Journal of Pharmaceutical and Clinical Research 11, no. 1 (January 1, 2018): 257. http://dx.doi.org/10.22159/ajpcr.2018.v11i1.18616.

Full text

Abstract:

Objective: Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction (NMJ) caused by antibodies that attack components of the postsynaptic membrane, impair neuromuscular transmission, and lead to weakness and fatigue of skeletal muscle. Acetylcholine is also used as a neurotransmitter in the autonomic nervous system. Striated cardiac muscle can be a target for immune attack manifesting as heart failure, arrhythmia, and sudden death. Involvement of the heart rate (HR) has been claimed and reported, but a causal connection between MG and altered cardiac function has not been found.Methods: For this study of experimental autoimmune MG (EAMG) is used rocuronium, prostigmine, and Acalypha indica (AI) Linn. compared with HR.Results: From the results, the study found that sympathetic activity of HR variability in EAMG injected with rocuronium 10 mg/kg body weight (BW) in 10 min significantly found increasing in measures of short-term variations in HR variability, indicating parasympathetic impairment.Conclusion: We conclude that in MG, cholinergic transmission is affected more diffusely than previously thought. Furthermore, AI was given orally 30 mg/kg BW has an effect similar to the injecting of prostigmine 10 mg/kg BW that can reduce HR. Driven by the fact that the pharmacological treatment of MG is unsatisfied, it needs the therapeutic development for MG using herbal ingredients of AI. This means that the AI compositions containing anti-MG whose composition should be investigated for the next research.

APA, Harvard, Vancouver, ISO, and other styles

39

Rahman, Rahmathulla S., Moayyad S. Bauthman, Amer M. Alanazi, Naif N. Alsillah, Ziyad M. Alanazi, Mahdi I. Almuhaysin, Ruyuf K. Almutairi, et al. "Guillain–Barré syndrome: pathophysiology, etiology, causes, and treatment." International Journal Of Community Medicine And Public Health 8, no. 7 (June 25, 2021): 3624. http://dx.doi.org/10.18203/2394-6040.ijcmph20212324.

Full text

Abstract:

Guillain–Barré syndrome (GBS) is a polyradiculoneuropathy autoimmune disease that is characterized by significant inflammation that affects the peripheral nervous system in a rapidly progressive pattern that is mainly clinically presented by muscle weakness. The present literature review aims to broadly discuss GBS: etiology, pathophysiology and management in order to gain an understading of the existing studies that are relevant to this literature review. Among the reported antibodies, anti-GM1 and anti-GQ1B have been reported to be responsible for attacking and damaging either the neuromuscular junctions or peripheral nerves. Moreover, it has been found that the anti-GD1a antibodies in patients bind to the neuromuscular junction and also bind to the nodes of Ranvier of the peripheral nerves and the paranodal myelin of the affected nerves. Reports have shown that this disease is identified as special forms of neuropathies that develop in immune-mediated, post-infection sequelae. Furthermore, in another study it was reported that Molecular mimicry has been previously reported to significantly correlate with the development of the disease as it was investigated in animal models. In addition, Campylobacter jejuni, a pathogen that causes gastrointestinal infections has been previously reported to predispose to the development of GBS in humans. However, scientists have found that plasma exchange and intravenous immunoglobulins (IVIG) remain the most significant and efficacious factors in managing the disease. Nevertheless, recent trials have investigated other approaches that are less efficacious and can lead to serious adverse events and complications.

APA, Harvard, Vancouver, ISO, and other styles

40

BR., Dr Usha, Dr Nandhini K, and Dr Chaitra MC. "Seeking spark- ocular myasthenia gravis in a juvenile – an Indian case report." Tropical Journal of Ophthalmology and Otolaryngology 5, no. 7 (September 28, 2020): 190–93. http://dx.doi.org/10.17511/jooo.2020.i07.04.

