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Sample records for autoimmune myasthenia gravis

  1. Myasthenia Gravis

    Science.gov (United States)

    Myasthenia gravis is a disease that causes weakness in your voluntary muscles. These are the muscles that you ... gets worse with activity, and better with rest. Myasthenia gravis is an autoimmune disease. Your body's immune system ...

  2. Autoimmune Myasthenia Gravis after Sternal Fracture

    Directory of Open Access Journals (Sweden)

    Jens A. Petersen

    2012-01-01

    Full Text Available We report a 54-year-old woman who suffered a commotio cerebri, whiplash injury and a chest trauma with sternal fracture due to a high-velocity car accident. Two months later, she developed unilateral ptosis and blurred vision, which worsened during the day. Multiple diagnoses were suggested, ranging from thoracic outlet syndrome towards depression. Symptoms persisted and five years later, the patient consulted a neurologist. Laboratory analysis revealed significantly elevated levels of antibodies to acetylcholine receptors, and the diagnosis of myasthenia gravis was made. Speculatively, the damage of retrosternal thymic remnants due to a sternal fracture might have precipitated the condition or exacerbated subclinical disease.

  3. Complicating autoimmune diseases in myasthenia gravis: a review.

    Science.gov (United States)

    Nacu, Aliona; Andersen, Jintana Bunpan; Lisnic, Vitalie; Owe, Jone Furlund; Gilhus, Nils Erik

    2015-01-01

    Myasthenia gravis (MG) is a rare autoimmune disease of skeletal muscle endplates. MG subgroup is relevant for comorbidity, but usually not accounted for. MG patients have an increased risk for complicating autoimmune diseases, most commonly autoimmune thyroid disease, systemic lupus erythematosus and rheumatoid arthritis. In this review, we present concomitant autoimmune disorders associated with the different MG subgroups, and show how this influences treatment and prognosis. Concomitant MG should always be considered in patients with an autoimmune disorder and developing new neuromuscular weakness, fatigue or respiratory failure. When a second autoimmune disorder is suspected, MG should be included as a differential diagnosis.

  4. Myasthenia gravis - resources

    Science.gov (United States)

    Resources - myasthenia gravis ... The following organizations provide information on myasthenia gravis : Myasthenia Gravis Foundation of America -- www.myasthenia.org National Institute of Neurological Disorders and Stroke -- www.ninds.nih.gov/disorders/myasthenia_gravis/ ...

  5. Diagnostic and clinical classification of autoimmune myasthenia gravis.

    Science.gov (United States)

    Berrih-Aknin, Sonia; Frenkian-Cuvelier, Mélinée; Eymard, Bruno

    2014-01-01

    Myasthenia gravis is characterized by muscle weakness and abnormal fatigability. It is an autoimmune disease caused by the presence of antibodies against components of the muscle membrane localized at the neuromuscular junction. In most cases, the autoantibodies are against the acetylcholine receptor (AChR). Recently, other targets have been described such as the MuSK protein (muscle-specific kinase) or the LRP4 (lipoprotein related protein 4). Myasthenia gravis can be classified according to the profile of the autoantibodies, the location of the affected muscles (ocular versus generalized), the age of onset of symptoms and thymic abnormalities. The disease generally begins with ocular symptoms (ptosis and/or diplopia) and extends to other muscles in 80% of cases. Other features that characterize MG include the following: variability, effort induced worsening, successive periods of exacerbation during the course of the disease, severity dependent on respiratory and swallowing impairment (if rapid worsening occurs, a myasthenic crisis is suspected), and an association with thymoma in 20% of patients and with other autoimmune diseases such as hyperthyroidism and Hashimoto's disease. The diagnosis is based on the clinical features, the benefit of the cholinesterase inhibitors, the detection of specific autoantibodies (anti-AChR, anti-MuSK or anti-LRP4), and significant decrement evidenced by electrophysiological tests. In this review, we briefly describe the history and epidemiology of the disease and the diagnostic and clinical classification. The neonatal form of myasthenia is explained, and finally we discuss the main difficulties of diagnosis.

  6. [Pathogenesis of myasthenia gravis].

    Science.gov (United States)

    Téllez Zenteno, J F; Morales Buenrostro, L E; Torre Delgadillo, A

    2000-01-01

    Myasthenia gravis is a neuromuscular, autoimmune, and acquired disturbance characterized by weakness and fatigue of skeletal muscles. During the past two decades, remarkable progress has been made in the understanding of myasthenia gravis, and the new knowledge has been applied directly to the clinical diagnosis and treatment of this formerly severe disease. Myasthenia gravis is undoubtedly the most thoroughly understood of all human autoimmune diseases and has served as a model for the elucidation of mechanisms underlying other autoimmune disorders. In this review we mention the most important physiopathological aspects and its application in the clinic practice.

  7. Myasthenia Gravis

    Science.gov (United States)

    ... of the thymus gland in myasthenia gravis? The thymus gland, which lies in the chest area beneath the breastbone, plays an important role in the development of the immune system in early life. Its ...

  8. Combined short-term immunotherapy for experimental autoimmune myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Pestronk, A.; Drachman, D.B.; Teoh, R.; Adams, R.N.

    1983-08-01

    A therapeutic strategy was designed to eliminate the humoral immune response to acetylcholine receptor (AChR) in ongoing experimental autoimmune myasthenia gravis (EAMG). Rats with EAMG were treated with a protocol consisting of three components: (1) A single high dose of cyclophosphamide (200 mg/kg) was used to produce a rapid and sustained fall in the anti-AChR antibody levels by preferential destruction of antibody-producing B-lymphocytes. ''Memory'' lymphocytes were not eliminated by cyclophosphamide. (2) Irradiation (600 rads) was used to eliminate the ''memory'' cells. It eliminated the anamnestic response to a challenge with the antigen AChR. (3) Bone marrow transplantation was used to repopulate the hematopoietic system after the otherwise lethal dose of cyclophosphamide. We used bone marrow from syngeneic rats with active EAMG to simulate an autologous transplant. Rats with EAMG treated with this combined protocol showed a prompt and sustained fall in the anti-AChR antibody levels and had no anamnestic response to a challenge with AChR. Thus, an affected animal's own marrow could be stored and used later for repopulation after cyclophosphamide-irradiation treatment. This treatment eliminates the animal's ongoing immune responses and reconstitutes the immune system in its original state. The success of this approach suggests that, if their safety could be established, similar ''curative'' strategies might be developed for the treatment of patients with severe antibody-mediated autoimmune disorders, such as myasthenia gravis.

  9. Thymus, thymoma and myasthenia gravis.

    Science.gov (United States)

    Fujii, Yoshitaka

    2013-05-01

    Myasthenia gravis is an autoimmune disease. An autoantibody directed toward acetylcholine receptor (AChR) causes the destruction of the postsynaptic membrane and a reduction of the number of AChRs at neuromuscular junctions. A very puzzling, but interesting characteristic of myasthenia gravis is that many of the patients have an abnormality in their thymus. Many have a hyperplastic thymus with germinal centers, while others have a thymic tumor. How is the abnormality of the thymus related to myasthenia gravis? This review will summarize the existing evidence and try to find the missing link between the thymus and myasthenia gravis. The review will also comment on two distinct populations of myasthenia gravis patients without thymoma. The autoimmunity found in elderly patients is nonspecific and initiated via a different mechanism from the initiation of myasthenia gravis in younger patients.

  10. Effectiveness of Qiangjijianli capsule on experimental autoimmune myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    Shunmin Li; Bo Fu; Yuyan Li; DongYang; Shudong Yang

    2006-01-01

    BACKGROUND: Experimental autoimmune myasthenia gravis(EAMG) and anti-AchR antibody of human myasthenia gravis are the same immune globulin.This antibody restricts the activity of nicotinic acetylcholine receptor and the amount of receptor binding sites is decreased,so myasthenia gravis occurs.OBJECTIVE: To observe the therapeutic effect and mechanism of action of Qiangjijianli capsule on EAMG rats.DESIGN:A randomized controlled animal experiment.SETTING: Experimental Animal Center of Guangzhou University of Traditional Chinese Medicine.MATERIALS: Acetylcholine receptors(AchR)were extracted from electric skate's electric organ which lives in the sea near Guangzhou.It had high biological activity and the protein content was 1.63 g/L.Qiangjijianli capsule (Astragalus mongholicus,Codonopsis pilosula,Atractylodes macrocephala,Angelica sinensis,Bupleurum chinense,Cairo morningglory root or leaf,Glycyrrhixa uralensis,etc.0.5 g crude drug per capsule) was bought from the Manufacturing Laboratory of Guangzhou University of Traditional Chinese Medicine with the Batch No.89-11-1.METHODS:This experiment was carried out in the Experimental Animal Center of Guangzhou University of Traditional Chinese Medicine from May to August 1990.①Adult female SD rats were immunized with AchR.The animals' movement condition was observed and recorded everyday. Ten rats were chosen as normal control group.and they were not given any treatment and raised normally.After modeled,20 successful rats were randomly assigned into 2 groups:treatmental group and model group. 2 mL Qiangjijianli capsule suspension(1 g)was intragastrically administrated into each rat of treatmental group for 30 days; The same amount of clean water was intragastrically administrated into the rats of model group for 30 days.(2)Serum AchR antibody was measured with ABC-ELISA method. After administration,the rats were sacrificed.The complete diaphragmatic muscle was extracted for in vitro receptor binding test

  11. Myasthenia Gravis Tests

    Science.gov (United States)

    ... be limited. Home Visit Global Sites Search Help? Myasthenia Gravis Share this page: Was this page helpful? Overview | ... Tests The goals with testing are to diagnose myasthenia gravis (MG), distinguish it from other conditions with similar ...

  12. Ocular myasthenia gravis coincident with thyroid ophthalmopathy

    Directory of Open Access Journals (Sweden)

    Yaman A

    2003-01-01

    Full Text Available Graves' disease and myasthenia gravis are both auto-immune diseases and the coexistence of these two diseases is well recognized. Myasthenia gravis is more frequent in patients with thyroid disease. Here we present a case of thyroid ophthalmopathy and ocular myasthenia.

  13. Myasthenia Gravis Foundation of America

    Science.gov (United States)

    ... serves patients, their families and caregivers. What is myasthenia gravis (MG)? Myasthenia gravis (pronounced My-as-theen-ee- ... please click here Thymus Removal Effective Treatment for Myasthenia Gravis The long awaited study results from the Randomized ...

  14. The Effect of CD3-Specific Monoclonal Antibody on Treating Experimental Autoimmune Myasthenia Gravis

    Institute of Scientific and Technical Information of China (English)

    Ruonan Xu; Jianan Wang; Guojiang Chen; Gencheng Han; Renxi Wang; Beffen Shen; Yan Li

    2005-01-01

    CD3-specific monoclonal antibody was the first one used for clinical practice in field of transplantation. Recently,renewed interests have elicited in its capacity to prevent autoimmune diabetes by inducing immune tolerance. In this study, we tested whether this antibody can also be used to treat another kind of autoimmune disease myasthenia gravis (MG) and explored the possible mechanisms. MG is caused by an autoimmune damage mediated by antibody- and complement-mediated destruction of AChR at the neuromuscular junction. We found that administration of CD3-specific antibody (Fab)2 to an animal model with experimental autoimmune myasthenia gravis (EAMG) (B6 mice received 3 times of AChR/CFA immunization) could not significantly improve the clinical signs and clinical score. When the possible mechanisms were tested, we found that CD3 antibody treatment slightly down-regulated the T-cell response to AChR, modestly up-regulation the muscle strength. And no significant difference in the titers of IgG2b was found between CD3 antibody treated and control groups. These data indicated that CD3-specific antibody was not suitable for treating MG, an antibody- and complementmediated autoimmune disease, after this disease has been established. The role of CD3-specific antibody in treating this kind of disease remains to be determined.

  15. Myasthenia Gravis Complications

    Science.gov (United States)

    ... antibodies inhibiting this protein are related to the development of myasthenia gravis. Thymus gland Researchers believe that the thymus gland, a ... with myasthenia gravis also have tumors of the thymus (thymomas). Usually, ... part in the development of this condition. Genetic factors also may be ...

  16. Nutrition and Myasthenia Gravis

    Science.gov (United States)

    Good nutrition is important for everyone. This is especially true when you have a chronic disorder like myasthenia gravis ( ... difficulty with chewing and swallowing may interfere with nutrition and create safety issues. Diet modifications may be ...

  17. Treatment for Myasthenia Gravis (MG)

    Science.gov (United States)

    ... are individualized There are many effective treatments for myasthenia gravis. Spontaneous improvement and even remission (although uncommon) may ... Created by Kellen Interactive Web Design © copyright 2010 Myasthenia Gravis Foundation of America, Inc.

  18. Presumed Isotretinoin-Induced, Concomitant Autoimmune Thyroid Disease and Ocular Myasthenia Gravis: A Case Report

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    Huseyin Gursoy

    2012-11-01

    Full Text Available Introduction: There are many adverse effects that have been described for isotretinoin. To the best of our knowledge, this is the first report of a possible association of oral isotretinoin intake with autoimmune thyroiditis and ocular myasthenia gravis (OMG. Case Presentation: A 19-year-old Caucasian male, who had used oral isotretinoin for severe acne disease for the previous six months, was referred to our clinic. He had a three-week history of diplopia and variable bilateral ptosis. Physical examination showed moderate periorbital edema and limitations of up- and down-gaze in the left eye. Laboratory findings and thyroid ultrasound were consistent with autoimmune thyroiditis. Antithyroid therapy did not relieve the clinical symptoms. Concomitant OMG was suspected. Variable ptosis and a positive response to oral prednisolone of 40 mg/day and pyridostigmine of 360 mg/day supported the diagnosis of concomitant autoimmune thyroiditis and OMG. Conclusion: Autoimmune disorders may be triggered by oral isotretinoin treatment. Clinicians prescribing isotretinoin should be aware of the possible association between isotretinoin intake and concomitant autoimmune thyroiditis and OMG.

  19. Assays for myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Lindstrom, J.M.

    1988-12-06

    This patent describes an improvement in a process for diagnosing myasthenia gravis. The process comprises the steps of preparing a complex of acetycholine receptor protein, toxin and a radioactive isotope, incubating the complex with a serum sample from a patient so as to join antibodies engendered in connection with myasthenia gravis to the complex, precipitating the complex joined with antibody with anti-immunoglobulin and measuring radioactivity, from the radioactive isotope, of the precipitated complex. The improvement is that the acetylcholine receptor protein is derived from cells of the TE671 Line.

  20. Masticatory performance in patients with myasthenia gravis

    NARCIS (Netherlands)

    Weijnen, FG; Kuks, JBM; van der Glas, HW; Oudenaarde, [No Value; Bosman, F

    2002-01-01

    Masticatory muscle electromyograms (EMGs) were recorded while patients with bulbar myasthenia gravis chewed artificial food and compared with those of patients with ocular myasthenia gravis, patients in clinical remission who had previously suffered from bulbar myasthenia gravis and healthy individu

  1. Gallium scans in myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Swick, H.M. (Univ. of Kentucky, Lexington); Preston, D.F.; McQuillen, M.P.

