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1
Sweeney, Michael. "Autoimmune Neurologic Diseases in Children." Seminars in Neurology 38, no. 03 (June 2018): 355–70. http://dx.doi.org/10.1055/s-0038-1660520.
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AbstractAutoimmune diseases of the nervous system in children are composed of a heterogeneous group of rare disorders that can affect the central or peripheral nervous system at any level. Presentations may occur in children of any age and are typically acute or subacute in onset. Consideration of an autoimmune process as the etiology of neurologic diseases in children is important, as it may lead to early initiation of immunotherapy and an improvement in long-term neurologic outcomes. The developing nervous and immune systems in children create unique challenges in diagnosis and treatment of these rare diseases. In this review, autoimmune diseases affecting the brain, spinal cord, nerve roots, peripheral nerves, neuromuscular junction, and muscle in children are described.
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Zhang, Tan, Xin Feng, Bo Feng, Juan Dong, Karen Haas, Barbara M. Nicklas, Osvaldo Delbono, and Stephen Kritchevsky. "CARDIAC TROPONIN T MEDIATED AUTOIMMUNE RESPONSE AND ITS ROLE IN SKELETAL MUSCLE AGING." Innovation in Aging 3, Supplement_1 (November 2019): S882. http://dx.doi.org/10.1093/geroni/igz038.3231.
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Abstract Cardiac troponin T (cTnT), a key component of contractile machinery essential for muscle contraction, is also expressed in skeletal muscle under certain conditions (e.g. neuromuscular diseases and aging). We have reported that skeletal muscle cTnT regulates neuromuscular junction denervation preferentially in fast skeletal muscle of old mice. Here, we further report that cTnT is also enriched within some myofibers, and/or along microvascular walls in old mice fast skeletal muscle. Strikingly, immunoglobulin G (IgG), together with markers of complement system activation, cell death (necroptosis or apoptosis), and macrophage infiltration, were all found to be co-localized with cTnT and IgG in those areas. In addition, elevated cTnT and IgG are associated with lower dystrophin expression on muscle fiber membrane, lower muscle capillary density, and reduced muscle performance (wire hanging test). Using purified recombinant TnT proteins, we confirmed that only cTnT, but not slow or fast skeletal muscle TnT1 or TnT3, was detected by immunoblot using sera from old (but not young) mice with pre-determined elevated cTnT and IgG in their skeletal muscle, indicating the existence of anti-cTnT autoantibodies in sera (previously found in human blood) and skeletal muscle of old mice. Immunoblotting further revealed that the age related changes in skeletaI muscle cTnT and IgG are more prominent in fast skeletal muscle than in slow. Importantly, elevated cTnT and IgG were also detected in skeletal muscles from 4 older adults (65-70 yrs, IMFIT). Our finding suggests a novel autoimmune mechanism mediated by cTnT that underlies age related skeletal muscle abnormalities and dysfunction.
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Mathis, Stéphane, Laurent Magy, Philippe Corcia, Karima Ghorab, Laurence Richard, Jonathan Ciron, Mathilde Duchesne, and Jean-Michel Vallat. "Simultaneous Combined Myositis, Inflammatory Polyneuropathy, and Overlap Myasthenic Syndrome." Case Reports in Neurological Medicine 2016 (2016): 1–11. http://dx.doi.org/10.1155/2016/6108234.
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Immune-mediated neuromuscular disorders include pathologies of the peripheral nervous system, neuromuscular junction, and muscles. If overlap syndromes (or the association of almost two autoimmune disorders) are recognized, the simultaneous occurrence of several autoimmune neuromuscular disorders is rare. We describe two patients presenting the simultaneous occurrence of inflammatory neuropathy, myositis, and myasthenia gravis (with positive acetylcholine receptor antibodies). For each patient, we carried out a pathological analysis (nerve and muscle) and an electrophysiological study (and follow-up). To our knowledge, this is the first description of such a triple immune-mediated neuromuscular syndrome. We compared our observations with a few other cases of simultaneous diagnosis of two inflammatory neuromuscular disorders.
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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.
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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.
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Amaya-Amaya, Jenny, Laura Montoya-Sánchez, and Adriana Rojas-Villarraga. "Cardiovascular Involvement in Autoimmune Diseases." BioMed Research International 2014 (2014): 1–31. http://dx.doi.org/10.1155/2014/367359.
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Autoimmune diseases (AD) represent a broad spectrum of chronic conditions that may afflict specific target organs or multiple systems with a significant burden on quality of life. These conditions have common mechanisms including genetic and epigenetics factors, gender disparity, environmental triggers, pathophysiological abnormalities, and certain subphenotypes. Atherosclerosis (AT) was once considered to be a degenerative disease that was an inevitable consequence of aging. However, research in the last three decades has shown that AT is not degenerative or inevitable. It is an autoimmune-inflammatory disease associated with infectious and inflammatory factors characterized by lipoprotein metabolism alteration that leads to immune system activation with the consequent proliferation of smooth muscle cells, narrowing arteries, and atheroma formation. Both humoral and cellular immune mechanisms have been proposed to participate in the onset and progression of AT. Several risk factors, known as classic risk factors, have been described. Interestingly, the excessive cardiovascular events observed in patients with ADs are not fully explained by these factors. Several novel risk factors contribute to the development of premature vascular damage. In this review, we discuss our current understanding of how traditional and nontraditional risk factors contribute to pathogenesis of CVD in AD.
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Troshina, Ekaterina A., Elena A. Panfilova, and Taras S. Panevin. "Autoimmune polyglandular disorders in myotonic dystrophy." Problems of Endocrinology 65, no. 3 (September 12, 2019): 155–60. http://dx.doi.org/10.14341/probl9775.
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Myotonic dystrophy (MD) is the most common muscle disorder in adults. MD is a hereditary disease with an autosomal dominant mode of inheritance, almost 100% penetrance and pronounced clinical polymorphism. The mechanism for the development of the disease is that a mutation of the DMPK (dystrophia myotonica protein kinase) gene disrupts the normal metabolism of RNA, which leads to a defect in the maturation and translation of mRNA. The disorder in the DMPK gene affects not only striated musculature, but also smooth myocytes and cardiomyocytes. The main clinical symptom that distinguishes MD from others is a spontaneous or provoked inability to relax muscles (myotonia phenomenon). Endocrine disorders arising from type 1 MD (MD1) with a higher than average frequency in the population include hypergonadotropic hypogonadism, impaired glucose tolerance with hyperinsulinism, and insulin resistance. Thyroid function may remain normal, although many cases of autoimmune thyroiditis resulting in hypothyroidism, as well as Graves’ disease, have been described. A description is given of a patient suffering from MD1 with a number of endocrine disorders, including hypergonadotropic hypogonadism, autoimmune thyroid disease, hyperinsulinism, and also impaired calcium-phosphorus metabolism. Important features are the absence of any significant complaints from the muscular system in the presence of an increase in creatine phosphokinase (CPK), which is characteristic of this disease, as well as the temporal dynamics of thyroid status and the nature of the autoimmune thyroid disease.
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Shidlovskyi, V. O., O. V. Shidlovskyi, and V. V. Kravtsiv. "The effect of autoimmune thyroiditis on the organs and systems of the body (a literature review)." INTERNATIONAL JOURNAL OF ENDOCRINOLOGY (Ukraine) 17, no. 2 (May 11, 2021): 145–54. http://dx.doi.org/10.22141/2224-0721.17.2.2021.230569.
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Background. In recent years, scientific reports on the effects of autoimmune thyroiditis on the body have been published. They concern separate organs and systems that does not allow receiving the general picture of pathological reactions of the body to autoimmune aggression. The review analyzes the literature sources about the pathological significance of autoimmune thyroiditis for the body as a whole. Sources of information. The sources of information were reports in domestic and, mainly, foreign periodicals on general medicine, pathophysiology, immunology, and endocrinology. Synthesis of evidence. The effect of autoimmune thyroiditis and hypothyroidism on the function of body systems is considered from the standpoint of the interaction of both autoimmune thyroiditis on organs and systems and body systems on the thyroid gland, in particular on the development of its autoimmune pathology. In general, autoimmune thyroiditis and its consequence — hypothyroidism affect all organs and systems of the body without exception by reducing the level of metabolic processes and the accumulation of glucosaminoglycans, mainly glucuronic acid in the tissues of the body. As a result, interstitial edema develops, mainly in muscle tissue, including the muscular layer of the hollow organs of the digestive tract. The clinical symptoms of such lesions depend on the disease severity and the depth of metabolic disorders and have significant individual differences. They concern to all the systems and organs of the body. The effects on the cardiovascular and reproductive systems, musculoskeletal system, gastrointestinal tract are the most significant for health. Conclusions. The mentioned data expand knowledge and create a holistic view of autoimmune thyroiditis not as a local autoimmune disease of the thyroid gland but as a local manifestation of general autoimmune disease of the body.
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Pejin, Radoslav, Edita Stokic, Mile Novkovic, Sofija Banic-Horvat, and Milan Cvijanovic. "Autoimmune polyglandular syndrome, type 2 associated with myasthenia gravis." Vojnosanitetski pregled 69, no. 4 (2012): 358–62. http://dx.doi.org/10.2298/vsp1204358p.
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Introduction. Autoimmune polyglandular syndrome type 2 is defined as adrenal insufficiency associated with autoimmune primary hypothyroidism and/or with autoimmune type 1 diabetes mellitus, but very rare with myasthenia gravis. Case report. We presented a case of an autoimmune polyglandular syndrome, type 2 associated with myasthenia gravis. A 49-year-old female with symptoms of muscle weakness and low serum levels of cortisol and aldosterone was already diagnosed with primary adrenal insufficiency. Primary hypothyroidism was identified with low values of free thyroxine 4 (FT4) and raised values of thyroidstumulating hormone (TSH). The immune system as a cause of hypothyroidism was confirmed by the presence of thyroid antibodies to peroxidase and TSH receptors. Myasthenia gravis was diagnosed on the basis of a typical clinical feature, positive diagnostic tests and an increased titre of antibodies against the acetylcholine receptors. It was not possible to confirm the immune nature of adrenal insufficiency by the presence of antibodies to 21- hydroxylase. The normal morphological finding of the adrenal glands was an indirect confirmation of the condition as well as the absence of other diseases that might have led to adrenal insufficiency and low levels of both serum cortisol and aldosterone. Hormone replacement therapy, anticholinergic therapy and corticosteroid therapy for myasthenia gravis improved the patient?s general state of health and muscle weakness. Conclusion. This case report indicates a need to examine each patient with an autoimmune disease carefully as this condition may be associated with another autoimmune diseases.