Full text

Abstract:

Myasthenia gravis (MG) is a rare autoimmune disorder affecting neuromuscular junction by muscleweakness. Myasthenia gravis can be generalized or localized as ocular myasthenia gravis. Casepresentation: We report an 8-year-old boy who presented with 10 days history of drooping of botheyelids and 8 days history of diplopia. Examination revealed bilateral ptosis. A diagnosis of JuvenileOcular Myasthenia gravis was made when symptoms improved with intramuscular Edrophoniumadministration. He was commenced on oral Neostigmine at a dose of 2mg/Kg/ day,4 hourly individed doses and is on regular follow up and had a good response. Conclusion: Ocular Myastheniagravis (OMG) is a rare disease in itself. A high index of suspicion is required in a juvenile as it iseven rarer.

APA, Harvard, Vancouver, ISO, and other styles

41

Poursadeghfard, Maryam, and Sara Azhdari. "Simultaneous Presence of Acetylcholine Receptor and Muscle Specific Tyrosine Kinase Antibodies in Myasthenia Gravis." International Journal of Basic Science in Medicine 4, no. 4 (December 31, 2019): 128–30. http://dx.doi.org/10.34172/ijbsm.2019.01.

Full text

Abstract:

Myasthenia gravis (MG) is known as an autoimmune disorder which affects transmission in neuromuscular junction. The serologic tests used for diagnosis include acetylcholine receptor and muscle specific receptor tyrosine kinase antibodies. Studies often have reported that patients with formal antibody are negative for the latter one. However, very limited studies have reported positive anti-muscle specific receptor tyrosine kinase antibody in a small percentage of patients with acetylcholine receptor antibody. Here, we reported a young woman who was diagnosed with MG and had a rapid and progressive course of the disease. She was seropositive for both acetylcholine receptor and muscle-specific receptor tyrosine kinase antibodies simultaneously. However, she discharged from the hospital with good condition after treatment.

APA, Harvard, Vancouver, ISO, and other styles

42

Altay, Fatma Aybala, Hayat Güven, Gönül Çiçek Şentürk, Sevgi Ferik, İrfan Şencan, and Selçuk Çomoğlu. "Is West Nile Virus infection associated with myasthenia gravis?" Tropical Doctor 47, no. 1 (July 10, 2016): 30–34. http://dx.doi.org/10.1177/0049475516655069.

Full text

Abstract:

Background Myasthenia Gravis (MG) is an autoimmune disease which is characterised by disruption of signal transmission at neuromuscular junction. We aimed to search about a newly reported association between MG and West Nile Virus (WNV) infection. Methods We searched WNV IgG by ELISA in serum samples of 50 available MG patients and 38 controls. Results None of the samples gave positive results for past WNV infection. Conclusion No evidence of past WNV infection was found in our study population of MG patients. This may have been because MG has been showed to be related with neuroinvasive WNV, which none of our study subjects seem to have had based on their stories. New multicentre studies focusing on immunological mechanisms and held with larger groups or especially neuroinvasive disease patients can cast light onto the answer of this question.

APA, Harvard, Vancouver, ISO, and other styles

43

Vellipuram, Cruz-Flores, Chaudhry, Rawla, Maud, Rodriguez, Kassar, Piriyawat, Qureshi, and Khatri. "Comparative Outcomes of Respiratory Failure Associated with Common Neuromuscular Emergencies: Myasthenia Gravis versus Guillain–Barré Syndrome." Medicina 55, no. 7 (July 15, 2019): 375. http://dx.doi.org/10.3390/medicina55070375.