    1976-01-01

    A study was conducted to determine whether /sup 67/Ga scans could be used for the detection of thymomas and to investigate the activity of the thymus gland in patients with myasthenia gravis. Scans of the anterior mediastinum proved to be a reliable way to detect thymomas. The scans were positive in eight patients including three with myasthenia gravis and histologically proved thymomas, three others with severe myasthenia gravis and thymic tumors, and two with histologically proved thymomas not associated with myasthenia. Activity on /sup 67/Ga scans was not directly related to the increased activity of the thymus gland that is presumed to be associated with myasthenia gravis. (HLW)

  2. Current Treatment, Emerging Translational Therapies, and New Therapeutic Targets for Autoimmune Myasthenia Gravis.

    Science.gov (United States)

    Guptill, Jeffrey T; Soni, Madhu; Meriggioli, Matthew N

    2016-01-01

    Myasthenia gravis (MG) is an autoimmune disease associated with the production of autoantibodies against 1) the skeletal muscle acetylcholine receptor; 2) muscle-specific kinase, a receptor tyrosine kinase critical for the maintenance of neuromuscular synapses; 3) low-density lipoprotein receptor-related protein 4, an important molecular binding partner for muscle-specific kinase; and 4) other muscle endplate proteins. In addition to the profile of autoantibodies, MG may be classified according the location of the affected muscles (ocular vs generalized), the age of symptom onset, and the nature of thymic pathology. Immunopathologic events leading to the production of autoantibodies differ in the various disease subtypes. Advances in our knowledge of the immunopathogenesis of the subtypes of MG will allow for directed utilization of the ever-growing repertoire of therapeutic agents that target distinct nodes in the immune pathway relevant to the initiation and maintenance of autoimmune disease. In this review, we examine the pathogenesis of MG subtypes, current treatment options, and emerging new treatments and therapeutic targets.

  3. Thymus irradiation for myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Currier, R.D.; Routh, A.; Hickman, B.T.; Douglas, M.A.

    1983-01-01

    Twenty-eight patients with progressive myasthenia gravis without thymoma received treatment of 3000 rads (30 Gy) to the anterior mediastinum, and a followup was conducted for five to 18 years. Twenty-four patients had generalized myasthenia, and four had ocular myasthenia gravis. Twenty patients with generalized myasthenia survived the several month post-treatment period and improved, but four died during that period. The improvement lasted a median of 1.5 years, and older patients had longer remissions than younger patients. The four patients who had ocular myasthenia did not change after treatment. Mediastinal irradiation produces a temporary remission in generalized myasthenia.

  4. Experimental autoimmune myasthenia gravis may occur in the context of a polarized Th1- or Th2-type immune response in rats

    DEFF Research Database (Denmark)

    Saoudi, A; Bernard, I; Hoedemaekers, A

    1999-01-01

    Experimental autoimmune myasthenia gravis (EAMG) is a T cell-dependent, Ab-mediated autoimmune disease induced in rats by a single immunization with acetylcholine receptor (AChR). Although polarized Th1 responses have been shown to be crucial for the development of mouse EAMG, the role of Th cell...

  5. Experimental Study on Induction of Tolerance to Experimental Autoimmune Myasthenia Gravis by Immature Dendritic Cells

    Institute of Scientific and Technical Information of China (English)

    2005-01-01

    To investigate the effect of immature dendritic cells (iDCs) on experimental autoimmune myasthenia gravis (MG), iDCs were generated in low dose of GM-CSF, and then they were pulsed with acetylcholine receptor (AchR) and transferred to allogeneic rats. After 3 weeks, all rats were immunized with AchR and complete Freund's adjuvant (CFA) and observed for the corresponding indices of MG for 7 weeks. Our results showed that compared with mature DCs (mDCs) generated at high dose of GM-CSF plus additional stimulation by lipopolysaccharide, iDCs expressed significantly lower levels of MHC-Ⅱ , CD80 and CD86, and their ability to uptake FITC-Dextran was stronger but the ability of stimulating proliferation of allogeneic T cells were weaker. Like controls,after immunization, all rats transferred with iDCs, mDCs and AchR-pulsed mDCs showed typical symptoms in 4 to 7 weeks. The amplitude of electromyogram wave dropped obviously, the level of serum AchRab increased and neuromuscular junction showed typical damage of MG. In contrast, no conspicuous changes were noted in rats transferred with AchR-pulsed iDCs. The results suggest that iDCs could be generated by inducing bone marrow precursors in low dose of GM-CSF, AchRpulsed iDCs could induce tolerance of EAMG. The dysfunction of DCs may play an important role in the initiation and maintenance of normal immune response in MG.

  6. Myasthenia gravis: subgroup classification and therapeutic strategies.

    Science.gov (United States)

    Gilhus, Nils Erik; Verschuuren, Jan J

    2015-10-01

    Myasthenia gravis is an autoimmune disease that is characterised by muscle weakness and fatigue, is B-cell mediated, and is associated with antibodies directed against the acetylcholine receptor, muscle-specific kinase (MUSK), lipoprotein-related protein 4 (LRP4), or agrin in the postsynaptic membrane at the neuromuscular junction. Patients with myasthenia gravis should be classified into subgroups to help with therapeutic decisions and prognosis. Subgroups based on serum antibodies and clinical features include early-onset, late-onset, thymoma, MUSK, LRP4, antibody-negative, and ocular forms of myasthenia gravis. Agrin-associated myasthenia gravis might emerge as a new entity. The prognosis is good with optimum symptomatic, immunosuppressive, and supportive treatment. Pyridostigmine is the preferred symptomatic treatment, and for patients who do not adequately respond to symptomatic therapy, corticosteroids, azathioprine, and thymectomy are first-line immunosuppressive treatments. Additional immunomodulatory drugs are emerging, but therapeutic decisions are hampered by the scarcity of controlled studies. Long-term drug treatment is essential for most patients and must be tailored to the particular form of myasthenia gravis.

  7. "Warming yang and invigorating qi" acupuncture alters acetylcholine receptor expression in the neuromuscular junction of rats with experimental autoimmune myasthenia gravis

    Directory of Open Access Journals (Sweden)

    Hai-peng Huang

    2016-01-01

    Full Text Available Myasthenia gravis is an autoimmune disorder in which antibodies have been shown to form against the nicotinic acetylcholine nicotinic postsynaptic receptors located at the neuromuscular junction. "Warming yang and invigorating qi" acupuncture treatment has been shown to reduce serum inflammatory cytokine expression and increase transforming growth factor beta expression in rats with experimental autoimmune myasthenia gravis. However, few studies have addressed the effects of this type of acupuncture on the acetylcholine receptors at the neuromuscular junction. Here, we used confocal laser scanning microscopy to examine the area and density of immunoreactivity for an antibody to the nicotinic acetylcholine receptor at the neuromuscular junction in the phrenic nerve of rats with experimental autoimmune myasthenia gravis following "warming yang and invigorating qi" acupuncture therapy. Needles were inserted at acupressure points Shousanli (LI10, Zusanli (ST36, Pishu (BL20, and Shenshu (BL23 once daily for 7 consecutive days. The treatment was repeated after 1 day of rest. We found that area and the integrated optical density of the immunoreactivity for the acetylcholine receptor at the neuromuscular junction of the phrenic nerve was significantly increased following acupuncture treatment. This outcome of the acupuncture therapy was similar to that of the cholinesterase inhibitor pyridostigmine bromide. These findings suggest that "warming yang and invigorating qi" acupuncture treatment increases acetylcholine receptor expression at the neuromuscular junction in a rat model of autoimmune myasthenia gravis.

  8. “Warmingyang and invigoratingqi” acupuncture alters acetylcholine receptor expression in the neuromuscular junction of rats with experimental autoimmune myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    Hai-peng Huang; Hong Pan; Hong-feng Wang

    2016-01-01

    Myasthenia gravis is an autoimmune disorder in which antibodies have been shown to form against the nicotinic acetylcholine nicotinic postsynaptic receptors located at the neuromuscular junction. “Warmingyang and invigoratingqi” acupuncture treatment has been shown to reduce serum inlfammatory cytokine expression and increase transforming growth factor beta expression in rats with experimental au-toimmune myasthenia gravis. However, few studies have addressed the effects of this type of acupuncture on the acetylcholine receptors at the neuromuscular junction. Here, we used confocal laser scanning microscopy to examine the area and density of immunoreactivity for an antibody to the nicotinic acetylcholine receptor at the neuromuscular junction in the phrenic nerve of rats with experimental autoimmune myasthenia gravis following “warmingyang and invigoratingqi” acupuncture therapy. Needles were inserted at acupressure pointsShou-sanli (LI10),Zusanli(ST36),Pishu (BL20), and Shenshu (BL23) once daily for 7 consecutive days. The treatment was repeated after 1 day of rest. We found that area and the integrated optical density of the immunoreactivity for the acetylcholine receptor at the neuromuscular junction of the phrenic nerve was signiifcantly increased following acupuncture treatment. This outcome of the acupuncture therapy was similar to that of the cholinesterase inhibitor pyridostigmine bromide. These ifndings suggest that “warmingyangand invigoratingqi” acu-puncture treatment increases acetylcholine receptor expression at the neuromuscular junction in a rat model of autoimmune myasthenia gravis.

  9. Genetics Home Reference: myasthenia gravis

    Science.gov (United States)

    ... genetic conditions more common in particular ethnic groups? Genetic Changes Researchers believe that variations in particular genes may increase the risk of myasthenia gravis , but the identity of these genes is unknown. Many factors likely ...

  10. Statin-associated weakness in myasthenia gravis: a case report

    Directory of Open Access Journals (Sweden)

    Keogh Michael J

    2010-02-01

    Full Text Available Abstract Introduction Myasthenia gravis is a commonly undiagnosed condition in the elderly. Statin medications can cause weakness and are linked to the development and deterioration of several autoimmune conditions, including myasthenia gravis. Case presentation We report the case of a 60-year-old Caucasian man who presented with acute onset of dysarthria and dysphagia initially attributed to a brain stem stroke. Oculobulbar and limb weakness progressed until myasthenia gravis was diagnosed and treated, and until statin therapy was finally withdrawn. Conclusion Myasthenia gravis may be underappreciated as a cause of acute bulbar weakness among the elderly. Statin therapy appeared to have contributed to the weakness in our patient who was diagnosed with myasthenia gravis.

  11. Effect of Polygoni Cuspidati Rhizoma et Radix and Its Ingredient Resveratrol on Experimental Autoimmune Myasthenia Gravis by Suppressing Immune Response

    Institute of Scientific and Technical Information of China (English)

    Shen Liu; Xin-xin Zhang; Shan Zhuang; Chun-hong Li; Yan-bin Li

    2016-01-01

    Objective To investigate the effects of Polygoni Cuspidati Rhizoma et Radix(PCRR) and its ingredient resveratrol(Res) on experimental autoimmune myasthenia gravis(EAMG). Methods EAMG was induced in Lewis rats by the immunization of a synthetic peptide corresponding to region 97–116 of the rat acetylcholine receptor(ACh R) α subunit(R97-116). EAMG rats were randomly divided into PCRR group, Res group, and control(C) group, and were ig administered respectively with PCRR(2 g/kg), Res(20 mg/kg), and DMSO(0.4 m L/kg) every day from day 5 after immunization to day 42. Clinical evaluation, lymphocyte proliferation, cytokines, and anti-97-116 antibodies were performed for examination of their therapeutic effects. Results Treatments with PCRR and Res significantly ameliorated clinical symptoms, down-regulated TNF-α and up-regulated IL-10 in serum and culture supernatants of lymphocytes stimulated with R97-116, and decreased levels of anti-R97-116 Ig G1 and Ig G2 a in serum compared with C group. Unexpectedly, PCRR but not Res inhibited lymphocyte proliferation compared with C group. Conclusion PCRR and Res ameliorating EAMG is associated with suppressing immune response, and indicates a therapeutic potential for EAMG and even human myasthenia gravis(MG). Res may be the main effective ingredient from PCRR ameliorating EAMG, but further experiments are necessary.

  12. Tongue force in patients with myasthenia gravis

    NARCIS (Netherlands)

    Weijnen, FG; Kuks, JBM; van der Glas, HW; Wassenberg, MWM; Bosman, F

    2000-01-01

    Objectives - The aim was to study tongue force in patients with bulbar myasthenia gravis and compare it with that of patients with ocular myasthenia gravis, patients in clinical remission who previously suffered from bulbar myasthenia gravis, and healthy subjects. Material and methods - Tongue force

  13. Mouse models of myasthenia gravis.

    Science.gov (United States)

    Ban, Joanne; Phillips, William D

    2015-01-01

    Myasthenia gravis is a muscle weakness disease characterized by autoantibodies that target components of the neuromuscular junction, impairing synaptic transmission. The most common form of myasthenia gravis involves antibodies that bind the nicotinic acetylcholine receptors in the postsynaptic membrane. Many of the remaining cases are due to antibodies against muscle specific tyrosine kinase (MuSK). Recently, autoantibodies against LRP4 (another component of the MuSK signaling complex in the postsynaptic membrane) were identified as the likely cause of myasthenia gravis in some patients. Fatiguing weakness is the common symptom in all forms of myasthenia gravis, but muscles of the body are differentially affected, for reasons that are not fully understood. Much of what we have learnt about the immunological and neurobiological aspects of the pathogenesis derives from mouse models. The most widely used mouse models involve either passive transfer of autoantibodies, or active immunization of the mouse with acetylcholine receptors or MuSK protein. These models can provide a robust replication of many of the features of the human disease. Depending upon the protocol, acute fatiguing weakness develops 2 - 14 days after the start of autoantibody injections (passive transfer) or might require repeated immunizations over several weeks (active models). Here we review mouse models of myasthenia gravis, including what they have contributed to current understanding of the pathogenic mechanisms and their current application to the testing of therapeutics.

  14. Myasthenia gravis and endurance exercise.

    Science.gov (United States)

    Scheer, Bernd Volker; Valero-Burgos, Encarna; Costa, Ricardo

    2012-08-01

    This is the first report of a runner with myasthenia gravis who completed an ultra endurance event. Myasthenia gravis, a neuromuscular disease that usually results in skeletal muscle weakness, which worsens with exercise and strenuous aerobic exercise, is generally contraindicated. Our runner completed a 220-km, 5-day ultramarathon and presented with various symptoms including muscular skeletal weakness, cramps, generalized fatigue, unintelligible speech, involuntary eye and mouth movements, problems swallowing, food lodging in his throat, and problems breathing. Risk factors identified for exacerbations are running in extreme temperatures, prolonged runs (especially a distance of 30 km or more), running uphill, lack of sleep, and stress. The medical team was in the novel situation to look after a runner with myasthenia gravis and needed to be aware of the patient's condition, symptoms, and risk factors to safely care for him.

  15. Multiple Thymoma with Myasthenia Gravis

    Science.gov (United States)

    Seo, Dong Hyun; Cho, Sukki

    2017-01-01

    The actual incidence of multiple thymoma is unknown and rarely reported because it remains controversial whether the cases represent a disease of multicentric origin or a disease resulting from intrathymic metastasis. In this case, a patient underwent total thymectomy for multiple thymoma with myasthenia gravis via bilateral video-assisted thoracic surgery. A well-encapsulated multinodular cystic mass, measuring 57 mm×50 mm×22 mm in the right lobe of the thymus, and a well-encapsulated mass, measuring 32 mm×15 mm×14 mm in the left lobe, were found. Both tumors were type B2 thymoma. Few cases of multiple thymoma with myasthenia gravis have ever been reported in the literature. We report a case of synchronous multiple thymoma associated with myasthenia gravis. PMID:28180109

  16. 重症肌无力的治疗现状%Circumstance of the therapy of myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    于逢春; 余华锋

    2004-01-01

    Myasthenia gravis is a most common neurological disease with autoimmune disorder. Most of the research on myasthenia gravis has focused on treatment . we will assess the effect of the treatments of myasthenia gravis, which including corticosteroids, immunosuppressive , IV immunoglobulin , plasmapheresis and thymectomy. The treatment for patients with myasthenia gravis usually depends on age, clinical characteristics and thymoma or nonthymoma-related MG and so on.

  17. New diagnosis myasthenia gravis and preeclampsia in late pregnancy.

    Science.gov (United States)

    Ozcan, John; Balson, Ian Frank; Dennis, Alicia T

    2015-02-26

    Myasthenia gravis is a chronic autoimmune disease of neuromuscular transmission resulting in fatigable skeletal muscle weakness. Preeclampsia is a multisystem disease of pregnancy which is characterised by hypertension and involvement of one or more organ systems. Both diseases are responsible for considerable morbidity and mortality for mother and fetus. The occurrence of both preeclampsia and myasthenia gravis in pregnancy is very rare, and conflicts arise when considering the optimal management of each disease.We present a case of a parturient who was newly diagnosed with both myasthenia gravis and preeclampsia in late pregnancy. Myasthenia treatment was started with prednisolone and pyridostigmine, and delivery was by caesarean section at 37 weeks gestation under spinal anaesthesia. Postnatally, the patient developed worsening of myasthenia and preeclampsia symptoms. We consider the anaesthetic implications for both diseases and describe our approach for the management of this case.