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Hristov, K. "OBESITY, INFLAMMATION, AND T-CELL METABOLISM." Trakia Journal of Sciences 17, no. 4 (2019): 392–98. http://dx.doi.org/10.15547/tjs.2019.04.017.
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Aim: The overview of the interdependence of the immune system and the system metabolism. Regulation of metabolism is immunomodulatory, and targeting key cellular metabolic enzymes impacts T-cell development, altering the immune functions. Background: The diet, gastrointestinal microbiota and the balanced function of liver, adipose and muscle tissues underlie the immune ecology. Chronic inflammation (macrophage, TH1, and TH17 T-cell infiltration) associates with obesity, and the development of metabolic syndrome, cardiovascular diseases, type 2 diabetes, IBD and intestinal malignancies. While naive T-cells use beta-oxidation, TCA cycle, and mitochondrial respiration to produce ATP, activated T-cells, similarly to cancer cells, employ the Warburg’s effect to power their function. The development of T-cells depends on key metabolic regulators, like mTORC1 (TH1 and TH17 T-cells) and mTORC2 (TH2 T-cells). Inhibition of HIF1-alpha (critical for TH17 T-cells) results in the development of FOXP3+ Treg T-cells, improving autoimmune disorders. Metabolic flexibility of normal cells underlines the successful treatment of neoplastic, autoimmune and hyper-sensitivity disorders. Conclusions: The immune system influences the system metabolism, and depends on the function of adipose tissue, muscles, liver, pancreas, lungs and gastro-intestinal tract. Diet and pharmacological regulation of T-cell metabolic activity influence immune function during autoimmunity, infections, and vaccinations.
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Váncsa, Andrea, and Katalin Dankó. "Újabb adatok a myositisspecifikus és -asszociált antitestekről juvenilis és felnőttkori myositisekben." Orvosi Hetilap 157, no. 30 (July 2016): 1179–84. http://dx.doi.org/10.1556/650.2016.30404.
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Myositis, which means inflammation of the muscles, is a general term used for inflammatory myopathies. Myositis is a rare idiopathic autoimmune disease. It is believed that environmental factors such as virus, bacteria, parasites, direct injuries, drugs side effect can trigger the immune system of genetically susceptible individuals to act against muscle tissues. There are several types of myositis with the same systemic symptoms such as muscle weakness, fatigue, muscle pain and inflammation. These include dermatomyositis, juvenile dermatomyositis, inclusion-body myositis, polymyositis, orbital myositis and myositis ossificans. Juvenile and adult dermatomyositis are chronic, immune-mediated inflammatory myopathies characterized by progressive proximal muscle weakness and typical skin symptoms. The aim of the authors was to compare the symptoms, laboratory and serological findings and disease course in children and adult patients with idiopathic inflammatory myopathy. Early diagnosis and aggressive immunosuppressive treatment improve the mortality of these patients. Myositis-specific autoantibodies have predictive and prognostic values regarding the associated overlap disease, response to treatment and disease course. The authors intend to lighten the clinical and pathogenetic significance of the new target autoantigens. Orv. Hetil., 2016, 157(29), 1179–1184.
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Furmaniak, J., J. Bradbury, and B. Rees Smith. "Antibodies to membrane antigens in autoimmune thyroid disease." Acta Endocrinologica 116, no. 1 (September 1987): 13–20. http://dx.doi.org/10.1530/acta.0.1160013.
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Abstract. The possibility that sera from patients with autoimmune thyroid diseases contain autoantibodies to thyroid membrane proteins distinct from microsomal antigen and the TSH receptor has been investigated using (a) solid phase assay system based on human thyroid membranes and 125I-labelled protein A and (b) immunoprecipitation of detergent solubilized 125I-labelled thyroid membranes followed by gel electrophoresis and autoradiography. In the solid phase assay binding to membranes showed a highly significant correlation with binding to microsomes (r = 0.82; P < 0.001; N = 82) indicating that the interaction between the serum and the membranes was due principally to microsomal antibody binding to microsomal antigen contaminating the membrane preparations. However, there were some discrepancies suggesting that an additional antigen-antibody system was involved. This possibility was then investigated using immunoprecipitation of 125I-labelled thyroid membranes. A labelled protein with mol wt 54 K was specifically immunoprecipitated (relative to normal pool serum) by 3 out of 4 sera from patients with Graves' disease who showed high binding to thyroid membranes. A further 4 sera from such patients with low membrane binding affinity failed to immunoprecipitate the 54 K protein. Sera from some patients with Hashimoto's disease and some patients with rheumatoid arthritis and one patient with Addison's disease also immunoprecipitated the 54 K protein from solubilized thyroid membranes. These studies suggested that antibodies interacting with the 54 K protein contributed to the discrepancies between thyroid membrane and microsome binding. However, the 54 K protein was also immunoprecipitated from detergent solubilized membranes prepared from human placenta, skeletal muscle and adrenal tissue. Immunoprecipitation studies with antisera to cytoskeleton proteins suggested that the 54 K band was the intermediate filament protein desmin. Consequently, thyroid specific antibody-antigen systems distinct from those involving microsomal antibody (or thyroglobulin antibody) could not be detected in thyroid membranes by immunoprecipitation.
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Plastiras, Sotiris C., and Haralampos M. Moutsopoulos. "Arrhythmias and Conduction Disturbances in Autoimmune Rheumatic Disorders." Arrhythmia & Electrophysiology Review 10, no. 1 (April 12, 2021): 17–25. http://dx.doi.org/10.15420/aer.2020.43.
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Rhythm and conduction disturbances and sudden cardiac death are important manifestations of cardiac involvement in autoimmune rheumatic diseases (ARD), which have a serious impact on morbidity and mortality. While the underlying arrhythmogenic mechanisms are multifactorial, myocardial fibrosis plays a pivotal role. It accounts for a substantial portion of cardiac mortality and may manifest as atrial and ventricular arrhythmias, conduction system abnormalities, biventricular cardiac failure or sudden death. In patients with ARD, myocardial fibrosis is considered to be the hallmark of cardiac involvement as a result of inflammatory process or to coronary artery occlusive disease. Myocardial fibrosis constitutes the pathological substrates for reentrant circuits. The presence of supraventricular extra systoles, tachyarrhythmias, ventricular activity and conduction disturbances are not uncommon in patients with ARDs, more often in systemic lupus erythematosus, systemic sclerosis, rheumatoid arthritis, inflammatory muscle disorders and anti-neutrophil cytoplasm antibody-associated vasculitis. In this review, the type, the relative prevalence and the underlying mechanisms of rhythm and conduction disturbances in the emerging field of cardiorheumatology are provided.
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Milanick, Mark, Kerri Graham, and Melissa Wessel. "Why Is That Dog Paralyzed? A Problem-Based Case & Laboratory Exercise about Neuromuscular Transmission." American Biology Teacher 75, no. 1 (January 1, 2013): 36–39. http://dx.doi.org/10.1525/abt.2013.75.1.8.
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Students are provided with a mystery concerning dogs that are paralyzed. This motivates a laboratory exercise to measure parameters from the dog’s “blood” to determine whether the paralysis is due to pesticide poisoning or an autoimmune attack on nerve myelin. Most of the materials are available from the grocery store. The real-world nature of the problem, and the mystery, engages the students in thinking about nerve, muscle, and immune system function. Alternative versions require less familiarity with physiology and can be used as engagement activities to encourage learning laboratory skills and experimental design or as motivation for learning nerve and muscle physiology.
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Carranza-León, D. A., A. Oeser, A. Marton, P. Wang, J. C. Gore, J. Titze, C. M. Stein, C. P. Chung, and M. J. Ormseth. "Tissue sodium content in patients with systemic lupus erythematosus: association with disease activity and markers of inflammation." Lupus 29, no. 5 (February 18, 2020): 455–62. http://dx.doi.org/10.1177/0961203320908934.
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Objectives Sodium (Na+) is stored in the skin and muscle and plays an important role in immune regulation. In animal models, increased tissue Na+ is associated with activation of the immune system, and high salt intake exacerbates autoimmune disease and worsens hypertension. However, there is no information about tissue Na+ and human autoimmune disease. We hypothesized that muscle and skin Na+ content is (a) higher in patients with systemic lupus erythematosus (SLE) than in control subjects, and (b) associated with blood pressure, disease activity, and inflammation markers (interleukin (IL)-6, IL-10 and IL-17 A) in SLE. Methods Lower-leg skin and muscle Na+ content was measured in 23 patients with SLE and in 28 control subjects using 23Na+ magnetic resonance imaging. Demographic and clinical information was collected from interviews and chart review, and blood pressure was measured. Disease activity was assessed using the SLE Disease Activity Index (SLEDAI). Plasma inflammation markers were measured by multiplex immunoassay. Results Muscle Na+ content was higher in patients with SLE (18.8 (16.7–18.3) mmol/L) than in control subjects (15.8 (14.7–18.3) mmol/L; p < 0.001). Skin Na+ content was also higher in SLE patients than in controls, but this difference was not statistically significant. Among patients with SLE, muscle Na+ was associated with SLEDAI and higher concentrations of IL-10 after adjusting for age, race, and sex. Skin Na+ was significantly associated with systolic blood pressure, but this was attenuated after covariate adjustment. Conclusion Patients with SLE had higher muscle Na+ content than control subjects. In patients with SLE, higher muscle Na+ content was associated with higher disease activity and IL-10 concentrations.