Full text

Abstract:

Background and objectives: Myasthenia gravis (MG) and Guillain–Barré Syndrome (GBS) are autoimmune neuromuscular disorders that may present as neuromuscular emergencies requiring mechanical ventilation and critical care. Comparative outcomes of these disease processes, once severe enough to require mechanical ventilation, are not known. In this study, we compared the patients requiring mechanical ventilation in terms of in-hospital complications, length of stay, disability, and mortality between these two disease entities at a national level. Materials and Methods: Mechanically ventilated patients with primary diagnosis of MG (n = 6684) and GBS (n = 5834) were identified through retrospective analysis of Nationwide Inpatient Sample (NIS) database for the years 2006 to 2014. Results: Even though mechanically ventilated MG patients were older (61.0 ± 19.1 versus 54.9 ± 20.1 years) and presented with more medical comorbidities, they had lower disease severity on admission, as well as lower in-hospital complications sepsis, pneumonia, and urinary tract infections as compared with GBS patients. In the multivariate analysis, after adjusting for confounders including treatment, GBS patients had significantly higher disability (odds ratio (OR) 15.6, 95% confidence interval (CI) 10.9–22.2) and a longer length of stay (OR 3.48, 95% CI 2.22–5.48). There was no significant difference in mortality between the groups (8.45% MG vs. 10.0% GBS, p = 0.16). Conclusion: Mechanically ventilated GBS patients have higher disease severity at admission along with more in-hospital complications, length of stay, and disability compared with MG patients. Potential explanations for these findings include delay in the diagnosis, poor response to immunotherapy particularly in patients with axonal GBS variant, or longer recovery time after nerve damage.

APA, Harvard, Vancouver, ISO, and other styles

44

Bain, Jennifer M., Codi-Ann Dyer, Megan Galvin, Sylvie Goldman, Jay Selman, Wendy G. Silver, and Sarah E. Tom. "How Providers in Child Neurology Transitioned to Telehealth During COVID-19 Pandemic." Child Neurology Open 8 (January 1, 2021): 2329048X2110229. http://dx.doi.org/10.1177/2329048x211022976.

Full text

Abstract:

To understand child neurology care practices in telehealth (TH), we conducted an online survey interested in identifying which patients should be triaged for in-person evaluations in lieu of telehealth management. We also sought to identify provider and patient/parent limitations of the TH experience. One hundred fourteen clinicians completed the online survey. The majority of child neurologists transitioned within 3 weeks of the pandemic onset and found it inappropriate to evaluate a child under 1 year of age via TH. We identified specific disorders considered inappropriate for initial evaluation via TH, including neuromuscular disease, neuropathy, weakness, autoimmune disease and autism spectrum disorders. Patient and parent technical and economic issues are significant limitations of TH. We suggest quality improvement measures to provide additional training, focusing on particular disorders and increased access for those patients currently excluded from or limited in using or accessing TH.

APA, Harvard, Vancouver, ISO, and other styles

45

Spear, Estelle T., and Gary M. Mawe. "Enteric neuroplasticity and dysmotility in inflammatory disease: key players and possible therapeutic targets." American Journal of Physiology-Gastrointestinal and Liver Physiology 317, no. 6 (December 1, 2019): G853—G861. http://dx.doi.org/10.1152/ajpgi.00206.2019.

Full text

Abstract:

Intestinal functions, including motility and secretion, are locally controlled by enteric neural networks housed within the wall of the gut. The fidelity of these functions depends on the precision of intercellular signaling among cellular elements, including enteric neurons, epithelial cells, immune cells, and glia, all of which are vulnerable to disruptive influences during inflammatory events. This review article describes current knowledge regarding inflammation-induced neuroplasticity along key elements of enteric neural circuits, what is known about the causes of these changes, and possible therapeutic targets for protecting and/or repairing the integrity of intrinsic enteric neurotransmission. Changes that have been detected in response to inflammation include increased epithelial serotonin availability, hyperexcitability of intrinsic primary afferent neurons, facilitation of synaptic activity among enteric neurons, and attenuated purinergic neuromuscular transmission. Dysfunctional propulsive motility has been detected in models of colitis, where causes include the changes described above, and in models of multiple sclerosis and other autoimmune conditions, where autoantibodies are thought to mediate dysmotility. Other cells implicated in inflammation-induced neuroplasticity include muscularis macrophages and enteric glia. Targeted treatments that are discussed include 5-hydroxytryptamine receptor 4 agonists, cyclooxygenase inhibitors, antioxidants, B cell depletion therapy, and activation of anti-inflammatory pathways.