  18. Acquired generalised neuromyotonia, cutaneous lupus erythematosus and alopecia areata in a patient with myasthenia gravis.

    LENUS (Irish Health Repository)

    O'Sullivan, S S

    2012-02-03

    We describe a patient with the diagnoses of acquired neuromyotonia, cutaneous lupus erythematosus and alopecia areata, occurring many years after a thymectomy for myasthenia gravis associated with a thymoma. We review the current literature on autoimmune conditions associated with myasthenia gravis and thymectomy. To our knowledge, this combination of multiple autoimmune conditions has not been reported previously.

  19. A Genetic Study on Myasthenia Gravis

    Institute of Scientific and Technical Information of China (English)

    Lu Chuan-Zhen; Zhou Zhigang; Wu Yongqin

    2000-01-01

    @@Myasthenia Gravis is considered as an autoimmune disease caused by circulating ant:bodies against acetylcholine receptor(AchR) at neuromuscular junctions. Although thousands of studies in the field of function of AchR, activation passway of immune response on MG, genetic control of o-subunit of AchR have been done in the world since 1970's, it is still unclear what is the initial factor of autoimmune response and what hind of genes control are in MG patients.

  20. Myasthenia gravis: an update for the clinician.

    Science.gov (United States)

    Sieb, J P

    2014-03-01

    This paper provides a thorough overview of the current advances in diagnosis and therapy of myasthenia gravis (MG). Nowadays the term 'myasthenia gravis' includes heterogeneous autoimmune diseases, with a postsynaptic defect of neuromuscular transmission as the common feature. Myasthenia gravis should be classified according to the antibody specificity [acetylcholine, muscle-specific receptor tyrosine kinase (MuSK), low-density lipoprotein receptor-related protein 4 (LRP4), seronegative], thymus histology (thymitis, thymoma, atrophy), age at onset (in children; aged less than or more than 50 years) and type of course (ocular or generalized). With optimal treatment, the prognosis is good in terms of daily functions, quality of life and survival. Symptomatic treatment with acetylcholine esterase inhibition is usually combined with immunosuppression. Azathioprine still remains the first choice for long-term immunosuppressive therapy. Alternative immunosuppressive options to azathioprine include cyclosporin, cyclophosphamide, methotrexate, mycophenolate mofetil and tacrolimus. Rituximab is a promising new drug for severe generalized MG. Emerging therapy options include belimumab, eculizumab and the granulocyte- macrophage colony-stimulating factor. One pilot study on etanercept has given disappointing results. For decades, thymectomy has been performed in younger adults to improve non-paraneoplastic MG. However, controlled prospective studies on the suspected benefit of this surgical procedure are still lacking. In acute exacerbations, including myasthenic crisis, intravenous immunoglobulin, plasmapheresis and immunoadsorption are similarly effective.

  1. Miastenia gravis juvenil Juvenile myasthenia gravis

    Directory of Open Access Journals (Sweden)

    Oscar Papazian

    2009-01-01

    administración endovenosa de gamma globulina.Juvenile myasthenia gravis is a chronic autoimmune disorder which occurs when serum antibodies combine with nicotinic acetylcholine receptors at the muscle membrane of the motor endplate imparing the neuromuscular transmission. It results in early muscle fatigability with progression to a complete paralysis during repetitive muscle contraction (movements or steady muscle contraction (postures, and less common persistent paralysis at rest. The cranial nerves, mainly the one innervating the extraoccular and palpebral levator, are the most susceptible to permanent weakness and paralysis at rest. Initial clinical presentations are generalized, ocular and respiratory forms. The diagnosis is suspected through medical history of abnormal fatigability and corroborated by physical examination, repetitive nerve stimulation of an affected but not complete paralyzed muscle, correction of fatigability by the intravenous administration of acetylcholine esterase inhibitors, and by the presence of serum acetylcholine receptors antibodies (ACRA. The long term treatment is symptomatic (acetylcholine inhibitors and etiopathogenic (immunosupresor drugs, plasmapheresis, intravenous gamma globulin and thymectomy. Spontaneous or post symptomatic and etiopathogenic treatment remissions occur from 1 to 10 years. Fatality is rare but children are at high risk during myasthenia crisis.

  2. Ocular myasthenia gravis: A review

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    Akshay Gopinathan Nair

    2014-01-01

    Full Text Available Myasthenia gravis (MG is a disease that affects the neuro-muscular junction resulting in classical symptoms of variable muscle weakness and fatigability. It is called the great masquerader owing to its varied clinical presentations. Very often, a patient of MG may present to the ophthalmologist given that a large proportion of patients with systemic myasthenia have ocular involvement either at presentation or during the later course of the disease. The treatment of ocular MG involves both the neurologist and ophthalmologist. Thus, the aim of this review was to highlight the current diagnosis, investigations, and treatment of ocular MG.

  3. RI scintigraphy in myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Kuroda, Yoshikazu; Miyamoto, Mariko (Tokyo Metropolitan Fuchu Hospital (Japan)); Maki, Masako; Yamazaki, Toshiro

    1982-10-01

    35 cases of myasthenia gravis were studied with RI scintigraphy. /sup 67/Ga-citrate was used in 34 patients and /sup 76/Se-selenomethionine in 5 patients for thymus scintigraphy. RI scintigram was negative in non-tumorous thymus, regardless of the severity of illness and it was positive in seven of nine patients with thymomas. RI accumulation in thymus was found both in benign and malignant thymomas. RI seems to accumulated in lymphocytic and mixed thymus more than epithelial type.

  4. Early-onset acquired myasthenia gravis secondary to anti-muscle-specific kinase autoantibodies.

    Science.gov (United States)

    Yilmaz, Sanem; Gokben, Sarenur; Serdaroglu, Gul; Akcay, Ayfer

    2014-01-01

    Autoimmune myasthenia gravis is rarely seen during infancy. Similar to adults, 85% to 90% of generalized pediatric myasthenia gravis cases have acetylcholine receptor antibodies. Approximately 30% of the remaining cases have antibodies against muscle-specific kinase. Information on the clinical course, treatment alternatives, and prognosis of pediatric muscle-specific kinase antibody-positive myasthenia gravis is limited because of the small number of cases. Here, we present a 14-month-old girl with muscle-specific kinase antibody-positive myasthenia gravis as one of the youngest patients described so far in the literature.

  5. Isolated laryngeal myasthenia gravis for 26 years.

    Science.gov (United States)

    Renard, Dimitri; Hedayat, Amir; Gagnard, Corinne

    2015-02-01

    Laryngeal myasthenia gravis is a relatively rare variant of myasthenia gravis. A vast portion of patients with initial laryngeal myasthenia gravis develop involvement of ocular and/or extra-ocular muscles during the years after symptom onset although a minority of laryngeal myasthenia gravis patients continues to have isolated laryngeal muscle involvement for several years. We present a 58-year-old woman with recurrent episodic isolated dysphonia (associated with diffuse bilateral vocal cord paresis on laryngoscopy) since the age of 32. Dysphonia became permanent since 6 months. A diagnosis of laryngeal myasthenia gravis was made based on abnormal single-fiber electromyography and spectacular response to pyridostigmine treatment. Repetitive nerve stimulation was normal and anti-acetylcholine receptor and anti-muscle specific tyrosine kinase antibodies were absent. This case shows that laryngeal myasthenia gravis can be isolated during 26 years of follow-up. We propose that even when myasthenia gravis seems unlikely as underlying mechanism of isolated dysphonia (because of lack of antibodies, normal repetitive nerve stimulation, and absence of extra-laryngeal involvement after years of follow-up), single-fiber electromyography should be performed and myasthenia gravis treatment should be tried.

  6. Immune mechanism of thymomas in the development of myasthenia gravis

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    Wei WANG

    2013-07-01

    Full Text Available Myasthenia gravis is an acquired autoimmune disorder which is cellular-immunity-dependent, humoral-immunity¬mediated and complement participated. It is still not clear which initial factor causes the immune response. The characteristic of myasthenia gravis is that many of the patients have an abnormality in their thymus. So it is speculated that the immune response is initiated in thymus. The thymus is the central organ of the immune system, playing the key role in T cell generation, diversification and maturation. T cells are positively selected for their recognition of antigens associated with MHC expressed in the thymus, and negatively selected according to their response to the self-antigens. The result of these selection is that the T cell use the MHC expressed on the other cells of the body for recognition of foreign antigens but do not react with self-antigens. Perhaps the most important role of thymus is the induction of immune self-tolerance that functions to prevent self-harm or autoimmunity. Thymomas are functional tumors originated from epithelial of the thymus, which have the capacity to induce T cell to differentiate and mature. What mechanism is Myasthenia gravis associated thymoma initiated by? This review will summarize the existing evidence of thymus, thymoma and myasthenia gravis and try to clarify the immune mechanism of initiation of myasthenia gravis associated thymomas.

  7. Myasthenia Gravis Medication Information Card (Drugs to be Avoided or Used with Caution in Myasthenia Gravis)

    Science.gov (United States)

    MYASTHENIA GRAVIS MEDICATION INFORMATION CARD Drugs to be Avoided or Used with Caution in MG My Name _______________________________________________ Address ________________________________________________ ... the MGFA web site; reference document “Medications and Myasthenia Gravis (A Reference for Health Care Professionals.” www.myasthenia. ...

  8. Juvenile Myasthenia Gravis: A Paediatric Perspective

    Directory of Open Access Journals (Sweden)

    Maria F. Finnis

    2011-01-01

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

  9. Psychiatric disorders in myasthenia gravis

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    Mariana Inés Ybarra

    2011-04-01

    Full Text Available OBJECTIVE: To investigate the prevalence of psychiatric disorders in patients with myasthenia gravis (MG. METHOD: Forty-one patients with MG answered to a structured psychiatric interview (MINI-Plus. RESULTS: Eleven (26.1% patients were diagnosed with a depressive disorder and 19 (46.3% were diagnosed with an anxiety disorder. Patients with dysthymia were older (p=0.029 and had longer disease duration (p=0.006. Patients with social phobia also had longer disease duration (p=0.039. CONCLUSION: Psychiatric disorders in MG are common, especially depressive and anxiety disorders.

  10. Adult celiac disease with acetylcholine receptor antibody positive myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    Hugh J Freeman; Helen R Gillett; Peter M Gillett; Joel Oger

    2009-01-01

    Celiac disease has been associated with some autoimmune disorders. A 40-year-old competitive strongman with celiac disease responded to a glutenfree diet, but developed profound and generalized motor weakness with acetylcholine receptor antibody positive myasthenia gravis, a disorder reported to occur in about 1 in 5000. This possible relationship between myasthenia gravis and celiac disease was further explored in serological studies. Frozen stored serum samples from 23 acetylcholine receptor antibody positive myasthenia gravis patients with no intestinal symptoms were used to screen for celiac disease. Both endomysial and tissue transglutaminase antibodies were examined. One of 23 (or, about 4.3%) was positive for both IgA-endomysial and IgA tissue transglutaminase antibodies. Endoscopic studies subsequently showed duodenal mucosal scalloping and biopsies confirmed the histopathological changes of celiac disease. Celiac disease and myasthenia gravis may occur together more often than is currently appreciated. The presence of motor weakness in celiac disease may be a clue to occult myasthenia gravis, even in the absence of intestinal symptoms.

  11. Complement associated pathogenic mechanisms in myasthenia gravis.

    Science.gov (United States)

    Tüzün, Erdem; Christadoss, Premkumar

    2013-07-01

    The complement system is profoundly involved in the pathogenesis of acetylcholine receptor (AChR) antibody (Ab) related myasthenia gravis (MG) and its animal model experimental autoimmune myasthenia gravis (EAMG). The most characteristic finding of muscle pathology in both MG and EAMG is the abundance of IgG and complement deposits at the nerve-muscle junction (NMJ), suggesting that AChR-Ab induces muscle weakness by complement pathway activation and consequent membrane attack complex (MAC) formation. This assumption has been supported with EAMG resistance of complement factor C3 knockout (KO), C4 KO and C5 deficient mice and amelioration of EAMG symptoms following treatment with complement inhibitors such as cobra venom factor, soluble complement receptor 1, anti-C1q, anti-C5 and anti-C6 Abs. Moreover, the complement inhibitor decay accelerating factor (DAF) KO mice exhibit increased susceptibility to EAMG. These findings have brought forward improvisation of novel therapy methods based on inhibition of classical and common complement pathways in MG treatment.

  12. Stiff person syndrome and myasthenia gravis.

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    Saravanan P

    2002-01-01

    Full Text Available Association of stiff person syndrome, an immune related disorder of anterior horn cells and myasthenia gravis an endplate disorder with similar pathogenesis, is rare. This communication documents this association in the Indian literature for the first time.

  13. Myasthenia gravis developing in an HIV-negative patient with Kaposi's sarcoma.

    Science.gov (United States)

    Mantero, Vittorio; Mascolo, Maria; Bandettini di Poggio, Monica; Caponnetto, Claudia; Pardini, Matteo

    2013-07-01

    Myasthenia gravis is a disorder of neuromuscular transmission caused by autoimmune mechanisms. We reported a possible association between seropositive myasthenia gravis and Kaposi's sarcoma in a HIV-negative subject and the observed interactions between the treatment regimen for these two conditions. A 62-year-old man came to our attention for ocular myasthenia gravis. He suffered from a classic form of Kaposi's sarcoma since about 1 year. When myasthenic symptoms worsened, the patient was started on prednisone and azathioprine. The patient had a significant worsening of Kaposi's sarcoma, so prednisone and azathioprine were reduced and he was treated with vinblastine, with improvement both in dermatologic than in neurological symptomatology. We propose some considerations: the potential correlation between Kaposi's sarcoma and myasthenia gravis through immunological mechanism; myasthenia gravis as a paraneoplastic manifestation of Kaposi's sarcoma, and the role of an antitumoral agent as a treatment for both the conditions.

  14. Multiple thymoma in a patient with myasthenia gravis: case report

    Energy Technology Data Exchange (ETDEWEB)

    Ko, Eun Sook; Jeon, Kyung Nyeo; Bae, Kyung Soo; Yoo, Jin Jong [College of Medicine, Gyeongsang National Univ., Jinju (Korea, Republic of); Kang, Duk Sik [College of Medicine, Kyungpook National Univ., Daegu (Korea, Republic of)

    2004-02-01

    A thymoma often occurs in patients with myasthenia gravis, but the development of multiple thymoma is very rare. The authors report the radiologic and pathologic findings of multiple invasive thymoma in a 59-year-old male with myasthenia gravis.

  15. Familial myasthenia gravis: report of four cases

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    José Lamartine de Assis

    1976-09-01

    Full Text Available Two pairs of siblings with myasthenia gravis, belonging to two different families, are reported. This is the only record of familial myasthenia during the past twenty years, in a total of 145 patients seen at the Neurological Clinic of the São Paulo Medical School. In spite of the fact that myasthenia gravis does not show hereditary characteristics, the peculiar features of the four cases justify the present report. The two pairs of siblings were born from non myasthenic nor consanguineous parents. The disease started at birth showing bilateral partial eyelid ptosis in all patients. The course of the illness has been favorable. There was no thymoma.

  16. Metaplastic thymoma with myasthenia gravis presumably caused by an accumulation of intratumoral immature T cells: a case report.

    Science.gov (United States)

    Tajima, Shogo; Yanagiya, Masahiro; Sato, Masaaki; Nakajima, Jun; Fukayama, Masashi

    2015-01-01

    Among human neoplasms, thymomas are well known for their association with paraneoplastic autoimmune diseases such as myasthenia gravis. However, regarding rare metaplastic thymoma, only one case of an association with myasthenia gravis has been reported. Here, we present the second case of a 44-year-old woman with metaplastic thymoma associated with myasthenia gravis. In metaplastic thymoma, intratumoral terminal deoxynucleotidyl transferase-positive T-cells (immature T-cells) are generally scarce, while they were abundant in the present case. We believe that these immature T-cells could be related to the occurrence of myasthenia gravis.