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Bondar', Irina Arkad'evna, and Vadim Valer'evich Klimontov. "The role of CD40 receptor-ligand system in the development of diabetes mellitus and its complications." Diabetes mellitus 14, no. 3 (September 15, 2011): 21–25. http://dx.doi.org/10.14341/2072-0351-6219.
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The CD40 receptor-CD40 ligand system, along with other signal molecules, plays an important role in the development of immune and inflammatoryreactions. CD40R and CD40L are expressed in lymphocytes, monocytes, platelets, endothelial and smooth muscle cells, pancreatic beta-cells, adipocytes,and other cells. The CD40-CD40L system participates in the formation of immune and inflammatory reactions in the cardiovascular system,platelet production and development of autoimmune diseases. Signals mediated through CD40 receptors are involved in the autoimmune process intype 1 diabetes, inflammation of adipose tissue in DM2, development of atherosclerosis and diabetic nephropathy. Therefore, the CD40-CD40Lsystem can be regarded as a universal pathogenetic factor responsible for immune and inflammatory processes, hyperglycemia, and vascular complications.Measurement of CD40 expression in lymphocytes, monocytes, and adipocytes and sCD40L in blood and urine can be used to diagnose andforetell immune and inflammatory processes associated with diabetes mellitus. HMG-CoA reductase inhibitors (statins) and disaggregants (aspirin,clopidogrel) inhibit activity of the CD40-CD40L system. Further studies of possibilities of pharmacological correction of this system may provide abasis for the development of new therapeutic modalities for the management of diabetes and its complications.
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Musumeci, Giuseppe, Paola Castrogiovanni, Ignazio Barbagallo, Daniele Tibullo, Cristina Sanfilippo, Giuseppe Nunnari, Giovanni Pellicanò, et al. "Expression of the OAS Gene Family Is Highly Modulated in Subjects Affected by Juvenile Dermatomyositis, Resembling an Immune Response to a dsRNA Virus Infection." International Journal of Molecular Sciences 19, no. 9 (September 17, 2018): 2786. http://dx.doi.org/10.3390/ijms19092786.
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Background: Juvenile dermatomyositis (JDM) is a systemic, autoimmune, interferon (IFN)-mediated inflammatory muscle disorder that affects children younger than 18 years of age. JDM primarily affects the skin and the skeletal muscles. Interestingly, the role of viral infections has been hypothesized. Mammalian 2′-5′-oligoadenylate synthetase (OAS) genes have been thoroughly characterized as components of the IFN-induced antiviral system, and they are connected to several innate immune-activated diseases. The main purpose of the paper is to define the potential interrelationship between the OAS gene family network and the molecular events that characterize JDM along with double-stranded RNA (dsRNA) molecular pathways. Methods: We analyzed three microarray datasets obtained from the NCBI in order to verify the expression levels of the OAS gene family network in muscle biopsies (MBx) of JDM patients compared to healthy controls. Furthermore, From GSE51392, we decided to select significant gene expression profiles of primary nasal and bronchial epithelial cells isolated from healthy subjects and treated with polyinosinic-polycytidylic acid (poly(I:C)), a synthetic analog of double-stranded RNA (dsRNA), a molecular pattern associated with viral infection. Results: The analysis showed that all OAS genes were modulated in JDM muscle biopsies. Furthermore, 99% of OASs gene family networks were significantly upregulated. Of importance, 39.9% of modulated genes in JDM overlapped with those of primary epithelial cells treated with poly(I:C). Moreover, the microarray analysis showed that the double-stranded dsRNA virus gene network was highly expressed. In addition, we showed that the innate/adaptive immunity markers were significantly expressed in JDM muscles biopsies. and that their levels were positively correlated to OAS gene family expression. Conclusion: OAS gene expression is extremely modulated in JDM as well as in the dsRNA viral gene network. These data lead us to speculate on the potential involvement of a viral infection as a trigger moment for this systemic autoimmune disease. Further in vitro and translational studies are needed to verify this hypothesis in order to strategically plan treatment interventions.
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Ng, Xiulin, Mona Sadeghian, Simon Heales, and Iain P. Hargreaves. "Assessment of Mitochondrial Dysfunction in Experimental Autoimmune Encephalomyelitis (EAE) Models of Multiple Sclerosis." International Journal of Molecular Sciences 20, no. 20 (October 9, 2019): 4975. http://dx.doi.org/10.3390/ijms20204975.
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Multiple sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system (CNS) that involves the autoreactive T-cell attack on axonal myelin sheath. Lesions or plaques formed as a result of repeated damage and repair mechanisms lead to impaired relay of electrical impulses along the nerve, manifesting as clinical symptoms of MS. Evidence from studies in experimental autoimmune encephalomyelitis (EAE) models of MS strongly suggests that mitochondrial dysfunction presents at the onset of disease and throughout the disease course. The aim of this study was to determine if mitochondrial dysfunction occurs before clinical symptoms arise, and whether this is confined to the CNS. EAE was induced in C57B/L6 mice, and citrate synthase and mitochondrial respiratory chain (MRC) complex I–IV activities were assayed at presymptomatic (3 or 10 days post first immunisation (3 or 10 DPI)) and asymptomatic (17 days post first immunisation (17 DPI) time-points in central nervous system (CNS; spinal cord) and peripheral (liver and jaw muscle) tissues. Samples from animals immunised with myelin oligodendrocyte glycoprotein (MOG) as EAE models were compared with control animals immunised with adjuvant (ADJ) only. Significant changes in MOG compared to control ADJ animals in MRC complex I activity occurred only at presymptomatic stages, with an increase in the spinal cord at 10 DPI (87.9%), an increase at 3 DPI (25.6%) and decrease at 10 DPI (22.3%) in the jaw muscle, and an increase in the liver at 10 DPI (71.5%). MRC complex II/III activity changes occurred at presymptomatic and the asymptomatic stages of the disease, with a decrease occurring in the spinal cord at 3 DPI (87.6%) and an increase at 17 DPI (36.7%), increase in the jaw muscle at 10 DPI (25.4%), and an increase at 3 DPI (75.2%) and decrease at 17 DPI (95.7%) in the liver. Citrate synthase activity was also significantly decreased at 10 DPI (27.3%) in the liver. No significant changes were observed in complex IV across all three tissues assayed. Our findings reveal evidence that mitochondrial dysfunction is present at the asymptomatic stages in the EAE model of MS, and that the changes in MRC enzyme activities are tissue-specific and are not confined to the CNS.
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Knezevic-Pogancev, Marija. "Juvenile myasthenia." Medical review 64, no. 5-6 (2011): 295–98. http://dx.doi.org/10.2298/mpns1106295k.
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Introduction. Juvenile myasthenia is a chronic autoimmune neuromuscular disease characterized by varying degrees of fluctuating, painless muscle weakness and rapid fatigue of any muscles under voluntary control. Juvenile myasthenia is a form of myasthenia appearing in adolescent age, representing 10% to 15% of all cases of myasthenia gravis. Juvenile myasthenia is presented by a defect in the transmission of nerve impulses to muscles, resulting from a breakdown in the normal communication between nerves and muscles. In myasthenia, antibodies produced by the body?s own immune system block, alter, or destroy the receptors for acetylcholine. Juvenile myasthenia is neither directly inherited nor is it contagious. Signs and Symptoms. The first noticeable symptoms may be eye muscle weakness, difficulty in swallowing, or slurred speech. Juvenile myasthenia usually affects muscles innervated by the cranial nerves (face, lips, tongue, neck and throat), but it can affect any muscle group. Symptoms vary in type and severity with typical periods of exacerbation interspersed with periods of remission. When the muscles necessary for breathing are affected, a patient is said to be in a myasthenic crisis, which is a life-threatening situation. Disease Outcome and Treatment. Juvenile myasthenia produces sporadic but progressive weakness and abnormal fatigability of striated (skeletal) muscles, exacerbated by exercise and repeated movement, but improved by rest and anticholinesterase drugs. Juvenile myasthenia follows an unpredictable course of recurring exacerbations and periodic remissions. With current therapies, however, most cases of juvenile myasthenia are not as serious as the name implies. Although there is no known cure, drug treatment has improved prognosis and allows patients to lead relatively normal lives, except during exacerbations.
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Škubník, Jan, Vladimíra Pavlíčková, and Silvie Rimpelová. "Cardiac Glycosides as Immune System Modulators." Biomolecules 11, no. 5 (April 29, 2021): 659. http://dx.doi.org/10.3390/biom11050659.
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Cardiac glycosides (CGs) are natural steroid compounds occurring both in plants and animals. They are known for long as cardiotonic agents commonly used for various cardiac diseases due to inhibition of Na+/K+-ATPase (NKA) pumping activity and modulating heart muscle contractility. However, recent studies show that the portfolio of diseases potentially treatable with CGs is much broader. Currently, CGs are mostly studied as anticancer agents. Their antiproliferative properties are based on the induction of multiple signaling pathways in an NKA signalosome complex. In addition, they are strongly connected to immunogenic cell death, a complex mechanism of induction of anticancer immune response. Moreover, CGs exert various immunomodulatory effects, the foremost of which are connected with suppressing the activity of T-helper cells or modulating transcription of many immune response genes by inhibiting nuclear factor kappa B. The resulting modulations of cytokine and chemokine levels and changes in immune cell ratios could be potentially useful in treating sundry autoimmune and inflammatory diseases. This review aims to summarize current knowledge in the field of immunomodulatory properties of CGs and emphasize the large area of potential clinical use of these compounds.
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Oliveira, Ezequiel Fernandes, Sergio Roberto Nacif, Nina Teixeira Fonseca, Nadua Apostólico, Jessica Julioti Urbano, Letícia Lopes Guimarães, Eduardo De Araujo Perez, Valéria Cavalcante, Acary Sousa Bulle, and Luis Vicente Franco Oliveira. "Pulmonary function and ventilatory muscle strengh in Myasthenia Gravis." Manual Therapy, Posturology & Rehabilitation Journal 12 (November 10, 2014): 198. http://dx.doi.org/10.17784/mtprehabjournal.2014.12.198.