APA, Harvard, Vancouver, ISO, and other styles

46

Rasool, Suhail, Vinay Goyal, Mohd Irshad, and Bansi Lal Jailkhani. "ALPHA AND BETA BUNGAROTOXIN BINDING PROTEINS IN HUMAN CADAVER MUSCLE." Journal of Musculoskeletal Research 17, no. 01 (March 2014): 1450005. http://dx.doi.org/10.1142/s0218957714500055.

Full text

Abstract:

The nicotinic acetylcholine receptors (nAChR) in skeletal muscle, which are targets of the autoimmune response that cause myasthenia gravis (MG). MG is an autoimmune disease caused by anti-acetylcholine (anti-AChR) antibodies, resulting in functional loss of AChR at the neuromuscular junction. Specific binding of radio-iodinated α and β Bgtx was carried out in membranes (rapid filtration method) and triton extract (ammonium sulfate precipitation method) of fresh muscle. The specific binding of α Bgtx to membranes and triton extract of fresh muscle was found to be 19.8 ± 0.2 and 10.2 ± 0.12 fmoles/mg tissue, respectively whereas the same for β-Bgtx was found to be 10.2 ± 0.15 and 7.2 ± 0.12 fmoles/mg tissue, respectively. It was observed that there was no significant decline in specific binding to membranes and triton extract of muscle with increase in post-mortem time from 5 to 24 h. By indirect ELISA with MG pool sera, in addition to age the immunoreactivity varies from one cadaver to another. It was observed that β bungarotoxin binding proteins in muscle shows high immunoreactivity as compared to α Bungarotoxin binding protein.

APA, Harvard, Vancouver, ISO, and other styles

47

Cortez Grippe, Talyta, Ana Carolina Da Bouza Ferreira, Ana Carolina Aguilar, André Gustavo Fonseca Ferreira, Manoel Wilkley Gomes Sousa, Ronaldo Maciel Dias, Rubens NelsonMorato Fernandez, Elza Dias Tosta, and Gustavo Fonseca Ferreira. "Case report." Revista Saúde e Inovação 1, no. 1 (December 1, 2020): 1–6. http://dx.doi.org/10.51208/journalofhi.v1i1.11.

Full text

Abstract:

Myasthenia gravis (MG) is a rare autoimmune disease in which antibodies bind to acetylcholine receptors in the postsynaptic membrane at the neuromuscular junction. Muscle-specific kinase (MuSK) antibody-associated MG patients often have severe symptoms, including bulbar dysfunction, respiratory insufficiency, and atrophy of the facial and tongue muscles. Due to its fluctuating nature and the similarity to the symptoms other disorders MG is one of the most challenging medical diagnoses.Fluctuating character and the similarity of symptoms to those of other disorders make MG one of the most challenging medical diagnoses. Initial misdiagnosis of MuSK-MG can lead to worsening of symptoms. The diagnosis is confirmed by positive results on pharmacological testing, electrodiagnostictesting and serum antibodyassay. Symptomatic, immunoactive, and supportive approaches to therapy have very good effect and the prognosis is improved with precocious interventions.

APA, Harvard, Vancouver, ISO, and other styles

48

Cortez Grippe, Talyta, Ana Carolina Da Bouza Ferreira, Ana Carolina Aguilar, André Gustavo Fonseca Ferreira, Manoel Wilkley Gomes Sousa, Ronaldo Maciel Dias, Rubens NelsonMorato Fernandez, Elza Dias Tosta, and Gustavo Fonseca Ferreira. "Case report." Revista Saúde e Inovação 1, no. 1 (December 10, 2020): 1–6. http://dx.doi.org/10.51208/saudeinovacao.v1i1.11.