  17. CD1d(hi)CD5+ B cells expanded by GM-CSF in vivo suppress experimental autoimmune myasthenia gravis.

    Science.gov (United States)

    Sheng, Jian Rong; Quan, Songhua; Soliven, Betty

    2014-09-15

    IL-10-competent subset within CD1d(hi)CD5(+) B cells, also known as B10 cells, has been shown to regulate autoimmune diseases. Whether B10 cells can prevent or suppress the development of experimental autoimmune myasthenia gravis (EAMG) has not been studied. In this study, we investigated whether low-dose GM-CSF, which suppresses EAMG, can expand B10 cells in vivo, and whether adoptive transfer of CD1d(hi)CD5(+) B cells would prevent or suppress EAMG. We found that treatment of EAMG mice with low-dose GM-CSF increased the proportion of CD1d(hi)CD5(+) B cells and B10 cells. In vitro coculture studies revealed that CD1d(hi)CD5(+) B cells altered T cell cytokine profile but did not directly inhibit T cell proliferation. In contrast, CD1d(hi)CD5(+) B cells inhibited B cell proliferation and its autoantibody production in an IL-10-dependent manner. Adoptive transfer of CD1d(hi)CD5(+) B cells to mice could prevent disease, as well as suppress EAMG after disease onset. This was associated with downregulation of mature dendritic cell markers and expansion of regulatory T cells resulting in the suppression of acetylcholine receptor-specific T cell and B cell responses. Thus, our data have provided significant insight into the mechanisms underlying the tolerogenic effects of B10 cells in EAMG. These observations suggest that in vivo or in vitro expansion of CD1d(hi)CD5(+) B cells or B10 cells may represent an effective strategy in the treatment of human myasthenia gravis.

  18. An occasional diagnosis of myasthenia gravis - a focus on thymus during cardiac surgery: a case report

    Directory of Open Access Journals (Sweden)

    Dainese Luca

    2009-10-01

    Full Text Available Abstract Background Myasthenia gravis, an uncommon autoimmune syndrome, is commonly associated with thymus abnormalities. Thymomatous myasthenia gravis is considered to have worst prognosis and thymectomy can reverse symptoms if precociously performed. Case report We describe a case of a patient who underwent mitral valve repair and was found to have an occasional thymomatous mass during the surgery. A total thymectomy was performed concomitantly to the mitral valve repair. Conclusion The diagnosis of thymomatous myasthenia gravis was confirmed postoperatively. Following the surgery this patient was strictly monitored and at 1-year follow-up a complete stable remission had been successfully achieved.

  19. Immune disease and HLA associations with myasthenia gravis.

    Science.gov (United States)

    Behan, P O

    1980-07-01

    In the late 1950's laboratory and clinical evidence suggested that myasthenia gravis was an autoimmune disorder. Since then a voluminous literature has developed documenting the many immunological abnormalities that occur in this condition. Recent findings point to a central disorder of immunoregulation. It is postulated that the disease occurs as a result of host genetic and environmental influences-the latter being, as yet unidentified and possibly a virus.

  20. Maximal bite force and surface EMG in patients with myasthenia gravis

    NARCIS (Netherlands)

    Weijnen, FG; Wokke, JHJ; Kuks, JBM; van der Glas, HW; Bosman, F

    2000-01-01

    Masticatory muscle strength was quantified in patients with bulbar myasthenia gravis and compared with that of patients with ocular myasthenia gravis, patients in clinical remission (whether or not pharmacological) who previously suffered from bulbar myasthenia gravis, and healthy subjects. Maximal

  1. Myasthenia Gravis: a population-based epidemiological study.

    LENUS (Irish Health Repository)

    Rutledge, S

    2016-02-01

    Myasthenia Gravis (MG) is a disorder affecting components of the neuromuscular junction. Epidemiological studies show rising incidence and prevalence rates. The aim of this study was to determine the incidence and prevalence of MG in the Republic of Ireland. Data sources included patient lists from consultant neurologists and ophthalmologists, a neuroimmunology laboratory, general practitioners and the Myasthenia Gravis Association. A total of 1,715 cases were identified, of which 706 definite, probable or possible autoimmune and congenital MG cases were included. The overall prevalence rate from the data obtained is 15.38\\/100,000. The study demonstrated a female preponderance (female:male of 1.3: 1) and some geographical variation within Ireland. The average incidence rate for the years 2000 to 2009 was 11.3 per year; the rate for the current decade is 18 per year. The increasing number of diagnoses may be due to improved access to diagnostic investigations and increasing awareness of the clinical manifestations.

  2. Third caesarean section in patient with myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    A Pizzo; R De Dominici; I Borrielli; FV Ardita; I Lo Monaco; E Sturlese

    2012-01-01

    Myasthenia gravis(MG) is an autoimmune neuromuscular disease, characterised by muscle weakness and fatigability of the voluntary muscles, it affects young women in the second and third decade of life.We report a case of30 years old multigravida woman with myasthenia gravis submitted to third iterative caesarean section with no adverse neonatal and maternal outcome. The course myathenia is highly variable and unpredictable during gestation and can change in subsequent pregnancies.Delivery specially, through caesarean section is very stressful and may cause severe myasthenic crisis.Although pregnancy and delivery represent particular events, that require more attention in these patients, they are not associated, in most cases, with higher risks of complications compared to normal pregnancy, delivery and postpartum period.According our experiences, in the management of myasthenic pregnant woman is necessary cooperation in a multidisciplinary team between obstetricians, neurologist, anaesthetist, and neonatologist for ensure an optimum outcome.

  3. [The history of myasthenia gravis. Men and ideas.

    Science.gov (United States)

    Mimenza-Alvarado, A; Tellez-Zenteno, Jf; Garcia-Ramos, G; Estañol, B

    2007-06-28

    The first description of a patient with myasthenia gravis was done by Thomas Willis (1621-1675). He was an eminent professor of natural history at Oxford University who also described the arteries of the brain and made the first precise drawings of it. At the present time myasthenia gravis is considered one of the most well described autoimmune diseases with great advances in its diagnosis, pathophysiology and treatment. In this review we summarize the most important events and ideas in the history of this disease since the original description by Willis; mention the most important clinicians, anatomists and physiologists that were concerned with its understanding and make reference of some the most recent advances in its diagnosis and treatment and finally discuss some present controversies. Neurología 2007;22(0):0-0.

  4. CT evaluation of thymus in myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Guk Hee [Insung Hospital, Chuncheon (Korea, Republic of); Kang, Eun Young; Lee, Nam Joon; Suh, Won Hyuck [Korea University College of Medicine, Seoul (Korea, Republic of)

    1989-12-15

    The relationship between myasthenia gravis and the thymus was well establish and myasthenia gravis occurs in the presence of thymic hyperplasia or thymoma or occasionally in histologically normal thymus. Since not every patients with myasthenia gravis is a candidate for thymectomy, unless a thymoma is present, the differentiation of normal and hyperplastic thymus from thymoma becomes important. Authors reviewed retrospectively clinical records and chest CT scans of 18 patients with myasthenia gravis who underwent thymectomy during recent 5 years, to evaluate the role of CT scan. The results were as follows. 1 Of total 18 cases, 5 cases had normal thymus, 6 cases had thymic hyperplasia, 4 cases had benign thymoma and 3 cases had malignant thymoma. 2. Of 5 cases of normal thymus, no false positive cases were noted in CT scan. 3. Of 6 cases of thymic hyperplasia, CT findings were normal except 1 cases of thickened left thymic lobe. 4. Of 7 cases of thymoma, no false negative cases were noted in CT scan. 5. CT findings of benign thymoma were round or oval shaped, discrete, slightly enhancing soft tissue mass in anterior mediastinum. 6. CT findings of malignant thymoma were lobulated contoured, infiltrative, soft tissue mass lesion in anterior mediastinum with calcifications, pleural tumor implants, and SVC compression. CT yielded valuable information on differential diagnosis of thymoma, thymic hyperplasia and normal thymus. Also CT was a highly sensitive method in the detection of thymoma and determining the extent and invasiveness.

  5. Association of myasthenia gravis and Behçet's disease: A case report.

    Science.gov (United States)

    Kisabay, Aysin; Sari, Ummu Serpil; Boyaci, Recep; Batum, Melike; Yilmaz, Hikmet; Selcuki, Deniz

    2016-01-01

    Myasthenia gravis is a disease of neuromuscular junction due to auto-immune destruction of the acetylcholine receptors. Behçet's disease, on the other hand, is a multisystemic vascular-inflammatory disease. Both conditions are not common in the general population although their association has not been reported in the literature. We wanted to present our patient who developed clinical course of myasthenia gravis following discontinuation of medications due to complications of corticosteroid for Behçet's disease. It was observed that clinical findings of myasthenia gravis recovered following restarting steroid treatment and he did not experience attacks of both conditions. Although Myasthenia gravis and Behçet's disease are distinct entities clinically as well as in terms of pathogenesis, they share common physiopathological features and their treatment is based on their common features.

  6. Vocal cord palsy: An uncommon presenting feature of myasthenia gravis

    Directory of Open Access Journals (Sweden)

    Sethi Prahlad

    2011-01-01

    Full Text Available Vocal cord palsy can have myriad causes. Unilateral vocal cord palsy is common and frequently asymptomatic. Trauma, head, neck and mediastinal tumors as well as cerebrovascular accidents have been implicated in causing unilateral vocal cord palsy. Viral neuronitis accounts for most idiopathic cases. Bilateral vocal cord palsy, on the other hand, is much less common and is a potentially life-threatening condition. Myasthenia gravis, an autoimmune disorder caused by antibodies targeting the post-synaptic acetylcholine receptor, has been infrequently implicated in its causation. We report here a case of bilateral vocal cord palsy developing in a 68-year-old man with no prior history of myasthenia gravis 2 months after he was operated on for diverticulitis of the large intestine. Delay in considering the diagnosis led to endotracheal intubation and prolonged mechanical ventilation with attendant complications. Our case adds to the existing literature implicating myasthenia gravis as an infrequent cause of bilateral vocal cord palsy. Our case is unusual as, in our patient, acute-onset respiratory distress and stridor due to bilateral vocal cord palsy was the first manifestation of a myasthenic syndrome.

  7. Current aspects of the clinic, diagnosis and treatment of acquired myasthenia gravis (review. Part 1: diagnostics

    Directory of Open Access Journals (Sweden)

    S. N. Bardakov

    2016-01-01

    Full Text Available Acquired myasthenia gravis is an autoimmune disease, based on the imbalance of humoral and cellular immunity, characterized by weakness and fatigue of skeletal muscles, and in some cases involvement of the heart muscle. In most cases, the object of the autoimmune destruction is peripheral nicotinic acetylcholine receptors of the postsynaptic membrane of the neuromuscular synapse. In myasthenia gravis there has been identified a number of other antigenic targets: muscle-specific tyrosine kinase, lowdensity lipoprotein receptor-related protein 4, titin, and ryanodine receptors skeletal muscles. Their specificity and pathogenetic role have been disclosed, but it is about 15-20% of seronegative forms that require further immunological research. Accurate diagnosis depends on the efficient detection of clinical forms of myasthenia gravis and full analysis of the autoimmune mechanisms underlying the disease.

  8. Deficits in Endogenous Adenosine Formation by Ecto-5′-Nucleotidase/CD73 Impair Neuromuscular Transmission and Immune Competence in Experimental Autoimmune Myasthenia Gravis

    Directory of Open Access Journals (Sweden)

    Laura Oliveira

    2015-01-01

    Full Text Available AMP dephosphorylation via ecto-5′-nucleotidase/CD73 is the rate limiting step to generate extracellular adenosine (ADO from released adenine nucleotides. ADO, via A2A receptors (A2ARs, is a potent modulator of neuromuscular and immunological responses. The pivotal role of ecto-5′-nucleotidase/CD73, in controlling extracellular ADO formation, prompted us to investigate its role in a rat model of experimental autoimmune myasthenia gravis (EAMG. Results show that CD4+CD25+FoxP3+ regulatory T cells express lower amounts of ecto-5′-nucleotidase/CD73 as compared to controls. Reduction of endogenous ADO formation might explain why proliferation of CD4+ T cells failed upon blocking A2A receptors activation with ZM241385 or adenosine deaminase in EAMG animals. Deficits in ADO also contribute to neuromuscular transmission failure in EAMG rats. Rehabilitation of A2AR-mediated immune suppression and facilitation of transmitter release were observed by incubating the cells with the nucleoside precursor, AMP. These findings, together with the characteristic increase in serum adenosine deaminase activity of MG patients, strengthen our hypothesis that the adenosinergic pathway may be dysfunctional in EAMG. Given that endogenous ADO formation is balanced by ecto-5′-nucleotidase/CD73 activity and that A2ARs exert a dual role to restore use-dependent neurocompetence and immune suppression in myasthenics, we hypothesize that stimulation of the two mechanisms may have therapeutic potential in MG.

  9. Epidemiology of myasthenia gravis in Ontario, Canada.

    Science.gov (United States)

    Breiner, Ari; Widdifield, Jessica; Katzberg, Hans D; Barnett, Carolina; Bril, Vera; Tu, Karen

    2016-01-01

    Incidence and prevalence estimates in myasthenia gravis have varied widely. Recent studies based on administrative health data have large sample sizes but lack rigorous validation of MG cases, and have not examined the North American population. Our aim was to explore trends in MG incidence and prevalence for the years 1996-2013 in the province of Ontario, Canada (population 13.5 million). We employed a previously validated algorithm to identify MG cases. Linking with census data allowed for the calculation of crude- and age/sex-standardized incidence and prevalence rates for the years 1996-2013. The regional distribution of MG cases throughout the province was examined. Mean age at the first myasthenia gravis encounter was 60.2 ± 17.1 years. In 2013, there were 3611 prevalent cases in Ontario, and the crude prevalence rate was 32.0/100,000 population. Age- and sex-standardized prevalence rates rose consistently over time from 16.3/100,000 (15.4-17.1) in 1996 to 26.3/100,000 (25.4-27.3) in 2013. Standardized incidence rates remained stable between 1996 (2.7/100,000; 95% CL 2.3-3.0) and 2013 (2.3/100,000; 2.1-2.6). Incidence was highest in younger women and older men, and geographic variation was evident throughout the province. In conclusion, this large epidemiological study shows rising myasthenia gravis prevalence with stable incidence over time, which is likely reflective of patients living longer, possibly due to improved disease treatment. Our findings provide accurate information on the Canadian epidemiology of myasthenia gravis and burden for health care resources planning for the province, respectively.

  10. A study of the utility of azathioprine metabolite testing in myasthenia gravis.

    Science.gov (United States)

    Rae, William; Burke, Georgina; Pinto, Ashwin

    2016-04-15

    Myasthenia gravis (MG) is an autoimmune neuromuscular disorder characterised by fatigable voluntary skeletal muscle weakness. The underlying pathogenesis is complex involving adaptive autoimmune responses. Azathioprine remains a first line broad acting immunosuppressant for MG. Due to varied clinical responses to azathioprine we aimed to investigate the relationship between azathioprine metabolites and symptom control. Mild correlations between Quantitative Myasthenia Gravis Score (QMG) vs. 6-thioguanine nucleotides (R=-0.317 p=0.186) and QMG vs. lymphocyte count (R=0.402 p=0.08) were found. Azathioprine metabolite measurement should be considered in MG patients with; pancytopenia, deranged liver function or recurrent infections.

  11. AN ELECTROPHYSIOLOGICAL INVESTIGATION OF NEUROMUSCULAR TRANSMISSION IN MYASTHENIA GRAVIS,

    Science.gov (United States)

    with myasthenia gravis . With repetitive nerve stimulation at frequencies above 2/sec, only the first few stimuli elicited muscle contractions in most...It is tentatively concluded that in myasthenia gravis there is a deficiency in the amount of ACh in the quanta of transmitter released from the motor nerve terminals. (Author)

  12. Survivin as a potential mediator to support autoreactive cell survival in myasthenia gravis: a human and animal model study.