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Introduction: Myasthenia gravis (MG) is a neuromuscular autoimmune disease of unknown etiology, characterized by generalized muscle weakness and fatigue, especially after repetitive physical activities, with consequent improvement after rest. The MG follows a slowly progressive course, which can be fatal failure of the ventilatory muscles. The manifestations of the respiratory system are generally attributed to the weakness of the diaphragm and also accessory muscles of ventilation. Objetive: The objective was to evaluate the volumes and lung capacities and maximum pressure ventilation in patients with clinically stable MG. Methods: This is an observational study involving 15 patients (2 men) with MG. Subjects were recruited consecutively and screened for eligibility using the standardized protocol. Results: Spirometry, only two patients showed abnormalities of respiratory pattern, being a moderate restrictive pattern (50-60% predicted), and another patient with the congenital form showed a severe restrictive pattern. Not obstructive patterns were observed. Our results of spirometry showed an average value of FVC: 3.15 ± 0.77 and FEV1: 2.64 ± 0.65. Regarding the maximum pressure generated by the ventilatory muscles, the average value for the MIP was 45.5 cmH2O among women and the value of 56 cmH2O for men was observed. To MEP it was observed the average value of 45 cmH2O for women and 55 cmH2O for men. Conclusion: We conclude that patients with MG have lower values of maximal inspiratory and expiratory ventilatory associated with normal lung function values.
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Richard, A., C. Legault, E. de Villers-Sidani, and D. Gendron. "P.038 A case report of an interesting paraneoplastic voltage-gated channelopathy." Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 43, S2 (June 2016): S30. http://dx.doi.org/10.1017/cjn.2016.142.
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Background: Morvan syndrome is an autoimmune paraneoplastic disorder affecting of voltage-gated potassium channels, most commonly the CASPR-2 subunit. The disorder is primarily characterized by hyperexcitability of both the central and peripheral nervous system accompanied by autonomic dysfunction. Clinically, the syndrome manifests as confusion, hallucinations, insomnia, hyperhidrosis, orthostatic hypotension, and muscle cramps with myoclonus. Methods: Patient chart, imaging, electrophysiology, and laboratory findings were reviewed from the time of MS diagnosis and through the course of treatment until symptom resolution. Results: Here we report a case of Morvan Syndrome in a 56 year old male with a previous history of thymic squamous carcinoma accompanied by paraneoplastic myasthenia gravis and myositis. His clinical presentation was notable for subacute onset of muscle cramping, insomnia, which progressed to also include visual and auditory hallucinations. He also had notable dysautonomic symptoms including orthostatic blood pressure changes, sialorrhea, and hyperhidrosis. The diagnosis was confirmed with a positive serum assay for antibodies against the CASPR-2 subunit of voltage-gated potassium channels. Conclusions: This case is notable because to our knowledge it one of the first to document a voltage-gated channelopathy in association with previous thymic cancer (and not thymoma). Moreover, this is a patient presenting with two other other autoimmune syndromes, i.e. myasthenia gravis and myositis.
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Tsiortou, Popianna, Harry Alexopoulos, and Marinos C. Dalakas. "GAD antibody-spectrum disorders: progress in clinical phenotypes, immunopathogenesis and therapeutic interventions." Therapeutic Advances in Neurological Disorders 14 (January 2021): 175628642110034. http://dx.doi.org/10.1177/17562864211003486.
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Antibodies against glutamic acid decarboxylase (GAD), originally linked to stiff person syndrome (SPS), now denote the “ GAD antibody-spectrum disorders” ( GAD-SD) that also include autoimmune epilepsy, limbic encephalitis, cerebellar ataxia and nystagmus with overlapping symptomatology highlighting autoimmune neuronal excitability disorders. The reasons for the clinical heterogeneity among GAD-antibody associated syndromes remain still unsettled, implicating variable susceptibility of GABAergic neurons to anti-GAD or other still unidentified autoantibodies. Although anti-GAD antibody titers do not correlate with clinical severity, very high serum titers, often associated with intrathecal synthesis of anti-GAD-specific IgG, point to in-situ effects of GAD or related autoantibodies within the central nervous system. It remains, however, uncertain what drives these antibodies, why they persist and whether they are disease markers or have pathogenic potential. The review, focused on these concerns, describes the widened clinical manifestations and overlapping features of all GAD-SD; addresses the importance of GAD antibody titers and potential significance of GAD epitopes; summarizes the biologic basis of autoimmune hyperexcitability; highlights the electrophysiological basis of reciprocal inhibition in muscle stiffness; and provides practical guidelines on symptomatic therapies with gamma-aminobutyric acid-enhancing drugs or various immunotherapies.
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Nehls, Volker. "Osteoarthropathien und Myopathien bei Schilddrüsenerkrankungen." DMW - Deutsche Medizinische Wochenschrift 143, no. 16 (August 2018): 1174–80. http://dx.doi.org/10.1055/s-0043-121381.
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AbstractTriiodothyronine (T3) is a key regulator of bone, muscle and articular cartilage. Musculoskeletal symptoms of hyperthyroidism include loss of bone mass finally leading to osteoporosis and weakness of the skeletal musculature. Hypothyroidism on the other side frequently leads to muscle stiffness and cramping and, occasionally, results in rhabdomyolysis. To prevent terminal differentiation of chondrocytes with consecutive cartilage degeneration, cartilage probably depends on exact regulation of local T3 availability by the intracellular deiodinase system. Recent findings underline the importance of local T3 generation by deiodinase type 2 and support the existence of local hypo- or hyperthyroidism.In the review, the implications of the recent literature for current understanding of osteoarthritis, myopathies and diabetic osteoarthropathy will be discussed. Further emphasis will be placed on the association of autoimmune thyroiditis with musculoskeletal diseases and fibromyalgia.
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Hoduț, Andrei, I. Simedrea, I. Sabău, Oana Belei, and Ionela Babi. "Ulcerative Colitis associated with Sclerosing Cholangitis and Autoimmune Hepatitis." Acta Medica Marisiensis 59, no. 2 (April 1, 2013): 115–20. http://dx.doi.org/10.2478/amma-2013-0028.
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AbstractIntroduction: Ulcerative colitis is a chronic intestinal inflammation, part of inflammatory bowel disease, which also includes Crohn’s disease. Both have extraintestinal manifestations, but those that tend to occur more commonly with ulcerative colitis include chronic active hepatitis, pyoderma gangrenosum and ankylosing spondylitis. Many individuals present with overlapping non-diagnostic features of more than one of these conditions that is referred to in the literature as autoimmune overlap syndrome. Sclerosing cholangitis associated with IBD is often referred to as overlap syndrome.Material and methods: We present the case of a 15-year-old female, with an association between ulcerative colitis, primitive sclerosing cholalangitis and autoimmune hepatitis. She was admitted for: diarrheic bloody stools, abdominal pain, diminished appetite, headache and aphthous stomatitis. Blood sample analysis revealed: hypochromic anemia, iron deficiency, high levels of transaminase, abnormal protein electrophoresis, positive anti-neutrophil cytoplasmic antibodies and anti-smooth muscle antibodies, high level of faecal calprotectin, modified biliary tract on imaging of digestive system and suggestive modifications of colic mucosa for ulcerative colitis. We administered treatment with Arginine Chloride 5%, Sorbitol 10%, Aspartic acid, Vitamin B6, Ursodeoxycholic acid, 5-aminosalicylic acid.Results: With the administered therapy the evolution was good, macroscopic blood disappeared from stools, and tests for blood trace in stool were also negative.Conclusions: The patient had simultaneous onset of diarrhea with bloody stools and extraintestinal manifestations. Immunological markers didn’t fully match any of the associated diseases, so we concluded that there was an overlap syndrome. Budesonide was effective on both hepatic and intestinal disease.
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Zhang, Sicheng, Duane D. Miller, and Wei Li. "Non-Musculoskeletal Benefits of Vitamin D beyond the Musculoskeletal System." International Journal of Molecular Sciences 22, no. 4 (February 21, 2021): 2128. http://dx.doi.org/10.3390/ijms22042128.
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Vitamin D, a fat-soluble prohormone, is endogenously synthesized in response to sunlight or taken from dietary supplements. Since vitamin D receptors are present in most tissues and cells in the body, the mounting understanding of the role of vitamin D in humans indicates that it does not only play an important role in the musculoskeletal system, but has beneficial effects elsewhere as well. This review summarizes the metabolism of vitamin D, the research regarding the possible risk factors leading to vitamin D deficiency, and the relationships between vitamin D deficiency and numerous illnesses, including rickets, osteoporosis and osteomalacia, muscle weakness and falls, autoimmune disorders, infectious diseases, cardiovascular diseases (CVDs), cancers, and neurological disorders. The system-wide effects of vitamin D and the mechanisms of the diseases are also discussed. Although accumulating evidence supports associations of vitamin D deficiency with physical and mental disorders and beneficial effects of vitamin D with health maintenance and disease prevention, there continue to be controversies over the beneficial effects of vitamin D. Thus, more well-designed and statistically powered trials are required to enable the assessment of vitamin D’s role in optimizing health and preventing disease.
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Londzin-Olesik, Magdalena, Beata Kos-Kudła, Aleksandra Nowak, and Mariusz Nowak. "The role of oxidative stress in the pathogenesis of Graves’ orbitopathy." Postępy Higieny i Medycyny Doświadczalnej 75 (June 18, 2021): 448–55. http://dx.doi.org/10.5604/01.3001.0014.9482.