Full text

Abstract:

Myasthenia gravis (MG) is a rare autoimmune disease in which antibodies bind to acetylcholine receptors in the postsynaptic membrane at the neuromuscular junction. Muscle-specific kinase (MuSK) antibody-associated MG patients often have severe symptoms, including bulbar dysfunction, respiratory insufficiency, and atrophy of the facial and tongue muscles. Due to its fluctuating nature and the similarity to the symptoms other disorders MG is one of the most challenging medical diagnoses.Fluctuating character and the similarity of symptoms to those of other disorders make MG one of the most challenging medical diagnoses. Initial misdiagnosis of MuSK-MG can lead to worsening of symptoms. The diagnosis is confirmed by positive results on pharmacological testing, electrodiagnostictesting and serum antibodyassay. Symptomatic, immunoactive, and supportive approaches to therapy have very good effect and the prognosis is improved with precocious interventions.

APA, Harvard, Vancouver, ISO, and other styles

49

Qashqari, HF, I. Narang, H. Katzberg, K. Vezina, A. Khayat, N. Chrestian, and J. Vajsar. "P.069 Respiratory dysfunction and sleep disordered breathing in children with Myasthenia Gravis." Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 45, s2 (June 2018): S34. http://dx.doi.org/10.1017/cjn.2018.171.

Full text

Abstract:

Background: Myasthenia Gravis ( MG) is an autoimmune disease that affects the neuromuscular junction. It typically presents with fluctuating muscle weakness which can affect respiratory muscles. Data about the prevalence of sleep disordered breathing in children with MG and the benefits of non-invasive ventilation outside the setting of MG crisis has not been studied so far. Methods: Eleven children between 3 and 18 years old with confirmed MG were recruited from the The Hospital for Sick Children Neuromuscular clinic in a prospective observational study. Informed consent was obtained and patients underwent PFTs, MIP/MEP, SNIP, FVC and standard polysomnography testing’s. Results: In our study, we found that 2/11 children had abnormal Apnea Hypopnea index (AHI) and were diagnosed with obstructive sleep apnea (OSA). One of them has juvenile ocular MG with mild to moderate OSA and the second child has congenital MG with mild OSA. CPAP therapy was initiated for both patients. Conclusions: In our cohort, obstructive sleep apnea rate was significantly higher in children with MG than the known prevalence in general pediatric population ( 18% vs 2-3% ). Early diagnosis and management of OSA can have great impact on children’s health and quality of life. A larger study is needed to validate our findings.

APA, Harvard, Vancouver, ISO, and other styles

50

Bertani, Helga, Alessandro Messerotti, Fabrizio Di Benedetto, Raffaele Manta, Milena Greco, Federica Casoni, Luisa Losi, and Rita Conigliaro. "Unusual Paraneoplastic Syndrome Accompanies Neuroendocrine Tumours of the Pancreas." Case Reports in Medicine 2011 (2011): 1–4. http://dx.doi.org/10.1155/2011/309149.

Full text

Abstract:

Neuroendocrine tumours comprise a small percentage of pancreatic neoplasia (10%) (1). Diagnosis of neuroendocrine tumours is difficult, especially if the tumours are small and nonfunctional. CT scans, MRI, and nuclear scans are sufficiently sensitive assessment tools for tumours with diameters of at least 2 cm; otherwise, the sensitivity and specificity of these techniques is less than 50% (2). Myasthenia gravis (MG) is a heterogeneous neuromuscular junction disorder that is primarily caused when antibodies form against the acetylcholine receptors (Ab-AchR). MG can develop in conjunction with neoplasia, making MG a paraneoplastic disease. In those cases, MG is most commonly associated with thymomas and less frequently associated with extrathymic malignancies. The mechanism underlying this paraneoplastic syndrome has been hypothesized to involve an autoimmune response against the tumour cells (3). No published reports have linked malignant pancreatic diseases with MG. Here, we report the case of a young woman, negative for Ab-AchR, with a neuroendocrine tumour in the pancreatic head, who experienced a complete resolution of her MG-like syndrome after surgical enucleation of the tumour.

APA, Harvard, Vancouver, ISO, and other styles

Journal articles on the topic 'Neuromuscular autoimmune disease'

Create a spot-on reference in APA, MLA, Chicago, Harvard, and other styles