    Directory of Open Access Journals (Sweden)

    Linda L Kusner

    Full Text Available The mechanisms that underlie the development and maintenance of autoimmunity in myasthenia gravis are poorly understood. In this investigation, we evaluate the role of survivin, a member of the inhibitor of apoptosis protein family, in humans and in two animal models. We identified survivin expression in cells with B lymphocyte and plasma cells markers, and in the thymuses of patients with myasthenia gravis. A portion of survivin-expressing cells specifically bound a peptide derived from the alpha subunit of acetylcholine receptor indicating that they recognize the peptide. Thymuses of patients with myasthenia gravis had large numbers of survivin-positive cells with fewer cells in the thymuses of corticosteroid-treated patients. Application of a survivin vaccination strategy in mouse and rat models of myasthenia gravis demonstrated improved motor assessment, a reduction in acetylcholine receptor specific autoantibodies, and a retention of acetylcholine receptor at the neuromuscular junction, associated with marked reduction of survivin-expressing circulating CD20+ cells. These data strongly suggest that survivin expression in cells with lymphocyte and plasma cell markers occurs in patients with myasthenia gravis and in two animal models of myasthenia gravis. Survivin expression may be part of a mechanism that inhibits the apoptosis of autoreactive B cells in myasthenia gravis and other autoimmune disorders.

  13. Survivin as a potential mediator to support autoreactive cell survival in myasthenia gravis: a human and animal model study.

    Science.gov (United States)

    Kusner, Linda L; Ciesielski, Michael J; Marx, Alexander; Kaminski, Henry J; Fenstermaker, Robert A

    2014-01-01

    The mechanisms that underlie the development and maintenance of autoimmunity in myasthenia gravis are poorly understood. In this investigation, we evaluate the role of survivin, a member of the inhibitor of apoptosis protein family, in humans and in two animal models. We identified survivin expression in cells with B lymphocyte and plasma cells markers, and in the thymuses of patients with myasthenia gravis. A portion of survivin-expressing cells specifically bound a peptide derived from the alpha subunit of acetylcholine receptor indicating that they recognize the peptide. Thymuses of patients with myasthenia gravis had large numbers of survivin-positive cells with fewer cells in the thymuses of corticosteroid-treated patients. Application of a survivin vaccination strategy in mouse and rat models of myasthenia gravis demonstrated improved motor assessment, a reduction in acetylcholine receptor specific autoantibodies, and a retention of acetylcholine receptor at the neuromuscular junction, associated with marked reduction of survivin-expressing circulating CD20+ cells. These data strongly suggest that survivin expression in cells with lymphocyte and plasma cell markers occurs in patients with myasthenia gravis and in two animal models of myasthenia gravis. Survivin expression may be part of a mechanism that inhibits the apoptosis of autoreactive B cells in myasthenia gravis and other autoimmune disorders.

  14. Computer Aided Study of Mechanism of Immunoadsorbent for Myasthenia Gravis

    Institute of Scientific and Technical Information of China (English)

    2003-01-01

    Myasthenia Gravis (MG) is an organ specific autoimmune disease mediated by autoantibodies (AChR Ab) against the acetylcholine receptor (AChR). Literature reported that the AChR Ab can be removed by absorbent linked with tryptophan. It was studied in detail in our lab. With the aid of computer, we docked some ligands into AChR Ab, and the results from scores of docking under different generations showed that there was no specific binding between tryptophan and scFv fragment just as the binding between antigen-antibody. The interaction between Trp and immunoglobulin was a broad-spectrum binding.

  15. Steroids induce acetylcholine receptors on cultured human muscle: Implications for myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Kaplan, I.; Blakely, B.T.; Pavlath, G.K.; Travis, M.; Blau, H.M. (Stanford Univ. School of Medicine, CA (USA))

    1990-10-01

    Antibodies to the acetylcholine receptor (AChR), which are diagnostic of the human autoimmune disease myasthenia gravis, block AChR function and increase the rate of AChR degradation leading to impaired neuromuscular transmission. Steroids are frequently used to alleviate symptoms of muscle fatigue and weakness in patients with myasthenia gravis because of their well-documented immunosuppressive effects. The authors show here that the steroid dexamethasone significantly increases total surface AChRs on cultured human muscle exposed to myasthenia gravis sera. The results suggest that the clinical improvement observed in myasthenic patients treated with steroids is due not only to an effect on the immune system but also a direct effect on muscle. They propose that the identification and development of pharmacologic agents that augment receptors and other proteins that are reduced by human genetic or autoimmune disease will have broad therapeutic applications.

  16. Myasthenia gravis in pediatric and elderly patients

    Institute of Scientific and Technical Information of China (English)

    刘伟; 刘国津; 范志民; 盖学良

    2003-01-01

    Objective To determine whether the clinical and pathologic characteristics and prognoses of myasthenia gravis (MG) patients below 15 years differ from those patients over 50 years after thymectomy. Methods We reviewed the registry material of 30 pediatric and 32 elderly MG patients after thymectomy, including their age, sex, clinical classification, pathological types, and prognoses. The Chi-square test or Wilcoxon ' s rank-sum test was used to determine the statistical differences between the children and elderly groups.Results No significant difference was seen in sex distribution between the two groups (Chi-square test, P=0.625), but there were differences in clinical classification: more type Ⅰ was observed in the pediatric group than in the elderly group, but more type Ⅱor Ⅲ was seen in the elderly group (Wilcoxon ' s rank-sum test, P<0.001). As to pathological types, the pediatric group was also significantly different from the elderly group (Chi-square test, P<0.01). All of the patients (100%) in the pediatric group had thymus hyperplasia, but in the elderly group more than half (56.26%) were found to have thymoma (benign or malignant). The prognoses after thymectomy were better in the pediatric group than in the elderly group (Wilcoxon ' s rank-sum test, P<0.001). Conclusions Because the prognoses are generally better than those of the elderly patients, we should be careful when operating on pediatric patients of ocular type. The elderly patients tend to receive more aggressive treatment because of more severe generalized types often associated with thymoma and poor prognoses. Both pediatric and elderly patients are seldom associated with other autoimmune disease.

  17. Chlorpromazine induced ocular myasthenia gravis

    Directory of Open Access Journals (Sweden)

    Nitya S

    2013-10-01

    Full Text Available Drug induced bilateral ptosis is a very rare adverse drug reaction. Here we report a case of ten year old male child with chlorpromazine induced bilateral ptosis due to ocular myasthenia. [Int J Basic Clin Pharmacol 2013; 2(5.000: 653-654

  18. Treatment of experimental myasthenia gravis with total lymphoid irradiation

    Energy Technology Data Exchange (ETDEWEB)

    de Silva, S.; Blum, J.E.; McIntosh, K.R.; Order, S.; Drachman, D.B.

    1988-07-01

    Total lymphoid irradiation (TLI) has been reported to be effective in the immunosuppressive treatment of certain human and experimental autoimmune disorders. We have investigated the effects of TLI in Lewis rats with experimental autoimmune myasthenia gravis (EAMG) produced by immunization with purified torpedo acetylcholine receptor (AChR). The radiation is given in 17 divided fractions of 200 rad each, and nonlymphoid tissues are protected by lead shielding. This technique suppresses the immune system, while minimizing side effects, and permits the repopulation of the immune system by the patient's own bone marrow cells. Our results show that TLI treatment completely prevented the primary antibody response to immunization with torpedo AChR, it rapidly abolished the ongoing antibody response in established EAMG, and it suppressed the secondary (anamnestic) response to a boost of AChR. No EAMG animals died during TLI treatment, compared with six control animals that died of EAMG. TLI produces powerful and prompt immunosuppression and may eventually prove useful in the treatment of refractory human myasthenia gravis.

  19. Immunoglobulin G4-Related Inflammatory Abdominal Aortic Aneurysm Associated With Myasthenia Gravis, With Contained Rupture.

    Science.gov (United States)

    Jun, Heungman; Jung, Cheol Woong

    2016-11-01

    Immunoglobulin (Ig) G4-related disease is reportedly among the various causes of inflammatory abdominal aortic aneurysm (IAAA). Many IgG4-related diseases are closely related to allergic constitution and autoimmune disease. We report a case of a 72-year-old man with IgG4-related IAAA associated with myasthenia gravis, with contained rupture.

  20. An update on laboratory diagnosis in myasthenia gravis.

    Science.gov (United States)

    Oger, Joel; Frykman, Hans

    2015-09-20

    This review describes the state of the art for the use of laboratory testing in myasthenia gravis. The review brings a detailed description of the different clinical forms of auto-immune myasthenia and of the Lambert Eaton Myasthenic Syndrome (LEMS). They stress the differences between the different forms of acquired (auto-immune) myasthenia. Then they present a summary of the different antibodies found in the disease. They insist on the advantage of the RIPA assay to measure antibodies to the acetylcholine receptor. They stress the different types of contribution of each of these antibodies to the clinical diagnosis. They also describe the methods to measure each of the specific antibodies that have recently permitted to split the diagnosis: Abs to omega-conotoxin receptor in Lambert Eaton Myasthenic Syndrome (LEMS), abs to the acetylcholine receptor (AchR) in MG, Abs to muscle specific tyrosine kinase (MuSK) in Ab negative MG, and Abs to low molecular weight receptor related low-density lipo protein-4 (LRP-4). They also broach over the striated antibodies, less frequent and clinically less useful such as anti-titin, -ryanodine, -agrin and -rapsyn. This represent a 360° view of the field as presented in Toronto in October 2014.

  1. Emergency Management of Myasthenia Gravis

    Science.gov (United States)

    ... autoimmune neuromuscular disorder. It is characterized by fluctuating weakness of voluntary muscle groups. Muscle groups most commonly affected include the eye muscles, facial, chewing and swallowing muscles, and shoulder and hip muscles. Muscles that assist breathing may ...

  2. Myasthenia gravis and related disorders: Pathology and molecular pathogenesis.

    Science.gov (United States)

    Ha, James C; Richman, David P

    2015-04-01

    Disorders affecting the presynaptic, synaptic, and postsynaptic portions of the neuromuscular junction arise from various mechanisms in children and adults, including acquired autoimmune or toxic processes as well as genetic mutations. Disorders include autoimmune myasthenia gravis associated with acetylcholine receptor, muscle specific kinase or Lrp4 antibodies, Lambert-Eaton myasthenic syndrome, nerve terminal hyperexcitability syndromes, Guillain Barré syndrome, botulism, organophosphate poisoning and a number of congenital myasthenic syndromes. This review focuses on the various molecular and pathophysiological mechanisms of these disorders, characterization of which has been crucial to the development of treatment strategies specific for each pathogenic mechanism. In the future, further understanding of the underlying processes may lead to more effective and targeted therapies of these disorders. This article is part of a Special Issue entitled: Neuromuscular Diseases: Pathology and Molecular Pathogenesis.

  3. 重症肌无力合并自身免疫病的临床研究%Associated autoimmune diseases in myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    管宇宙; 崔丽英

    2012-01-01

    目的 分析重症肌无力(MG)合并自身免疫病的种类、发病年龄和临床特点.方法 回顾性分析作者医院2008-01-2012-01住院的MG患者,包括合并自身免疫病的MG患者的临床资料、影像学及实验室检查资料及治疗记录.结果 共收集MG患者305例,合并自身免疫病1种或1种以上者28例(9.18%),其中男9例、女19例,性别构成与非合并免疫病者无统计学差异(P>0.05).自身免疫病在MG之前发病者12例,平均(69.6±35.3)个月;在MG发病之后者14例,平均(72.4±38.4)个月;与MG同时发现2例;(1)MG合并自身免疫病组的MG发病年龄为(33.9±9.4)岁,与非合并自身免疫病患者的MG发病年龄[(35.3±12.2)岁]无统计学差异(P>0.05).合并甲状腺功能亢进(甲亢)患者MG发病年龄(27.4±12.5)岁,较合并其他自身免疫病患者的MG发病年龄[(41.6±10.3)岁]和非合并自身免疫病患者(35.3±12.2岁)均低(均P<0.05).(2)合并的自身免疫病中属甲亢最多(13例),其次为类风湿性关节炎(5例)、桥本甲状腺炎(4例)、吉兰-巴雷综合征(3例).其中有3例合并2种自身免疫病,1例合并3种自身免疫病.(3)合并自身免疫病MG患者的Osserman分型:发病初期Ⅰ型13例,Ⅱa型3例,Ⅱb型10例,Ⅲ型2例;其中MG合并甲亢患者以Ⅰ型发病10例,超过合并其他自身免疫病MG患者(P<0.05).结论 MG可合并多种神经系统或全身自身免疫病,以甲亢最多见.合并甲亢的MG患者中MG发病年龄较轻,以跟肌型多见.自身免疫病可在MG发病前、后或同时期出现.%Objective To investigate the coincidence and clinical features of associated autoimmune diseases in patients with myasthenia gravis. Methods All case records had been collected from admitted MG patients including those with associated autoimmune diseases from 2008-01 to 2012-01, Their clinical data, imaging studies and laboratory examination results were retrospectively analyzed. Results Three hundred and five

  4. Current and emerging therapies for the treatment of myasthenia gravis

    Directory of Open Access Journals (Sweden)

    Renato Mantegazza

    2011-03-01

    Full Text Available Renato Mantegazza, Silvia Bonanno, Giorgia Camera, Carlo AntozziDepartment of Neuromuscular Diseases and Neuroimmunology, Fondazione Istituto Neurologico Carlo Besta, Milan, ItalyAbstract: Myasthenia gravis (MG is an autoimmmune disease in which autoantibodies to different antigens of the neuromuscular junction cause the typical weakness and fatigability. Treatment includes anticholinesterase drugs, immunosuppression, immunomodulation, and thymectomy. The autoimmune response is maintained under control by corticosteroids frequently associated with immunosuppressive drugs, with improvement in the majority of patients. In case of acute exacerbations with bulbar symptoms or repeated relapses, modulation of autoantibody activity by plasmapheresis or intravenous immunoglobulins provides rapid improvement. Recently, techniques removing only circulating immunoglobulins have been developed for the chronic management of treatment-resistant patients. The rationale for thymectomy relies on the central role of the thymus. Despite the lack of controlled studies, thymectomy is recommended as an option to improve the clinical outcome or promote complete remission. New videothoracoscopic techniques have been developed to offer the maximal surgical approach with the minimal invasiveness and hence patient tolerability. The use of biological drugs such as anti-CD20 antibodies is still limited but promising. Studies performed in the animal model of MG demonstrated that several more selective or antigen-specific approaches, ranging from mucosal tolerization to inhibition of complement activity or cellular therapy, might be feasible. Investigation of the transfer of these therapeutic approaches to the human disease will be the challenge for the future.Keywords: myasthenia gravis, therapy, immunosuppression, thymectomy, plasmapheresis

  5. Clinical and electrophysiological evaluation of dysphagia in myasthenia gravis

    OpenAIRE

    1998-01-01

    OBJECTIVE—To evaluate dysphagia at the oropharyngeal stage of swallowing and to determine the pathophysiological mechanisms of dysphagia in patients with myasthenia gravis.
METHODS—Fifteen patients with myasthenia gravis with dysphagia and 10 patients without dysphagia were investigated by a combined electrophysiological and mechanical method described previously. Laryngeal movements were detected by a piezoelectric transducer and the related submental EMG (SM-EMG) and somet...

  6. Myasthenia Gravis – a beginning with no end

    Directory of Open Access Journals (Sweden)

    Croitoru Cristina Georgiana

    2016-03-01

    Full Text Available Myasthenia gravis is one of the neurological diseases with a relatively recent history, full of mistakes, in which the British and German neurology schools have attempted to find answers when confronted with the unknown. The paper aims at making a historical account of the disease from its discovery in the 16th century, when the first case of myasthenia gravis was medically diagnosed, to the beginning of the 20th century, when the dawn of modern therapy started to show.