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Graves’ disease (GD) is a chronic autoimmune condition in which the anti-thyroid stimulating hormone receptor antibodies (TRAb) activate the thyrotropin receptor (TSHR) located on thyrocytes, leading to excessive thyroid hormone production. TSHR is also expressed in extrathyroidal tissues, in particular, within the orbit. The serum levels of TRAb correlate with the severity and activity of thyroid orbitopathy (TO). TO is the most common extrathyroidal manifestation of GD. It is an autoimmune inflammation of orbital tissues, that is, extraocular muscles, orbital adipose tissue or a lacrimal gland. Increased orbital fibroblast and adipocyte proliferation, overproduction of glycosaminoglycans, as well as extraocular muscle oedema, result in increased orbital tissue volume and trigger the onset of TO symptoms. The pathophysiology of TO is complex and has not been fully unexplained to date. Orbital fibroblasts show expression of the TSHR, which is the main target of autoimmunity. It has been hypothesised that T-cell activation induced by orbital receptor stimulation by the target antibody results in orbital tissue infiltration, triggering a cascade of events which leads to the production of cytokines, growth factors and reactive oxygen species (ROS). ROS cause damage to many components of the cell: the cell membrane through the peroxidation of lipids and proteins leading to a loss of their function and enzymatic activity. Oxidative stress leads to the activation of the antioxidant system, which operates through two mechanisms: enzymatic and non-enzymatic. Assessment of the concentration of oxidative stress markers and the concentration or activity of anti-oxidative system parameters enables the evaluation of oxidative stress severity, which in the future may be utilized to assess treatment efficacy and prognosis in patients with active OT.
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Londzin-Olesik, Magdalena, Beata Kos-Kudła, Aleksandra Nowak, and Mariusz Nowak. "The role of oxidative stress in the pathogenesis of Graves’ orbitopathy." Postępy Higieny i Medycyny Doświadczalnej 75 (January 27, 2021): 1–10. http://dx.doi.org/10.5604/01.3001.0014.6969.
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Graves' disease (GD) is a chronic autoimmune condition, in which the anti-thyroid stimulating hormone receptor antibodies (TRAb) activate the thyrotropin receptor (TSHR) located on thyrocytes, leading to excessive thyroid hormone production. TSHR is also expressed in extrathyroidal tissues, in particular, within the orbit. The serum levels of TRAb corelate with severity and activity of thyroid orbitopathy (TO). TO is the most common extrathyroidal manifestation of GD. It is an autoimmune inflammation of orbital tissues, that is, extraocular muscles, orbital adipose tissue or a lacrimal gland. Increased orbital fibroblast and adipocyte proliferation, overproduction of glycosaminoglycans, as well as extraocular muscle oedema result in an increased orbital tissue volume and trigger the onset of TO symptoms. The pathophysiology of TO is complex and has not been fully unexplained to date. Orbital fibroblasts show expression of the TSHR, which is the main target of autoimmunity. It has been hypothesised that T-cell activation induced by orbital receptor stimulation by the target antibody results in orbital tissue infiltration, triggering a cascade of events which leads to the production of cytokines, growth factors and reactive oxygen species (ROS). ROS cause damage to many components of the cell: the cell membrane through the peroxidation of lipids and proteins leading to a loss of their function and enzymatic activity. Oxidative stress leads to activation of the antioxidant system which operates through two mechanisms: enzymatic and non-enzymatic. Assessment of the concentration of oxidative stress markers and the concentration or activity of antioxidative system parameters enables evaluation of oxidative stress severity, which in the future may be utilized for assessment of treatment efficacy and prognosis in patients with active OT.
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Wall, Jack R. "Graves' Disease Is a Multi-System Autoimmune Disorder in Which Extra Ocular Muscle Damage and Connective Tissue Inflammation Are Variable Features." Thyroid 12, no. 1 (January 2002): 35–36. http://dx.doi.org/10.1089/105072502753451940.
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Kapitza, Christopher, Rittika Chunder, Anja Scheller, Katherine S. Given, Wendy B. Macklin, Michael Enders, Stefanie Kuerten, Winfried L. Neuhuber, and Jürgen Wörl. "Murine Esophagus Expresses Glial-Derived Central Nervous System Antigens." International Journal of Molecular Sciences 22, no. 6 (March 22, 2021): 3233. http://dx.doi.org/10.3390/ijms22063233.
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Multiple sclerosis (MS) has been considered to specifically affect the central nervous system (CNS) for a long time. As autonomic dysfunction including dysphagia can occur as accompanying phenomena in patients, the enteric nervous system has been attracting increasing attention over the past years. The aim of this study was to identify glial and myelin markers as potential target structures for autoimmune processes in the esophagus. RT-PCR analysis revealed glial fibrillary acidic protein (GFAP), proteolipid protein (PLP), and myelin basic protein (MBP) expression, but an absence of myelin oligodendrocyte glycoprotein (MOG) in the murine esophagus. Selected immunohistochemistry for GFAP, PLP, and MBP including transgenic mice with cell-type specific expression of PLP and GFAP supported these results by detection of (1) GFAP, PLP, and MBP in Schwann cells in skeletal muscle and esophagus; (2) GFAP, PLP, but no MBP in perisynaptic Schwann cells of skeletal and esophageal motor endplates; (3) GFAP and PLP, but no MBP in glial cells surrounding esophageal myenteric neurons; and (4) PLP, but no GFAP and MBP in enteric glial cells forming a network in the esophagus. Our results pave the way for further investigations regarding the involvement of esophageal glial cells in the pathogenesis of dysphagia in MS.
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Nicolle, Amandine, Ye Zhang, and Karine Belguise. "The Emerging Function of PKCtheta in Cancer." Biomolecules 11, no. 2 (February 5, 2021): 221. http://dx.doi.org/10.3390/biom11020221.
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Protein Kinase C theta (PKCθ) is a serine/threonine kinase that belongs to the novel PKC subfamily. In normal tissue, its expression is restricted to skeletal muscle cells, platelets and T lymphocytes in which PKCθ controls several essential cellular processes such as survival, proliferation and differentiation. Particularly, PKCθ has been extensively studied for its role in the immune system where its translocation to the immunological synapse plays a critical role in T cell activation. Beyond its physiological role in immune responses, increasing evidence implicates PKCθ in the pathology of various diseases, especially autoimmune disorders and cancers. In this review, we discuss the implication of PKCθ in various types of cancers and the PKCθ-mediated signaling events controlling cancer initiation and progression. In these types of cancers, the high PKCθ expression leads to aberrant cell proliferation, migration and invasion resulting in malignant phenotype. The recent development and application of PKCθ inhibitors in the context of autoimmune diseases could benefit the emergence of treatment for cancers in which PKCθ has been implicated.
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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.
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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.
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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.
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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.
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Hicken, Jared, Daniel Ramirez, Mark Rigby, and Aram Minasian. "Stiff-Person Syndrome: Seeing Past Comorbidities to Reach the Correct Diagnosis." Case Reports in Neurological Medicine 2021 (January 31, 2021): 1–4. http://dx.doi.org/10.1155/2021/6698046.
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Stiff-person syndrome (SPS) is a rare disorder seen in approximately one in one million people. Although it is rare, the symptoms and findings of a typical case should paint a clear clinical picture for those who are familiar with the disease. The primary findings in SPS include progressive axial muscle rigidity as well as muscle spasms. These symptoms most commonly occur in the setting of antibodies against Glutamic Acid Decarboxylase (GAD), the rate-limiting enzyme in the production of Gamma-Aminobutyric Acid (GABA), which is the primary inhibitory enzyme in the central nervous system. Here, we report the case of a 65-year-old African-American female with a past medical history of hypothyroidism, anxiety, and depression with psychotic features who presented with axial muscle rigidity and lactic acidosis. She had been symptomatic for several months and reported extensive workups performed at two previous hospitals without a definitive diagnosis. A complete neurological and musculoskeletal investigation yielded no positive findings except for the presence of GAD antibodies. The patient was treated with diazepam, tizanidine, and Intravenous Immunoglobulin (IVIG) with significant improvement, thus solidifying the diagnosis of SPS, a rare autoimmune and/or paraneoplastic syndrome.
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Greco, Anna, Kirsten R. Straasheijm, Karlien Mul, Anita van den Heuvel, Silvère M. van der Maarel, Leo A. B. Joosten, Baziel G. M. van Engelen, and Ger J. M. Pruijn. "Profiling Serum Antibodies Against Muscle Antigens in Facioscapulohumeral Muscular Dystrophy Finds No Disease-Specific Autoantibodies." Journal of Neuromuscular Diseases 8, no. 5 (September 14, 2021): 801–14. http://dx.doi.org/10.3233/jnd-210653.
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Background: FSHD is caused by specific genetic mutations resulting in activation of the Double Homeobox 4 gene (DUX4). DUX4 targets hundreds of downstream genes eventually leading to muscle atrophy, oxidative stress, abnormal myogenesis, and muscle inflammation. We hypothesized that DUX4-induced aberrant expression of genes triggers a sustained autoimmune response against skeletal muscle cells. Objective: This study aimed at the identification of autoantibodies directed against muscle antigens in FSHD. Moreover, a possible relationship between serum antibody reactivity and DUX4 expression was also investigated. Methods: FSHD sera (N = 138, 48±16 years, 48% male) and healthy control sera (N = 20, 47±14 years, 50% male) were analyzed by immunoblotting for antibodies against several skeletal muscle protein extracts: healthy muscle, FSHD muscle, healthy and FSHD myotubes, and inducible DUX4 expressing myoblasts. In addition, DUX4 expressing myoblasts were analyzed by immunofluorescence with FSHD and healthy control sera. Results: The results showed that the reactivity of FSHD sera did not significantly differ from that of healthy controls, with all the tested muscle antigen extracts. Besides, the immunofluorescent staining of DUX4-expressing myoblasts was not different when incubated with either FSHD or healthy control sera. Conclusion: Since the methodology used did not lead to the identification of disease-specific autoantibodies in the FSHD cohort, we suggest that autoantibody-mediated pathology may not be an important disease mechanism in FSHD. Nevertheless, it is crucial to further unravel if and which role the immune system plays in FSHD pathogenesis. Other innate as well as adaptive immune players could be involved in the complex DUX4 cascade of events and could become appealing druggable targets.
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Maruszewska, Agnieszka, Lech Panasiuk, and Katarzyna Bryzek-Michalak. "Rehabilitation in Devica’s Syndrome. Case Report." Acta Balneologica 63, no. 1 (2021): 78–83. http://dx.doi.org/10.36740/abal202101111.