  7. Anaesthetic Management of Myasthenia Gravis with Thymic Mass in Facial Trauma

    OpenAIRE

    2010-01-01

    Myasthenia gravis is an immunologic disorder characterised by polyclonal antibodies directed against nicotinic acetylcholine receptors at postneuromuscular junction. This case describes perioperative management of a patient suffering facial trauma with underlying myasthenia gravis with thymic mass

  8. Worsening of myasthenia gravis after administration of injectable long-acting risperidone for treatment of schizophrenia; first case report and a call for caution.

    Science.gov (United States)

    Al-Hashel, Jasem Y; Ismail, Ismail Ibrahim; John, John K; Ibrahim, Mohammed; Ali, Mahmoud

    2016-01-01

    Myasthenia gravis is an autoimmune disease characterized by muscle weakness due to autoantibodies affecting the neuromuscular junction. Co-occurrence of myasthenia gravis and schizophrenia is very rare and raises a challenge in management of both diseases. Antipsychotic drugs exhibit anticholinergic side effects and have the potentials of worsening myasthenia. Long-acting risperidone is an injectable atypical antipsychotic drug that has not been previously reported to worsen myasthenia gravis in literature. We report the first case report of worsening of myasthenia after receiving long-acting risperidone injection for schizophrenia in a 29-year-old female with both diseases. She started to have worsening 2 weeks following the first injection and her symptoms persisted despite receiving plasma exchange. This could be explained by the pharmacokinetics of the drug. We recommend that long-acting risperidone should be used with caution in patients with myasthenia gravis, and clinicians must be aware of the potential risks of this therapy.

  9. Myasthenia gravis and pregnancy: clinical implications and neonatal outcome

    Directory of Open Access Journals (Sweden)

    Estanol Bruno

    2004-11-01

    Full Text Available Abstract Background The myasthenia gravis is twice as common in women as in men and frequently affects young women in the second and third decades of life, overlapping with the childbearing years. Generally, during pregnancy in one third of patients the disease exacerbates, whereas in two thirds it remains clinically unchanged. Complete remission can occur in some patients. Methods To describe the clinical course, delivery and neonatal outcome of 18 pregnant women with the diagnosis of myasthenia gravis. Retrospective chart review of pregnant patients with myasthenia gravis, followed at the National Institute of Perinatology in Mexico City over an 8-year period. Data was abstracted from the medical records on the clinical course during pregnancy, delivery and neonatal outcome. Results From January 1, 1996 to December 31, 2003 18 patients with myasthenia gravis were identified and included in the study. The mean ± SD maternal age was 27.4 ± 4.0 years. During pregnancy 2 women (11% had an improvement in the clinical symptoms of myasthenia gravis, 7 women (39% had clinical worsening of the condition of 9 other patients (50% remained clinically unchanged. Nine patients delivered vaginally, 8 delivered by cesarean section and 1 pregnancy ended in fetal loss. Seventeen infants were born at mean ± SD gestational age of 37.5 ± 3.0 weeks and a mean birth weight of 2710 ± 73 g. Only one infant presented with transient neonatal myasthenia gravis. No congenital anomalies were identified in any of the newborns. Conclusions The clinical course of myasthenia gravis during pregnancy is variable, with a significant proportion of patients experiencing worsening of the clinical symptoms. However, neonatal transient myasthenia was uncommon in our patient population.

  10. Clinical features of myasthenia gravis in southern China: a retrospective review of 2,154 cases over 22 years.

    Science.gov (United States)

    Huang, X; Liu, W B; Men, L N; Feng, H Y; Li, Y; Luo, C M; Qiu, L

    2013-06-01

    The objectives of the study are to study the clinical features of myasthenia gravis in southern China. A retrospective study was carried out on all patients who were diagnosed with myasthenia gravis at the First Affiliated Hospital of Sun Yat-sen University during 1987-2009. Of the 2,154 myasthenia gravis patients, the gender ratio (male:female) was 1:1.15. The median age at onset was 18 years. There was a single peak distribution of age at onset, and 44.8 % were children (≤ 14 years) at first onset. 1,766 patients (82.0 %) only had ocular symptoms at onset. 1,451 patients (67.4 %) were classified as Osserman grade I. 250 unselected patients received anti-acetylcholine receptor antibodies test, in which only 51.2 % were positive. Computed tomography scan/magnetic resonance Imaging of chest were done in 1,354 patients, of which 899 patients (66.4 %) had thymic hyperplasia and 201(14.8 %) had thymoma. There were 150 patients (7.0 %) with myasthenia gravis combined with other autoimmune diseases, in which hyperthyroidism was most common (84 %). 189 (8.8 %) patients experienced 267 episodes of crisis. The rate of family myasthenia gravis was 1.6 % (35/2,154). In conclusion, the clinical features and demography of myasthenia gravis patients in this study are significantly different from prior studies on other regions and ethnic groups.

  11. Myasthenia gravis: a clinical-immunological update.

    Science.gov (United States)

    Binks, Sophie; Vincent, Angela; Palace, Jacqueline

    2016-04-01

    Myasthenia gravis (MG) is the archetypic disorder of both the neuromuscular junction and autoantibody-mediated disease. In most patients, IgG1-dominant antibodies to acetylcholine receptors cause fatigable weakness of skeletal muscles. In the rest, a variable proportion possesses antibodies to muscle-specific tyrosine kinase while the remainder of seronegative MG is being explained through cell-based assays using a receptor-clustering technique and, to a lesser extent, proposed new antigenic targets. The incidence and prevalence of MG are increasing, particularly in the elderly. New treatments are being developed, and results from the randomised controlled trial of thymectomy in non-thymomatous MG, due for release in early 2016, will be of particular clinical value. To help navigate an evidence base of varying quality, practising clinicians may consult new MG guidelines in the fields of pregnancy, ocular and generalised MG (GMG). This review focuses on updates in epidemiology, immunology, therapeutic and clinical aspects of GMG in adults.

  12. Neuromyelitis optica and myasthenia gravis in a young Nigerian girl.

    Science.gov (United States)

    Balarabe, Salisu Abdullahi; Adamu, Mohammad Dantani; Watila, Musa Mamman; Jiya, Nma

    2015-09-03

    Neuromyelitis optica (NMO) and myasthenia gravis (MG) are rare autoimmune disorders. The coexistence of the two disorders, although rare, has been documented. This is a case report of a 16-year-old student who presented with recurrent episodes of transverse myelitis and optic neuritis, 8 years after diagnosis of MG. She presented with visual impairment, relapsing and remitting weakness, numbness and paraesthesia of her lower limbs, with bladder and bowel incontinence. Her examination revealed bilateral optic atrophy, spastic paraparesis of the lower limbs and patchy sensory loss up to thoracic level (T4-5). She had a positive acetylcholine receptor antibody, a positive aquaporin-4 antibody and chest CT finding of thymic enlargement. We therefore confirmed the previous diagnosis of MG and performed a recent diagnosis of background NMO. A high index of suspicion is needed to make a diagnosis of this rare coexistence of NMO and MG in resource-limited settings such as Nigeria.

  13. Severe Preeclampsia in the Setting of Myasthenia Gravis

    Science.gov (United States)

    Keeney, Renée

    2017-01-01

    Myasthenia gravis (MG) is a rare autoimmune disease that leads to progressive muscle weakness and is common during female reproductive years. The myasthenic mother and her newborn must be observed carefully, as complications during all stages of pregnancy and the puerperium may arise suddenly. Preeclampsia is a common obstetrical condition for which magnesium sulfate is used for seizure prophylaxis. However, magnesium sulfate is strongly contraindicated in MG as it impairs already slowed nerve-muscle connections. Similarly, many first-line antihypertensive medications, including calcium channels blockers and β-blockers, may lead to MG exacerbation. This case describes the effective obstetrical management of a patient with MG who developed severe preeclampsia. The effective use of levetiracetam and various antihypertensive medications including intravenous labetalol is described. A review of the ten reported cases of MG complicated by preeclampsia is examined to aggregate observations of clinical care, with focus on delivery methods, anticonvulsants, and antihypertensive medications. PMID:28280642

  14. Severe Preeclampsia in the Setting of Myasthenia Gravis

    Directory of Open Access Journals (Sweden)

    Adam J. Lake

    2017-01-01

    Full Text Available Myasthenia gravis (MG is a rare autoimmune disease that leads to progressive muscle weakness and is common during female reproductive years. The myasthenic mother and her newborn must be observed carefully, as complications during all stages of pregnancy and the puerperium may arise suddenly. Preeclampsia is a common obstetrical condition for which magnesium sulfate is used for seizure prophylaxis. However, magnesium sulfate is strongly contraindicated in MG as it impairs already slowed nerve-muscle connections. Similarly, many first-line antihypertensive medications, including calcium channels blockers and β-blockers, may lead to MG exacerbation. This case describes the effective obstetrical management of a patient with MG who developed severe preeclampsia. The effective use of levetiracetam and various antihypertensive medications including intravenous labetalol is described. A review of the ten reported cases of MG complicated by preeclampsia is examined to aggregate observations of clinical care, with focus on delivery methods, anticonvulsants, and antihypertensive medications.

  15. Quality of life in 188 patients with myasthenia gravis in China.

    Science.gov (United States)

    Yang, Yongxiang; Zhang, Min; Guo, Jun; Ma, Shan; Fan, Lingling; Wang, Xianni; Li, Chuan; Guo, Peng; Wang, Jie; Li, Hongzeng; Li, Zhuyi

    2016-01-01

    Myasthenia gravis (MG) is a kind of chronic autoimmune disease which can weaken patients' motor function and, furthermore, produce negative impact on the health-related quality of life (HRQoL). The primary purpose of this research was to evaluate factors that might affect the HRQoL of MG patients. A cross-sectional clinical research was carried out including 188 successive patients with MG. Myasthenia Gravis Foundation of America (MGFA) classification and Quantitative Myasthenia Gravis (QMG) score were applied to assess the severity of the disease. The Medical Outcome Survey 36-Item Short-Form Health Survey (SF-36) was used to estimate the HRQoL. Hamilton Depression Rating Scale (HDRS) and Hamilton Anxiety Rating Scale (HARS) were utilized to measure the depression and anxiety symptom. Factors may influence the HRQoL of MG patients include age, educational level, occupation, the situation of the thymus, the type of MG and generalized myasthenia gravis (GMG), the severity of the disease and the psychological disorder. Higher QMG and HARS scores were two significant factors that can prognosticate lower Physical Composite Score (PCS) and Mental Composite Score (MCS), while older age was just a significant factor which has prognostic value for lower PCS. The results of this research may have a potential guiding significance for the clinical treatment strategy and improve the quality of life in patients with MG consequently. In addition to the treatment of physical symptoms, the psychological symptoms such as anxiety and depression should be concerned as well.

  16. Two patients with co-morbid myasthenia gravis in a Brazilian cohort of inflammatory bowel disease.

    Science.gov (United States)

    Gondim, Francisco de A A; de Oliveira, Gisele R; Araújo, Davi F; Souza, Marcellus Henrique Loiola Ponte; Braga, Lúcia Libanez Bessa Campelo; Thomas, Florian P

    2014-11-01

    Co-morbid auto-immune disorders may affect 0.2% of the population. We present the clinical and electrodiagnostic findings of 2 patients with inflammatory bowel disease and myasthenia gravis from a Brazilian cohort of 218 inflammatory bowel disease patients. Patient 1: A 40year-old man was diagnosed with ulcerative colitis at age 37 and underwent total colectomy 3years later. After prednisone was tapered, he experienced a clinical relapse and was diagnosed with Crohn's disease. He then developed quadriparesis, bilateral ptosis, dysphagia and dysarthria. Patient 2: A 41year-old woman (diagnosed with ulcerative colitis and primary sclerosing cholangitis at age 35) developed speech impairment and ptosis. On both patients, symptoms quickly progressed over few weeks. Myasthenia gravis was diagnosed and confirmed by abnormal repetitive nerve stimulation and elevated anti-acetylcholine receptor antibody titers. Pyridostigmine and prednisone successfully treated both patients. Myasthenia gravis prevalence over 9years was 0.9%. Myasthenia gravis clinical course was not significantly modified by inflammatory bowel disease relapses and should be suspected with new onset weakness.

  17. 眼肌型重症肌无力研究进展%Update in ocular myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    成艳

    2014-01-01

    Myasthenia gravis is an autoimmune disease caused by the failure of neuromuscular transmission,which is mediated by autoantibodies against acetylcholine receptors.Ocular myasthenia is a form of myasthenia gravis in which weakened eye muscles cause double vision or drooping eyelids.It accounts for approximately 80% of juvenile myasthenia gravis.This review focuses on the extraocular muscle susceptibility to myasthenia gravis and the management of ocular myasthenia gravis.%重症肌无力是一种由乙酰胆碱受体抗体介导的、神经肌肉接头传递功能障碍所导致的自身免疫性疾病.眼肌型重症肌无力是重症肌无力的一种亚型,患者眼部肌肉肌无力引起复视或眼睑下垂.在儿童重症肌无力中,眼肌型占80%左右.该文就眼肌型重症肌无力的眼肌易患性及治疗进展作一综述.

  18. Kikuchi-Fujimoto Disease Associated with Myasthenia Gravis: A Case Report

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    Olukayode Onasanya

    2010-01-01

    Full Text Available Kikuchi-Fujimoto disease is a self-limited benign condition of unknown etiology characterized by cervical lymphadenopathy, fever, and leucopenia. An autoimmune hypothesis has been suggested and an association with systemic lupus erythematosus, Sjogren's disease, and antiphospholipid syndrome has been noted. We report a 27-year-old male who presented for evaluation of weakness and he was diagnosed with seropositive generalized myasthenia gravis and underwent a thymectomy. He was stable until five months post-thymectomy, when he developed a high fever associated with nontender cervical lymphadenopathy, chills, and night sweats. Histopathology of a cervical lymph gland biopsy was compatible with Kikuchi-Fujimoto lymphadenitis. He improved spontaneously and was asymptomatic at the followup six months later. Our case expands the association of Kikuchi-Fujimoto disease with autoimmune disorders to include myasthenia gravis.

  19. Characterization of CD4 and CD8 T Cell Responses in MuSK Myasthenia Gravis

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    Yi, JS; Guidon, A; Sparks, S.; Osborne, R.; Juel, VC; Massey, JM; Sanders, DB; Weinhold, KJ; Guptill, JT

    2013-01-01

    Muscle specific tyrosine kinase myasthenia gravis (MuSK MG) is a form of autoimmune MG that predominantly affects women and has unique clinical features, including prominent bulbar weakness, muscle atrophy, and excellent response to therapeutic plasma exchange. Patients with MuSK MG have predominantly IgG4 autoantibodies directed against MuSK on the postsynaptic muscle membrane. Lymphocyte functionality has not been reported in this condition. The goal of this study was to characterize T-cell...

  20. Stimulated jitter with concentric needle in 42 myasthenia gravis patients

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    João Aris Kouyoumdjian

    2013-04-01

    Full Text Available Objective: To estimate jitter parameters in myasthenia gravis in stimulated frontalis and extensor digitorum muscles using the concentric needle electrode. Methods: Forty-two confirmed myasthenia gravis patients, being 22 males (aged 45.6±17.2 years-old were studied. Jitter was expressed as the mean consecutive difference (MCD. Results: MCD in extensor digitorum was 61.6 µs (abnormal in 85.7% and in frontalis 57.3 µs (abnormal in 88.1%. Outliers represented 90.5% for extensor digitorum and 88.1% for frontalis. At least one jitter parameter was abnormal in 90.5% of the combined studies. Acetylcholine receptor antibody was abnormal in 85.7% of the cases. Conclusions: Stimulated jitter recordings measured from muscles using concentric needle electrode can be used for myasthenia gravis diagnosis with high sensitivity. Extensive normative studies are still lacking and, therefore, borderline findings should be judged with great caution.

  1. Rare association of celiac disease with myasthenia gravis in a patient with other immune disorders: a Case Report

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    Marcela de Almeida-Menezes

    Full Text Available Background: Celiac disease is described in association with several autoimmune diseases, but rarely with myasthenia gravis. Case Report: We describe the case of a 31-year-old white woman with celiac disease who presented manifestations related to a hyperactive immune system, including macroamylasemia, false-positive anti-HCV, positive antinuclear antibody, and Raynaud's phenomenon. The Introduction of a gluten-free diet (GFD resolved these features, but myasthenia gravis (MG symptoms unexpectedly occurred on that occasion. Discussion: The role of a GFD in the course of autoimmune diseases has been studied and improvement has been reported in many diseases. However, there is no consensus in the literature regarding the course of neurological disorders associated with celiac disease. In the present case, a GFD did not prevent the appearance of symptoms related to myasthenia gravis. There are few reports on the association of celiac disease with myasthenia gravis and therefore little is known about the course and time of onset of myasthenia in celiac patients. The present case increases the knowledge about this unusual autoimmune neurological disease associated with celiac disease.

  2. Refractory myasthenia gravis – clinical profile, comorbidities and response to rituximab

    Science.gov (United States)

    Sudulagunta, Sreenivasa Rao; Sepehrar, Mona; Sodalagunta, Mahesh Babu; Settikere Nataraju, Aravinda; Bangalore Raja, Shiva Kumar; Sathyanarayana, Deepak; Gummadi, Siddharth; Burra, Hemanth Kumar

    2016-01-01

    Introduction: Myasthenia gravis (MG) is an antibody mediated autoimmune neuromuscular disorder characterized by fatigable muscle weakness. A proportion of myasthenia gravis patients are classified as refractory due to non responsiveness to conventional treatment. This retrospective study was done to evaluate clinical profile, epidemiological, laboratory, and features of patients with MG and mode of management using rituximab and complications. Methods: Data of myasthenia gravis patients admitted or presented to outpatient department (previous medical records) with MG between January 2008 and January 2016 were included. A total of 512 patients fulfilled the clinical and diagnostic criteria of myasthenia gravis of which 76 patients met the diagnostic certainty for refractory myasthenia gravis and were evaluated. Results: Out of 76 refractory MG patients, 53 (69.73%) patients fulfilled all the three defined criteria. The median age of onset of the refractory MG group was 36 years with a range of 27–53 years. In our study 25 patients (32.89%) belonged to the age group of 21–30 years. Anti-MuSK antibodies were positive in 8 non-refractory MG patients (2.06%) and 36 refractory MG patients (47.36%). Mean HbA1C was found to be 8.6±2.33. The dose of administered prednisone decreased by a mean of 59.7% (p=3.3x10–8) to 94.6% (p=2.2x10–14) after the third cycle of rituximab treatment. Conclusion: The refractory MG patients are most commonly female with an early age of onset, anti-MuSK antibodies, and thymomas. Refractory MG patients have higher prevalence and poor control (HbA1C >8%) of diabetes mellitus and dyslipidemia probably due to increased steroid usage. Rituximab is very efficient in treatment of refractory MG with adverse effects being low. PMID:27790079

  3. A conceptual framework for evaluating impairments in myasthenia gravis.

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    Carolina Barnett

    Full Text Available BACKGROUND: Myasthenia gravis is characterized by weakness and fatigability of different muscle groups, including ocular, bulbar and the limbs. Therefore, a measure of disease severity at the impairment level in myasthenia needs to reflect all the relevant impairments, as well as their variations with activity and fatigue. We conducted a qualitative study of patients with myasthenia, to explore their experiences and related impairments, aimed at developing a conceptual framework of disease severity at the impairment level in myasthenia gravis. METHODS: Twenty patients representing the spectrum of disease participated in semi-structured interviews. Interviews were recorded and the transcripts were analyzed by content analysis using an inductive approach with line-by-line open coding. Themes were generated from these codes. RESULTS: Two main themes were identified: the severity of the impairments and fatigability (i.e., triggering or worsening of an impairment with activity. The impairments were further classified within body regions (ocular, bulbar and axial/limbs. Fatigability was described as a phenomenon affecting the whole body but also affecting specific impairments, and was associated with fluctuation of the symptoms. Patients were concerned that clinical examination at a single point in time might not reflect their true clinical state due to fatigability and fluctuations in severity. CONCLUSIONS: This conceptual framework reflects the relevance of both severity and fatigability in understanding impairment-based disease severity in myasthenia. This framework could inform the development of impairment measures in myasthenia gravis.

  4. Ectopic cervical thymoma in a patient with Myasthenia gravis

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    Chang Hung

    2011-07-01

    Full Text Available Abstract Ectopic cervical thymoma is rare and is often misdiagnosed as a thyroid tumor or other malignancy. Ectopic thymic tissue can be found along the entire thymic descent path during embryogenesis. However, a thymoma arising from such ectopic thymic tissue is extremely rare. Herein we report a patient with ectopic cervical thymoma and myasthenia gravis (MG and discuss the management.

  5. Detection of multiple antibodies in myasthenia gravis and its clinical significance

    Institute of Scientific and Technical Information of China (English)

    WANG Wei-wei; HAO Hong-jun; GAO Feng

    2010-01-01

    Background Antibodies against acetylcholine receptor, acetylcholinesterase, ryanodine receptor and titin have been found in patients with myasthenia gravis. However, the relations between these antibodies and character of myasthenia gravis are unknown. This study aimed to detect multiple antibodies in myasthenia gravis and to investigate its clinical significance.Methods These antibodies were detected by enzyme-linked immunoabsorbent assay in 89 cases of myasthenia gravis,66 cases of other neurological diseases and 66 healthy controls. The incidences of antibodies were compared using the chi-square test.Results Acetylcholine receptor, acetylcholinesterase, titin and ryanodine receptor antibodies were detected in 53.9%,20.2%, 64.0% and 55.0% of myasthenia gravis patients respectively, higher than in patients of other neurological diseases and controls groups. The combination of the four antibodies assays provided 94.4% sensitivity and 84.0% specificity for the diagnosis of myasthenia gravis. Acetylcholinesterase antibody occurred more frequently in acetylcholine receptor antibody negative patients with adverse reactions to neostigmine test. Titin antibody provided 82.1% sensitivity and 52.5% specificity for myasthenia gravis with thymoma. Incidences of titin and of ryanedine receptor antibody were higher in late onset myasthenia gravis than in early onset myasthenia gravis. The proportion of titin antibody positive patients increased with the severity of myasthenia gravis as graded by a modified Osserman scale.Conclusions Testing for acetylcholine receptor, acetylcholinesterase, titin and ryanodine receptor antibodies can offer a better diagnostic method for myasthenia gravis than each antibody test alone. Titin antibody combined with computed tomography was better for the diagnosis of thymoma. Titin antibody occurred most frequently in severe myasthenia gravis.

  6. Total body irradiation for myasthenia gravis with thymoma: case report

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    Kang, Ki Mun; Choi, Ihl Bohng; Kim, In Ah [College of Medicine, Catholic Univ., Seoul (Korea, Republic of)

    1999-06-01

    Myasthenia Gravis (MG) is relatively rare occuring as one of important autoimmune disease to affect neuromuscular junction. This study was clinically to evaluate total body irradiation (TBI) against two patients including 33-year and 39-year females for chronic MG with thymoma who hospitalized in the St. Mary's Hospital, Catholic University since 1994 as well as who showed no response by thymectomy, immunotherapy and hormonal therapy. TBI designed by the dose of 150-180 cGy consisting of 10 cGy per fraction, three times a week, for 5-6 weeks using linear accelerator of 6 MV. During the treatment of TBI, they did complain acute side effect such as vomiting and also appear improved physical condition from 4-6 weeks after TBI. Through the follow-up period of 18 or 42 months after TBI, they did not have any symptomatic recurrence. Consequently, the results suggest that TBI can be used as an alternative tool for the patients concurrently for MG with thymoma who had been refractory to various conventional therapies like thymectomy, immunotherapy and hormonal therapy.

  7. Three Types of Striational Antibodies in Myasthenia Gravis

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    Shigeaki Suzuki

    2011-01-01

    Full Text Available Myasthenia gravis (MG is caused by antibodies that react mainly with the acetylcholine receptor on the postsynaptic site of the neuromuscular junction. A wide range of clinical presentations and associated features allow MG to be classified into subtypes based on autoantibody status. Striational antibodies, which react with epitopes on the muscle proteins titin, ryanodine receptor (RyR, and Kv1.4, are frequently found in MG patients with late-onset and thymoma. Antititin and anti-RyR antibodies are determined by enzyme-linked immunosorbent assay or immunoblot. More recently, a method for the detection of anti-Kv1.4 autoantibodies has become available, involving 12–15% of all MG patients. The presence of striational antibodies is associated with more severe disease in all MG subgroups. Anti-Kv1.4 antibody is a useful marker for the potential development of lethal autoimmune myocarditis and response to calcineurin inhibitors. Detection of striational antibodies provides more specific and useful clinical information in MG patients.

  8. Phase II trial of methotrexate in myasthenia gravis.

    Science.gov (United States)

    Pasnoor, Mamatha; He, Jianghua; Herbelin, Laura; Dimachkie, Mazen; Barohn, Richard J

    2012-12-01

    Prednisone is a frequently used treatment for myasthenia gravis (MG) but it has numerous side effects. Methotrexate is a selective inhibitor of dihydrofolate reductase and lymphocyte proliferation and is an effective immuosuppressive medication for autoimmune diseases. Given the negative results of the mycophenolate mofetil study, search for an effective immunosuppressant drug therapy is ongoing. The objective is to determine if oral methotrexate is safe and effective for MG patients who take prednisone. We have initiated a randomized, double-blind, placebo-controlled multicenter trial of methotrexate versus placebo in patients taking at least 10 mg/day of prednisone at enrollment. The methotrexate dose is increased to 20 mg and the prednisone dose is adjusted per protocol during the study. Clinical and laboratory evaluations are performed monthly for 12 months, with the primary efficacy measure being the nine-month prednisone area under the curve (AUC) from months 3 to 12. Secondary outcome measures include MG outcomes, quality of life measures, and a polyglutamation biomarker assay. A total of 18 U.S. sites and 2 Canadian sites are participating, with 48 screened cases, 42 enrolled, with 19 still active in the study.

  9. Dysphonia as the primary complaint in a case of myasthenia gravis: diagnosis and speech therapy.

    Science.gov (United States)

    Nemr, Nair Kátia; Simões-Zenari, Marcia; Ferreira, Tainá Soares; Fernandes, Heloisa Regina; Mansur, Letícia Lessa

    2013-01-01

    Myasthenia gravis is an autoimmune disease, manifested by progressive muscular weakness and fatigue. There are frequent ocular and bulbar symptoms, among them, dysphonia. This article reports a case of dysphonia that contributed to the diagnosis of myasthenia gravis through a speech evaluation. The patient sought speech therapy with the ENT diagnosis of presbyphonia. The perceptual voice assessment and acoustic analysis pointed out respiration, glottal voice source and resonance affections. Considering that some of the data obtained from anamnesis and vocal assessments were not directly related to presbyphonia, the speech therapist discussed the case with the physician and they both concluded it was necessary to refer the patient to a neurological evaluation. The neurologist then raised the diagnostic hypotheses of myasthenia gravis and requested further examinations. The patient underwent speech therapy and drug treatment. A vocal reassessment, which occurred two months after the initial treatment, showed improvement in voice quality, with great impact on quality of life. This article shows the importance of detailed clinical speech evaluation and participation of a speech therapist in an interdisciplinary team.

  10. Investigation on the Mechanism of Exacerbation of Myasthenia Gravis by Aminoglycoside Antibiotics in Mouse Model

    Institute of Scientific and Technical Information of China (English)

    LIU Changqin; HU Fang

    2005-01-01

    Summary: To investigate the underlying mechanism of the exacerbation of myasthenia gravis by aminoglycoside antibiotics. C57/BL6 mice were immunized with acetylcholine receptor (AChR), extracted from electric organ of Narcine timilei according to Xu Haopeng's methods, in complete Fruend's adjuvant (CFA) to establish experimental autoimmune myasthenia gravis (EAMG). EAMG mice were divided randomly into 5 groups: MG group, NS group and three antibiotics groups. The clinical symptom scores of mice were evaluated on d7 after the last immunization and d14 of antibiotics treatment. Repetitive nerve stimulation (RNS) was performed and the levels of anti-AChR antibody (AChR-Ab) were tested at the same time. The mean clinical symptom grades of gentamycin group (1.312, 2.067), amikacin group (1.111, 1.889) and etimicin group (1.263, 1.632) were significantly higher than those of MG group (1.000, 1.200) (P<0.05). The positive rates of RNS of three antibiotics groups were 69.23 %, 58.82 % and 63.16 % respectively, which were significantly higher than those of MG group and NS group (40.00 %, 40.00 %, P<0.05). The AChR-Ab level in serum and the expression of AChR on neuromuscular junction (NMJ) of mice in three antibiotics groups were also higher than those of MG group. Our results indicated that aminoglycoside antibiotics could aggravate the symptom of myasthenia gravis. The exacerbation of myasthenia gravis by these antibiotics probably involves competitively restraining the release of acetylcholine from presynaptic membrane, impairing the depolarization of postsynaptic membrane, depressing the irritability of myocyte membrane around the end-plate membrane and consequently leading to the blockade of neuromuscular junction.

  11. Future perspectives in target-specific immunotherapies of myasthenia gravis.

    Science.gov (United States)

    Dalakas, Marinos C

    2015-11-01

    Myasthenia gravis (MG) is an autoimmune disease caused by complement-fixing antibodies against acetylcholine receptors (AChR); antigen-specific CD4+ T cells, regulatory T cells (Tregs) and T helper (Th) 17+ cells are essential in antibody production. Target-specific therapeutic interventions should therefore be directed against antibodies, B cells, complement and molecules associated with T cell signaling. Even though the progress in the immunopathogenesis of the disease probably exceeds any other autoimmune disorder, MG is still treated with traditional drugs or procedures that exert a non-antigen specific immunosuppression or immunomodulation. Novel biological agents currently on the market, directed against the following molecular pathways, are relevant and specific therapeutic targets that can be tested in MG: (a) T cell intracellular signaling molecules, such as anti-CD52, anti-interleukin (IL) 2 receptors, anti- costimulatory molecules, and anti-Janus tyrosine kinases (JAK1, JAK3) that block the intracellular cascade associated with T-cell activation; (b) B cells and their trophic factors, directed against key B-cell molecules; (c) complement C3 or C5, intercepting the destructive effect of complement-fixing antibodies; (d) cytokines and cytokine receptors, such as those targeting IL-6 which promotes antibody production and IL-17, or the p40 subunit of IL-12/1L-23 that affect regulatory T cells; and (e) T and B cell transmigration molecules associated with lymphocyte egress from the lymphoid organs. All drugs against these molecular pathways require testing in controlled trials, although some have already been tried in small case series. Construction of recombinant AChR antibodies that block binding of the pathogenic antibodies, thereby eliminating complement and antibody-depended-cell-mediated cytotoxicity, are additional novel molecular tools that require exploration in experimental MG.

  12. Bleeding Jejunal Diverticulosis in a Patient with Myasthenia Gravis

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    I. Zuber-Jerger

    2008-01-01

    Full Text Available A seventy-year-old male presented with severe myasthenia gravis and an episode of obscure bleeding. There was a history of gastric ulcer leading to Billroth II surgery twenty-five years ago. Upper endoscopy revealed no pathology. Colonoscopy showed a few solitary diverticula and traces of old blood in the terminal ileum. Capsule endoscopy pictured red smear in the upper jejunum. Diverticula were seen as well. Suspecting bleeding jejunal diverticulosis double balloon enteroscopy was performed. The complete jejunal ascending loop and about 100 cm of the jejunum through the descending jejunal loop could be inspected. Large diverticula with fecoliths were found in both loops. Bleeding had ceased. The patient was discharged to neurology for optimizing therapy for myasthenia gravis.