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Introduction: Devic’s syndrome also known as neuromyelitis optica is an autoimmune disease of central nervous system (CNS).It has an inflammatory, demyelizatory, chronic and relapsing nature, where ones own immune system attacks the spinal cord and optic nerves. Material and Methods: The authors presented a case report of a 54-year-old woman diagnosed with Devic’s syndrome 2 years ago. The physiotherapeutic examination revealed: visual and balance disturbances, decrease in muscle strength of upper and lower limbs, unsteady and shaky gait with a need to use a zimmer frame. At the start and at the end of a therapy, to objectively monitor the progress of rehabilitation treatment, a number of functional tests were used that included: Barthel Index, Brunnstrom’s test, Rankin Scale, Ashworth’s scale, Lovett’s test and a timed 20m walk. In physiotherapeutic treatment process patterns and techniques of PNF (prioprioceptive neuromuscular facilitation) ware used as well as exercise to correct muscles tone and strengthening exercise of trunk and lower limbs. Results: As a result of applying a comprehensive rehabilitation approach an increase of muscles strength of upper and lower limbs was obtained (Lovett’s scale R/L: shoulder 5/5, elbow 5/5, hand 5/5, hip 4+/4+, knee 4+/4+, foot 4+/4+). In addition all postural muscles gained in strength and an improvement in hands dexterity, body coordination and balance was noticed. Conclusion: Multidisciplinary approach and an individually selected rehabilitation program proves to show beneficial effects in a treatment process of patients with Devic’s syndrome.
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Choi, Ho-Jung, Myeong-Hoon Yeon, and Hee-Sook Jun. "Schisandrae chinensis Fructus Extract Ameliorates Muscle Atrophy in Streptozotocin-Induced Diabetic Mice by Downregulation of the CREB-KLF15 and Autophagy–Lysosomal Pathways." Cells 10, no. 9 (September 2, 2021): 2283. http://dx.doi.org/10.3390/cells10092283.
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Type 1 diabetes mellitus is an autoimmune disease caused by the destruction of pancreatic beta cells. Many patients with type 1 diabetes experience skeletal muscle wasting. Although the link between type 1 diabetes and muscle wasting is not clearly known, insulin insufficiency and hyperglycemia may contribute to decreased muscle mass. In this study, we investigated the therapeutic effect of the ethanolic extract of Schisandrae chinensis Fructus (SFe) on muscle wasting in streptozotocin (STZ)-induced diabetic mice. STZ-diabetic C57BL/6 mice (blood glucose level ≥300 mg/dL) were orally administered SFe (250 or 500 mg/kg/day) for 6 weeks. We observed that SFe administration did not change blood glucose levels but increased gastrocnemius muscle weight, cross-sectional area, and grip strength in STZ-induced diabetic mice. Administration of SFe (500 mg/kg) decreased the expression of atrophic factors, such as MuRF1 and atrogin-1, but did not alter the expression of muscle synthetic factors. Further studies showed that SFe administration decreased the expression of KLF15 and p-CREB, which are upstream molecules of atrophic factors. Examination of the expression of molecules involved in autophagy–lysosomal pathways (e.g., p62/SQSTM1, Atg7, Beclin-1, ULK-1, LC3-I, and LC3-II) revealed that SFe administration significantly decreased the expression of p62/SQSTM1, LC3-I, and LC3-II; however, no changes were observed in the expression of Atg7, Beclin-1, or ULK-1. Our results suggest that SFe ameliorated muscle wasting in STZ-induced diabetic mice by decreasing protein degradation via downregulation of the CREB-KLF15-mediated UPS system and the p62/SQSTM1-mediated autophagy–lysosomal pathway.
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Uzunalimoğlu, Berrin Pelit, Abdülhamit Sağlam, Büşra Şişman, Sefer Günaydın, Esen Gül Uzuner, Fikret Aysal, Erdem Tüzün, and Birgül Baştan. "Leucine-Rich Glioma-Inactivated Protein 1 Antibody-Positive Polyradiculopathy Associated with Epstein-Barr Virus Infection." Case Reports in Neurology 13, no. 2 (August 19, 2021): 549–54. http://dx.doi.org/10.1159/000518196.
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Epstein-Barr virus (EBV) has been associated with a plethora of neurological manifestations including polyneuropathy and polyradiculopathy. A 27-year-old man with a recent upper respiratory system infection presented with difficulty in walking. His neurological examination revealed reduced muscle strength in both proximal and distal lower limb muscles without sensory and autonomic signs. Needle electromyography showed abnormal spontaneous activity and reduced recruitment of motor units in muscles innervated by multiple lumbo-sacral roots. Cerebrospinal examination showed increased protein levels with normal cell counts. While spinal MRI was normal, whole-body CT and PET examination showed disseminated lymph node enlargement. Anti-EBV viral capsid antigen and anti-nuclear antigen IgG but not IgM was positive, whereas EBV PCR was negative in blood. Analysis of inguinal lymph node biopsy showed reactive lymphoid hyperplasia and EBV DNA. Leucine-rich glioma-inactivated protein 1 (LGI1) antibody was found in serum but not in CSF. All clinical, imaging, and electrophysiological findings improved following steroid and intravenous immunoglobulin treatment. These findings suggested the acute involvement of lumbo-sacral spinal roots and/or motor neurons. Purely motor polyradiculopathy has been reported in both EBV-positive and LGI1 antibody-positive patients, and EBV infection is known to precede different autoimmune manifestations. Whether EBV infection may trigger LGI1 autoimmunity and cause involvement of spinal motor roots and/or motor neurons needs to be further studied.
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Vyas, N., H. Alkhawam, R. Sogomonian, RA Ching Companioni, and A. Walfish. "ID: 37: SILENT BUT DEADLY CYTOMEGALOVIRUS TRIGGERING AUTOIMMUNE HEPATITIS." Journal of Investigative Medicine 64, no. 4 (March 22, 2016): 942.2–943. http://dx.doi.org/10.1136/jim-2016-000120.62.
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IntroductionTo consider that viruses, more specifically cytomegalovirus (CMV), can trigger autoimmune hepatitis.Case Report54 year-old female presents with new onset jaundice, which was associated with abdominal distension, lower extremity edema and 10 pound weight gain. She has no history of intravenous drug use, blood transfusions, any new sexual partners in over 8 years or a family history of liver disease. The physical examination was remarkable for spider angiomata, icteric sclera, ascites, and edema.The results of the biochemical analysis of the blood were the following: Liver enzymes were all elevated ALP 162 U/L, GGT 65 U/L, AST 154 U/L, ALT 72 U/L. Furthermore, her autoimmune workup was significant for an elevated ANA titer of 1:640, anti-smooth muscle ab titer 1:40 and a significant increase in immunoglobulins specifically IgG which was 4100 mg/dL. Interestingly, CMV Ab IgM was positive at 36.6 u/mL as well as CMV Ab IgG, which was positive at >10.00 u/mL. The rest of the work up was unremarkable in regards to hepatitis A, B, C, HIV, HSV, Epstein Barr virus (EBV), alpha1 antitrypsin, ceruloplasmin, iron level, ferritin and antimitochondrial ab. A liver biopsy was performed which showed heavy infiltration with lymphoplasmacytic inflammatory cells, interface hepatitis, bridging necrosis, and fibrosis. These pathologic and laboratory findings led us to a definitive diagnosis of autoimmune hepatitis (AIH) Type 1. In the setting of positive CMV IgG and IgM ab titers, we suggest that the trigger for AIH in this case was a preceding CMV infection. Her evolution was satisfactory under corticosteroid and azathioprine therapy.DiscussionAutoimmune hepatitis is a chronic hepatocellular inflammation and necrosis of unknown cause. The most supported pathogenesis of AIH postulates that a combination of environmental triggers, failure of immune system tolerance and a genetic predisposition that may induce a T cell–mediated immune attack against the liver. Case studies in the literature report AIH being triggered by virus and drugs. There is evidence of cross-reactivity between anti-LKM1 and antibodies against homologous regions of cytomegalovirus (exon CMV130-135). This case could explain an association between cytomegalovirus infection and autoimmune hepatitis.As clinicians, it is difficult to diagnose autoimmune hepatitis because its presentation can be acute, severe, asymptomatic or chronic. Diagnosis requires multiple findings and exclusions of similar diseases. When excluding, make sure viral etiologies are part of the differential, which in this case is CMV. If indeed a trigger is required to set off a sequence of events leading to autoimmune hepatitis in these predisposed individuals, viruses are among the most likely candidates.Abstract ID: 37 Figure 1
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Wirtshafter, Stephanie, and Iqra Iqbal. "Acute Inflammatory Demyelinating Polyneuropathy (AIDP) Masked by Autoimmune Thyroiditis." Journal of the Endocrine Society 5, Supplement_1 (May 1, 2021): A918. http://dx.doi.org/10.1210/jendso/bvab048.1875.
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Abstract Hashimoto’s thyroiditis and Guillain-Barre syndrome (GBS) are autoimmune disorders that are both well-known in their own right. Hashimoto’s is one of the most common causes of primary hypothyroidism, and GBS involves immune mediated damage to the peripheral nervous system. The association between the two is a rare clinical entity. This case demonstrates that these entities can occur together and could be related in similar pathophysiology. A 37 year old male presented with complaints of bilateral hand and feet numbness for one month. The numbness started in the hands, then involved the feet, and was mostly felt in tips of extremities. He also complained of weakness in arms and legs. Neurology exam showed bilateral patellar, ankle, and biceps hyporeflexia. Muscle strength was 5/5 in all extremities, but decreased grip strength was noted in the hands. Initial lab work including complete blood count, comprehensive metabolic profile and urinalysis were all in normal range. Computerized tomographic scan (CT) head was normal while CT abdomen/pelvis showed hepatic fatty infiltration. Other lab tests including HIV, syphilis, Hepatitis B, Hepatitis C, glycosylated hemoglobin A1c, lipid panel, anti-nuclear antibody, anti-neutrophil cytoplasmic antibodies, serum/urine protein electrophoresis, alcohol level, vitamin B1, B6, folate, copper, and creatine kinase were all negative or within normal range. Lab abnormalities included elevated thyroid stimulating hormone (TSH) of 20.2 mIU/l and low normal B12 level of 289 pg/ml. His triiodothyronine (T3) and thyroxine (T4) hormone levels were in normal range. A thyroid peroxidase antibody level came back as high as 966 IU/ml. A diagnosis of Hashimoto’s thyroiditis leading to subclinical hypothyroidism was made. Patient was discharged on vitamin B12 and 112mcg of Synthroid. Instead of getting better, he returned 1 week later with worsening numbness and tingling which was now ascending upward to bilateral knees and elbows. Meanwhile TSH improved to 10 mIU/l and vitamin B12 increased to 1162 pg/ml. A magnetic resonance imaging (MRI) of the cervical/thoracic spine was unremarkable. A lumbar puncture showed negative xanthochromia, 0 WBC, 0 RBC, 0 neutrophils, 0 lymphocytes, 0 monocytes, glucose 63 mg/dl, elevated protein of 57 mg/dl, and culture was negative. Guillain-Barre syndrome was then the working diagnosis, more specifically its most common subtype, acute inflammatory demyelinating polyneuropathy (AIDP). Patient received five days of intravenous immunoglobulins and his symptoms improved. He was then discharged to follow up with endocrinologist. This subtle presentation of GBS/AIDP masked by Hashimoto’s thyroiditis and vitamin B12 deficiency suggests a close association of autoimmune etiology between these disorders. Although rare, endocrinologists should consider this rare association in cases of paresthesias with unexplained symptoms.
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Byers, Mary Shannon. "Guillain-Barre Syndrome: Review and Summary." Journal of Biomedical Research & Environmental Sciences 2, no. 8 (August 2021): 685–89. http://dx.doi.org/10.37871/jbres1297.
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uillain-Barré Syndrome is a life-threatening, demyelinating, autoimmune condition in which the body’s immune system attacks the myelin of the peripheral nervous system. Guillain-Barré Syndrome is characterized by ascending motor weakness and acute flaccid paralysis. Demyelination results in nerve inflammation, numbness, tingling, muscle weakness, structural damage to the myelin sheath, and possible respiratory system complications. The annual incidence rate is 1.1 to 1.8 per 100,000 persons worldwide. Guillain-Barré Syndrome is thought to be triggered by an antecedent infection such as a viral, gastrointestinal, or bacterial infection, food poisoning, or reaction to a vaccine. Approximately 9-11% of cases result in severe disability or death. The acute phase can vary in length from a few days to several months, although over 90% of patients begin rehabilitation within four weeks. Patient care involves a team of neurologists, physiatrist, internist, nurses, physical, occupational, and speech therapists, social worker, psychologist and family physician. Elevated cerebrospinal fluid protein, symmetrical muscle weakness, the rate and order at which symptoms appear, and the absence or prolonged latency of reflexes are hallmarks for diagnosing Guillain-Barré Syndrome. A lumbar puncture to test for protein levels in the brain and spinal cord, and nerve conduction velocity test may aid in proper diagnosis, critical for optimizing treatment options and minimizing further progression. Although there is no cure, treatment may consist of plasmapheresis, typically performed four times during hospitalization, or intravenous immunoglobulin. Intravenous immunoglobulin combined with plasmapheresis should be avoided. Although glucocorticoids could repair damage to the blood-nerve barrier, oral corticosteroids could delay recovery.
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Gejalakshmi S, Harikrishnan N, and Anas Mohammied. "Molecular dynamic properties and Insilico screening of neuroprotective activity of Clitoria ternatea against Glutamate receptors." International Journal of Research in Pharmaceutical Sciences 11, no. 4 (December 20, 2020): 7043–47. http://dx.doi.org/10.26452/ijrps.v11i4.3809.
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Multiple sclerosis is a popular autoimmune disease attack mainly the Central nervous system. It attacks the age group of people from 20-50, mostly women are attacked than men. During multiple sclerosis, demyelination takes place along with axon damage and paralytic effect. Various symptoms of multiple sclerosis include muscle weakness, weak reflexes, muscle spasm, movement difficulty. Moreover, treatment of multiple sclerosis vai drugs includes various side effects. Medicinal plants possess many phytochemicals of greater therapeutic value and many of the possess effective to treat multiple sclerosis, Chemical constituents exhibit its effect over multiple sclerosis by inhibiting many proteins involved in demyelination. Molecular docking is a computational design approach which facilitates the best molecule from a group which may bind with the highest affinity with the intended target by providing a biological system. This process enables on the basis of the specific algorithm and involves a scoring function in order to rank molecules that fit the target. The study has been made to investigate the potential of phytochemicals from clitoria ternatea -inositol and quercetin as inhibitors of glutamate receptors. Drug likeness property determined based on molinspiration.com. The affinities of those selected chemical constituents over various glutamate receptor were studied for scoring function. Receptors with PDB code 1EQ8,4E0W,3KR2 were chosen to dock against the chemical constituent Inositol richest chemical constituent in Clitoria ternatea and scoring function was found to be -3.45,-4.56, -5.67kcal/mol.
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Kalinin, R. E., I. A. Suchkov, N. D. Mzhavanadze, and N. V. Korotkova. "Endothelial dysfunction in muscular dystrophies." NAUKA MOLODYKH (Eruditio Juvenium) 9, no. 2 (June 30, 2021): 326–34. http://dx.doi.org/10.23888/hmj202192326-334.
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Endotheliocytes are the key elements of the vascular wall and are involved in regulation of vascular tone and permeability, inflammation, hemostasis, angiogenesis etc. Impaired function of endothelial cells universally recognized as endothelial dysfunction is associated with a number of common diseases such as ischemic heart disease, arterial hypertension, atherosclerosis, peripheral arterial disease, septic shock, chronic kidney disease, obesity, oncological and autoimmune diseases. Less is known about the role of endothelial cells in pathogenesis of development and progression of rare diseases, such as muscular dystrophies. Muscular dystrophies involve over 30 genetically determined diseases, which are characterized by the development of a progressive muscular weakness and skeletal muscle degeneration. Presence of a nucleotide variant associated with a certain muscular dystrophy is primarily marked by a limited potential of skeletal muscle regeneration due to the impaired structure and function of myogenic cells. Inherited myopathies include a group of severe neuromuscular diseases caused by a mutation in the dysferlin gene DYSF, which leads to the synthesis of a dysfunctional dysferlin. Complex molecular and cellular interactions involved in skeletal muscle damage and endothelial dysfunction play an important role in the pathogenesis of dysferlinopathies. The possibility to produce an effect on different pathological aspects of dysferlinassociated myopathies such as complement system activation, inflammation, impaired function of endothelial cells, damage and necrosis of myofibrils are extensively studied in vitro and in vivo. This article is dedicated to the current understanding of relationship between the endothelium and its dysfunction in myogenesis and skeletal muscle regeneration in normal and pathological conditions caused by a group of inherited progressive myodystrophies, dysferlinopathies in particular, as well as possible clinical application of endothelial cells in treatment of muscular dystrophies.
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Spirig, Rolf, Janice Tsui, and Sidney Shaw. "The Emerging Role of TLR and Innate Immunity in Cardiovascular Disease." Cardiology Research and Practice 2012 (2012): 1–12. http://dx.doi.org/10.1155/2012/181394.
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Cardiovascular disease is a complex disorder involving multiple pathophysiological processes, several of which involve activation of toll-like receptors (TLRs) of the innate immune system. As sentinels of innate immunity TLRs are nonclonally germline-encoded molecular pattern recognition receptors that recognize exogenous as well as tissue-derived molecular dangers signals promoting inflammation. In addition to their expression in immune cells, TLRs are found in other tissues and cell types including cardiomyocytes, endothelial and vascular smooth muscle cells. TLRs are differentially regulated in various cell types by several cardiovascular risk factors such as hypercholesterolemia, hyperlipidemia, and hyperglycemia and may represent a key mechanism linking chronic inflammation, cardiovascular disease progression, and activation of the immune system. Modulation of TLR signaling by specific TLR agonists or antagonists, alone or in combination, may be a useful therapeutic approach to treat various cardiovascular inflammatory conditions such as atherosclerosis, peripheral arterial disease, secondary microvascular complications of diabetes, autoimmune disease, and ischemia reperfusion injury. In this paper we discuss recent developments and current evidence for the role of TLR in cardiovascular disease as well as the therapeutic potential of various compounds on inhibition of TLR-mediated inflammatory responses.
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van Lunteren, Erik, Michelle Moyer, and Henry J. Kaminski. "Adverse effects of myasthenia gravis on rat phrenic diaphragm contractile performance." Journal of Applied Physiology 97, no. 3 (September 2004): 895–901. http://dx.doi.org/10.1152/japplphysiol.01266.2003.
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Myasthenia gravis has variable effects on the respiratory system, ranging from no abnormalities to life-threatening respiratory failure. Studies characterized diaphragm muscle contractile performance in rat autoimmune myasthenia gravis. Rats received monoclonal antibody that recognizes acetylcholine receptor determinants (or inactive antibody); 3 days later, phrenic nerve and diaphragm were studied in vitro. Myasthenic rats segregated into two groups, those with normal vs. impaired limb muscle function when tested in intact animals (“mild” and “severe” myasthenic). Baseline diaphragm twitch force was reduced for both severe ( P < 0.01) and mild ( P < 0.05) myasthenic compared with control animals (twitch force: normal 1,352 ± 140, mild myasthenic 672 ± 99, severe myasthenic 687 ± 74 g/cm2). However, only severe myasthenic diaphragm had impaired diaphragm endurance, based on significantly ( P < 0.05) accelerated rate of peak force decline during the initial period of stimulation (0.02 + 0.02, 0.03 ± 0.01, and 0.09 ± 0.01%/pulse for normal, mild myasthenic, and severe myasthenic, respectively, during continuous stimulation) and intratrain fatigue (up to 30.5 ± 7.4% intratrain force drop in severe myasthenic vs. none in normal and mild myasthenic, P < 0.01). Furthermore, compared with continuous stimulation, intermittent stimulation had a protective effect on force of severe myasthenic diaphragm (force after 2,000 pulses was 31.4 ± 2.0% of initial during intermittent stimulation vs. 13.0 ± 2.1% of initial during continuous stimulation, P < 0.01) but not on normal diaphragm. These data indicate that baseline force and fatigue may be affected to different extents by varying severity of myasthenia gravis and furthermore provide a mechanism by which alterations in breathing pattern may worsen respiratory muscle function in neuromuscular diseases.
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Ridolo, E., R. Albertini, L. Borghi, T. Meschi, E. Montanari, and P. P. Dall'Aglio. "Acute Polyradiculoneuropathy Occurring after Hymenoptera Stings: A Clinical Case Study." International Journal of Immunopathology and Pharmacology 18, no. 2 (April 2005): 385–90. http://dx.doi.org/10.1177/039463200501800220.
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Hymenoptera stings may be responsible for both local and systemic reactions; these can be immediate or delayed, depending on the time between the sting and the development of signs or symptoms. Delayed clinical reactions have been reported, although unusual, due to serum sickness and/or affecting organs or systems generally not involved in the immediate reaction, such as heart, kidneys, central and peripheral nervous systems. This paper describes the clinical and immunological findings in a 51-year-old subject, who, after two stings of paper wasps, the second one after the third venom immunotherapy (VIT) injection, presented immediate large local and systemic allergic ractions which quickly improved after e.v. methilprednisolone administration. About 40 hours later, he developed acute polyradiculoneuropathy with muscle weakness, paresthesia, difficulties in standing up and walking. Skin tests and specific IgE determination showed allergy to paper wasp. The activation, by wasp venom, of peripheral blood mononuclear cells in primary culture, evaluated by tritiated thymidine incorporation proliferation assay, showed an important hypersensitivity to wasp venom. Therefore our results suggest the hypothesis that the polyradiculoneurtis causative etiopathogenetic mechanism might be a delayed immunological response to wasp antigens followed by an allergy-triggered autoimmune reaction, as previously suggested by other authors; they found lymphocytic infiltrates in demyelinization areas and at perivascular levels, by histologic examination of autoptical and bioptical material of patients with nervous system lesions after hymenoptera stings.
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Ferder, Marcelo, Felipe Inserra, Walter Manucha, and León Ferder. "The world pandemic of vitamin D deficiency could possibly be explained by cellular inflammatory response activity induced by the renin-angiotensin system." American Journal of Physiology-Cell Physiology 304, no. 11 (June 1, 2013): C1027—C1039. http://dx.doi.org/10.1152/ajpcell.00403.2011.
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This review attempts to show that there may be a relationship between inflammatory processes induced by chronic overstimulation of the renin-angiotensin system (RAS) and the worldwide deficiency of vitamin D (VitD) and that both disorders are probably associated with environmental factors. Low VitD levels represent a risk factor for several apparently different diseases, such as infectious, autoimmune, neurodegenerative, and cardiovascular diseases, as well as diabetes, osteoporosis, and cancer. Moreover, VitD insufficiency seems to predispose to hypertension, metabolic syndrome, left ventricular hypertrophy, heart failure, and chronic vascular inflammation. On the other hand, inappropriate stimulation of the RAS has also been associated with the pathogenesis of hypertension, heart attack, stroke, and hypertrophy of the left ventricle and vascular smooth muscle cells. Because VitD receptors (VDRs) and RAS receptors are almost distributed in the same tissues, a possible link between VitD and the RAS is even more plausible. Furthermore, from an evolutionary point of view, both systems were developed simultaneously, actively participating in the regulation of inflammatory and immunological mechanisms. Changes in RAS activity and activation of the VDR seem to be inversely related; thus any changes in one of these systems would have a completely opposite effect on the other, making it possible to speculate that the two systems could have a feedback relationship. In fact, the pandemic of VitD deficiency could be the other face of increased RAS activity, which probably causes lower activity or lower levels of VitD. Finally, from a therapeutic point of view, the combination of RAS blockade and VDR stimulation appears to be more effective than either RAS blockade or VDR stimulation individually.
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Khanenko, N., N. Svyrydova, G. Chuprina, T. Parnikosa, R. Sulik, V. Sereda, Т. Cherednichenko, and V. Svystun. "Hyperkinesis: pathogenesis, clinical features, diagnosis, treatment (clinical lecture)." East European Journal of Neurology, no. 3(21) (September 20, 2018): 13–18. http://dx.doi.org/10.33444/2411-5797.2018.3(21).13-18.
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Traditionally, the basic etiological concepts are considered in the views on the morphophysiological basis of hyperkinesis. Hyperkinesia is associated with hypotonia, a decrease in muscle tone, and hyperkinetic disorders are psychogenic and manifest in childhood. Hyperkinesia can be caused by a large number of various diseases, including metabolic disorders, endocrine disruption, hereditary disorders, vascular disorders or traumatic disorders. Other causes include intoxication of the nervous system, autoimmune diseases and infections. The classification of hyperkinesis is that hyperkinetic motions can be defined as any undesirable, excessive movements that can be distinguished from each other, based on the degree to which they are rhythmic, discrete, repetitive and random. When assessing a patient with suspected hyperkinesia, the doctor thoroughly records in the history of the disease a clear description of the movements, the medications prescribed in the past and present, the family history of the similar diseases, the history of the disease, including past infections, and any other influences. Treatment is aimed at reducing symptoms, restoring normal posture and improving the general condition of the patient.
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Ketineni, Sujitha, Sreenath Kodali, and Sasikanth Gorantla. "A Rare Case of Sensory Neuropathy Associated with Transitional Cell Carcinoma of the Bladder." Case Reports in Oncology 13, no. 3 (November 30, 2020): 1397–401. http://dx.doi.org/10.1159/000510742.
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Malignancies can trigger an autoimmune response against the nervous system and manifest as paraneoplastic neurological syndromes (PNS). Initial symptoms of PNS may develop up to 5 years prior to the diagnosis of the underlying malignancy. We report a rare case of PNS associated with transitional cell carcinoma of the bladder in a 70-year-old male with a 6-month history of rapidly progressive symmetric sensory neuropathy. Peripheral neuropathy serological workup was unremarkable. A paraneoplastic neuropathy panel revealed anti-Hu autoantibodies. Further evaluation with a whole-body PET scan could not identify the primary malignancy, but it showed hypermetabolic hilar lymph nodes. An endobronchial ultrasound biopsy of the hilar lymph nodes was negative for cancer. The patient developed painless hematuria 2.5 years after the onset of the sensory neuropathy. Cystoscopy with biopsy revealed non-muscle-invasive transitional cell carcinoma of the bladder. Progression of the sensory neuropathy stopped after tumor resection. This case highlights the importance of a diligent and systematic approach to diagnose PNS. A relentless search is often required to detect PNS-associated occult malignancies.
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Hertz, Risha, James Espinosa, Alan Lucerna, and Doug Stranges. "Multiple Sclerosis Presenting with Facial Twitching (Myokymia and Hemifacial Spasms)." Case Reports in Neurological Medicine 2017 (2017): 1–3. http://dx.doi.org/10.1155/2017/7180560.
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Multiple sclerosis (MS) is a chronic inflammatory demyelinating disease of the central nervous system. The etiology is insufficiently understood. Autoimmune, genetic, viral, and environmental factors have been hypothesized. MS is twice as common in women as in men between the ages of 20 and 50 years. There is no known cure for MS. Current medical treatment helps to prevent new attacks and improve function after an attack. MS is diagnosed by physical examination, diagnostic imaging, and examination of cerebral spinal fluid. The most common physical signs and symptoms of MS include constitutional symptoms, muscle weakness, motor and autonomic spinal cord symptoms, paresthesias, and vision changes. Here we present a case of MS diagnosed in a 33-year-old male with facial myokymia of left eyelid, which progressed to left hemifacial spasm. This is an unusual presentation for multiple sclerosis. An awareness of this presentation not only may lead to an earlier diagnosis in some patients but can be a sign of relapse in patients with established multiple sclerosis.
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Deeb, Omar, and Maisa Nabulsi. "Exploring Multiple Sclerosis (MS) and Amyotrophic Lateral Scler osis (ALS) as Neurodegenerative Diseases and their Treatments: A Review Study." Current Topics in Medicinal Chemistry 20, no. 26 (November 3, 2020): 2391–403. http://dx.doi.org/10.2174/1568026620666200924114827.
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: Growing concern about neurodegenerative diseases is becoming a global issue. It is estimated that not only will their prevalence increase but also morbidity and health burden will be concerning. Scientists, researchers and clinicians share the responsibility of raising the awareness and knowledge about the restricting and handicapping health restrains related to these diseases. : Multiple Sclerosis (MS), as one of the prevalent autoimmune diseases, is characterized by abnormal regulation of the immune system that periodically attacks parts of the nervous system; brain and spinal cord. Symptoms and impairments include weakness, numbness, visual problems, tingling pain that are quietly variable among patients. : Amyotrophic Lateral Sclerosis (ALS) is another neurodegenerative disease that is characterized by the degeneration of motor neurons in the brain and spinal cord. Unlike MS, symptoms begin with muscle weakness and progress to affect speech, swallowing and finally breathing. Despite the major differences between MS and ALS, misdiagnosis is still influencing disease prognosis and patient’s quality of life. : Diagnosis depends on obtaining a careful history and neurological examination as well as the use of Magnetic Resonance Imaging (MRI), which are considered challenging and depend on the current disease status in individuals. : Fortunately, a myriad of treatments is available now for MS. Most of the cases are steroid responsive. Disease modifying therapy is amongst the most important set of treatments. : In ALS, few medications that slow down disease progression are present. The aim of this paper is to summarize what has been globally known and practiced about MS and ALS, as they are currently classified as important growing key players among autoimmune diseases. In terms of treatments, it is concluded that special efforts and input should be directed towards repurposing of older drugs and on stem cells trials. As for ALS, it is highlighted that supportive measurements and supplementary treatments remain essentially needed for ALS patients and their families. On the other hand, it is noteworthy to clarify that the patient-doctor communication is relatively a cornerstone in selecting the best treatment for each MS patient.