  13. Myasthenia gravis: new therapeutic approaches based on pathophysiology.

    Science.gov (United States)

    Lewis, Richard A

    2013-10-15

    Over the past 40 years Dr. Robert Lisak has made important contributions to our understanding of the pathophysiology and therapy of myasthenia gravis. This review will touch upon some of his work as it discusses current therapies and the potential for new treatments based on the evolving knowledge of the underlying basis of the disease. The recognition of different immune mechanisms that can cause the phenotype that we acknowledge as myasthenia gravis coincides with the introduction of monoclonal antibodies and other new therapies that can target specific aspects of the disease. This has raised our hopes for treatments that will have less side effects and be more effective. In some cases these hopes have been realized. In other instances, the situation remains a "work in progress". Dr. Lisak's work and teachings remain cogent to our modern approach to this classic immunologic disease.

  14. Microthymoma in elderly-onset myasthenia gravis detected preoperatively.

    Science.gov (United States)

    Hino, Haruaki; Nishimura, Takashi; Seki, Atsuko; Nitadori, Jun-Ichi; Arai, Tomio; Nakajima, Jun

    2016-10-01

    A 77-year-old woman with a 3-month history of muscle weakness was diagnosed with elderly-onset generalized myasthenia gravis (Myasthenia Gravis Foundation of America classification IIa) based on a high serum acetylcholine receptor antibody level (25.4 nmol·L(-1)) and neurological findings. Computed tomography detected a small nodule (diameter 15 mm) in the anterior mediastinum, which was suspected to be a thymoma. An extended thymectomy was performed. The pathological examination revealed a 6-mm-diameter thymoma, termed a microthymoma, accompanied with a unilocular thymic cyst without capsule formation (type B2 according to the World Health Organization classification). Some fat tissue was also found within the tumor.

  15. Do associated auto-antibodies influence the outcome of myasthenia gravis after thymectomy?

    Science.gov (United States)

    Keijzers, Marlies; Damoiseaux, Jan; Vigneron, Alain; Bodart, Nicolas; Kessels, Alfons; Dingemans, Anne-Marie C; Hochstenbag, Monique; Maessen, Jos; De Baets, Marc

    2015-01-01

    Myasthenia gravis (MG) is a neuromuscular autoimmune disease, where antibodies against the acetylcholine receptor destroy this receptor. The role of thymectomy in the treatment of MG remains controversial. Because of the frequent association with other autoimmune diseases, we hypothesized that patients with multiple autoantibodies (autoAbs) might have a lower chance of reaching complete stable remission after thymectomy. We analyzed sera of 85 MG patients who underwent a thymectomy between April 2004 and December 2012. We used four different immunodot kits (D-Tek, Mons, Belgium): ANA25 Quantrix, Synthetase 10 Diver, Myositis 7 Diver and Liver 10 profile Diver, all automatized on the BlueDiver Instrument (D-Tek). The Myasthenia Gravis Foundation of America (MGFA) postintervention status was used to determine the outcome after thymectomy. AutoAbs other than anti-acetylcholine receptor (AChR) antibodies were detected in 29.4% of the patients of whom 16.5% clinically had a second autoimmune disease. In none of the seronegative patients other autoAbs were detected. No significant difference was observed in the 3-years remission rate after thymectomy in patients with or without antibodies other than anti-AChR antibodies. Although these autoAbs do not predict outcome in our MG patient cohort, screening for multiple autoAbs in MG patients might be warranted to identify patients with additional autoimmune diseases.

  16. Computed tomography of the mediastinum in myasthenia gravis

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    Guertler, K.F.; Janzen, R.W.C.; Hagemann, J.; Otto, H.F.

    1982-01-01

    Computed tomography of the mediastinum was performed in 45 patients with myasthenia gravis. Surgery was carried out in fourteen. Amongst these, there were four thymomas, one thymolipoma, eight thymic hyperplasias and one normal thymus gland. A further patient, who did not have surgery, probably also had a thymic tumour. The normal thymus and thymic hyperplasia cannot be distinguished on computed tomography. Differentiation of small thymomas from normal thymus is not always possible. Invasion by thymomas can only be appreciated with large tumours.

  17. Epidemiology of myasthenia gravis with anti-muscle specific kinase antibodies in the Netherlands

    NARCIS (Netherlands)

    Niks, Erik H.; Kuks, Jan B. M.; Verschuuren, Jan J. G. M.

    2007-01-01

    The epidemiology of myasthenia gravis subtypes and the frequency of antibodies to muscle-specific kinase (MuSK) was studied in patients with generalised myasthenia gravis without anti-acetylcholine receptor antibodies who had an onset of symptoms between 1990 and 2004 in a well-defined region in the

  18. Ocular Myasthenia Gravis, Hashimoto’s Thyroiditis, Iron Deficiency Anemia, and Reactive Hypoglycemia

    Directory of Open Access Journals (Sweden)

    Füsun Mayda Domaç

    2009-06-01

    Full Text Available Myasthenia gravis (MG may occur in association with other organ- specific or nonspecific autoimmune diseases. In our study, we present a patient with ocular myasthenia, Hashimoto’s thyroiditis, and iron deficiency anemia who later developed reactive hypoglycemia. A 25-year-old woman with complaints of fluctuating extraocular muscle weakness, ptosis, and diplopia was examined. MG, type I (ocular MG, was confirmed by an elevated titer of anti-acetylcholine receptor antibodies in serum (7 nmol/l, normal <0.6 and positive edrophonium test. Nerve conduction studies, needle electromyography, repetitive stimulation tests, and mediasten MRI were normal. The coexistence of Hashimoto's thyroiditis was diagnosed by the presence of a diffuse thyroid enlargement, elevated titers of anti-thyroglobulin antibodies (211.8 IU/ml, normal 0-115, thyroid peroxidase antibodies (356.4 IU/ml, normal 0-34, thyroid stimulating hormone (8.21 uIU/ml, normal 0.27-4.2, and a decreased titer of free-T4 (1.03ng/dl normal 1.8-4.6. Diplopia and ptosis have regressed with 180 mg/day pyridostigmine bromide. On her follow-up period, 8 months later, reactive hypoglycemia has been diagnosed. The coexistence of myastenia gravis, Hashimoto’s thyroiditis and reactive hypoglycemia was not found in the literature. We should take into consideration the association and the importance of recognizing and treating these pathologies in myastenia gravis. Turk Jem 2009; 13: 31-3

  19. Inferior oblique muscle paresis as a sign of myasthenia gravis.

    Science.gov (United States)

    Almog, Yehoshua; Ben-David, Merav; Nemet, Arie Y

    2016-03-01

    Myasthenia gravis may affect any of the six extra-ocular muscles, masquerading as any type of ocular motor pathology. The frequency of involvement of each muscle is not well established in the medical literature. This study was designed to determine whether a specific muscle or combination of muscles tends to be predominantly affected. This retrospective review included 30 patients with a clinical diagnosis of myasthenia gravis who had extra-ocular muscle involvement with diplopia at presentation. The diagnosis was confirmed by at least one of the following tests: Tensilon test, acetylcholine receptor antibodies, thymoma on chest CT scan, or suggestive electromyography. Frequency of involvement of each muscle in this cohort was inferior oblique 19 (63.3%), lateral rectus nine (30%), superior rectus four (13.3%), inferior rectus six (20%), medial rectus four (13.3%), and superior oblique three (10%). The inferior oblique was involved more often than any other muscle (pmyasthenia gravis can be difficult, because the disease may mimic every pupil-sparing pattern of ocular misalignment. In addition diplopia caused by paresis of the inferior oblique muscle is rarely encountered (other than as a part of oculomotor nerve palsy). Hence, when a patient presents with vertical diplopia resulting from an isolated inferior oblique palsy, myasthenic etiology should be highly suspected.

  20. Study on the Clinical Effectiveness of Thymectomy for Myasthenia Gravis

    Institute of Scientific and Technical Information of China (English)

    PANTiecheng; YANHua; ZHANGNi; PANYouming; TANGYingxiong; WENXiang; SONGDingwei; HUMing; LIULigang; LIUZixiong; YANGMingshan; XUJinzhi; PUBitao; GAOBoting

    2002-01-01

    Objectiv:To investigate the theoretical basis and clinical significance of thymectomy in the treatment of myasthenia gravis (MG). Methods:Analyze the changes in the contents of serum AchRab and sIL-2R, lymphocyte subtypes in Peripheral blood lymphocytes before and after thymectomy in 69 patients. Results:In the study group significant changes were found in serum contents of AchRab and sIL-2R before and after operation and there was also remarkable change in the number and constitution of peripheral blood lymphocyte subtypes. Conclusion:Thymectomy is an effective therapy for MG.

  1. Computed tomography of the thymus gland in myasthenia gravis

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    Thorvinger, B.; Lyttkens, K.; Samuelsson, L.

    Sixteen consecutive patients with myasthenia gravis were examined by computed tomography (CT) before thymectomy. Surgical and histologic findings were compared with those obtained at CT. The results, like those of most other recent studies, indicate that CT is a reliable method for identifying thymoma. In all three patients with thymoma, and in five out of eight patients with hyperplasia, the lesion was observed at CT, giving an overall accuracy of 80%. Although tymic hyperplasia is a basically histological diagnosis an enlarged gland may give a correct diagnosis of this abnormality.

  2. The value of computed tomography in myasthenia gravis

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    Brown, L.R.; Muhm, J.R.; Sheedy, P.F. II; Unni, K.K.; Bernatz, P.E.; Hermann, R.C. Jr.

    1983-01-01

    In a 5 year study, 19 patients with myasthenia gravis were studied by computed tomography (CT) and underwent thymectomy. CT was accurate in detecting the nine true thymic masses but could not differentiate thymomas from nonthymomatous masses, including thymic cysts. No thymoma was found in a patient under 25 years of age. In one case, the 18 sec scanner could not differentiate a large gland from a thymoma. In eight cases, glands with histologic thymic hyperplasia and histologically normal thymus appeared to be similar and could not be differentiated by CT.

  3. Myasthenia gravis and thymoma coexisting with myotonic dystrophy type 1.

    Science.gov (United States)

    Ekmekci, Ozgul; Karasoy, Hatice; Bademkiran, Fikret; Akkus, Dilek Evyapan; Yuceyar, Nur

    2014-01-01

    We describe a 34-year old man presenting with subacute generalized myasthenic symptoms. His clinical features and laboratory investigations demonstrated both myasthenia gravis and myotonic dystrophy type 1. The computerized tomography of chest revealed anterior mediastinal mass. The lymphocyte-rich thymoma was removed surgically and he received radiotherapy. Recent observations suggested that the patients with myotonic dystrophy may have an increased risk of benign and malignant tumours but its coexistence with thymoma is very rare. The risk of thymoma associated with myotonic dystrophy is unknown.

  4. Pathogenesis of myasthenia gravis: update on disease types, models, and mechanisms [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    William D. Phillips

    2016-06-01

    Full Text Available Myasthenia gravis is an autoimmune disease of the neuromuscular junction (NMJ caused by antibodies that attack components of the postsynaptic membrane, impair neuromuscular transmission, and lead to weakness and fatigue of skeletal muscle. This can be generalised or localised to certain muscle groups, and involvement of the bulbar and respiratory muscles can be life threatening. The pathogenesis of myasthenia gravis depends upon the target and isotype of the autoantibodies. Most cases are caused by immunoglobulin (IgG1 and IgG3 antibodies to the acetylcholine receptor (AChR. They produce complement-mediated damage and increase the rate of AChR turnover, both mechanisms causing loss of AChR from the postsynaptic membrane. The thymus gland is involved in many patients, and there are experimental and genetic approaches to understand the failure of immune tolerance to the AChR. In a proportion of those patients without AChR antibodies, antibodies to muscle-specific kinase (MuSK, or related proteins such as agrin and low-density lipoprotein receptor-related protein 4 (LRP4, are present. MuSK antibodies are predominantly IgG4 and cause disassembly of the neuromuscular junction by disrupting the physiological function of MuSK in synapse maintenance and adaptation. Here we discuss how knowledge of neuromuscular junction structure and function has fed into understanding the mechanisms of AChR and MuSK antibodies. Myasthenia gravis remains a paradigm for autoantibody-mediated conditions and these observations show how much there is still to learn about synaptic function and pathological mechanisms.

  5. Anti-MuSK-Positive Myasthenia Gravis in a Patient with Parkinsonism and Cognitive Impairment

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    S. Lanfranconi

    2011-01-01

    Full Text Available Muscle-specific tyrosine kinase- (MuSK- antibodies-positive Myasthenia Gravis accounts for about one third of Seronegative Myasthenia Gravis and is clinically characterized by early onset of prominent bulbar, neck, shoulder girdle, and respiratory weakness. The response to medical therapy is generally poor. Here we report a case of late-onset MuSK-antibodies-positive Myasthenia Gravis presenting with signs of cognitive impairment and parkinsonism in addition to bulbar involvement and external ophthalmoplegia. The pattern of involvement of both peripheral and central nervous system dysfunction might suggest a common pathogenic mechanism, involving impaired cholinergic transmission.

  6. Myasthenia gravis and pregnancy%妊娠合并重症肌无力

    Institute of Scientific and Technical Information of China (English)

    廖治; 杨业洲

    2009-01-01

    该文就妊娠与重症肌无力之间的相互关系进行了阐述,并介绍了重症肌无力患者的妊娠管理,为育龄期重症肌无力妇女的孕前指导、孕期保健及治疗提供临床指导.%The relationship between myasthenia gravis and pregnancy is narrated in the manscript, and presents management for pregnancy of women with myasthenia gravis . To supply the guidance of progestation, antenatal care and therapia for women of reproductive age with myasthenia gravis.

  7. Myasthenia gravis with presynaptic neurophysiological signs: Two case reports and literature review.

    Science.gov (United States)

    Alboini, Paolo Emilio; Damato, Valentina; Iorio, Raffaele; Luigetti, Marco; Evoli, Amelia

    2015-08-01

    The distinction between myasthenia gravis and Lambert-Eaton myasthenic syndrome is based on clinical, neurophysiological and immunological features. We hereby report two cases with a clinical diagnosis of myasthenia gravis and neurophysiological features consistent with a pre-synaptic neuromuscular transmission defect. Both patients had increased anti-acetylcholine receptor antibody titres and showed a good response to cholinesterase inhibitors, along with a >100% facilitation of the compound muscle action potential on electrophysiological studies. We provide a review of English literature studies on co-existing features of myasthenia gravis and Lambert-Eaton myasthenic syndrome, and discuss diagnostic controversies.

  8. Muscle-specific kinase antibody associated myasthenia gravis after bone marrow transplantation.

    Science.gov (United States)

    Heidarzadeh, Zeinab; Mousavi, Seyyed-Asadollah; Ostovan, Vahid Reza; Nafissi, Shahriar

    2014-02-01

    Myasthenia gravis is a rare complication of bone marrow transplantation and graft versus host disease. We report a 30-year-old woman presented with oculobulbar and proximal limb weakness after allogeneic bone marrow transplantation for chronic myelogenous leukemia. Also, she developed graft versus host disease following bone marrow transplantation. Investigations led to the diagnosis of muscle specific kinase antibody related myasthenia gravis. There have been only two case reports of muscle specific kinase antibody positive myasthenia gravis after bone marrow transplantation in the literature, but none of the previously reported cases had graft versus host disease.

  9. Demyelinating disease in patients with myasthenia gravis Doenças desmielinizantes em pacientes com miastenia gravis

    Directory of Open Access Journals (Sweden)

    Denis Bernardi Bichuetti

    2008-03-01

    Full Text Available Myasthenia gravis (MG is an autoimmune disease characterized by fluctuating muscle weakness, caused by impaired neuromuscular transmission. Patients with MG can present other autoimmune diseases in association, commonly hypo or hyperthyroidism. The association of MG to demyelinating disease is rare and has been described before. We report on three Brazilian patients with MG that presented distinct demyelinating diseases, two monophasic and one recurrent neuromyelitis optica, several years after the diagnosis of MG, and discuss their clinical courses.Miastenia gravis (MG é doença autoimune caracterizada por episódios de fraqueza muscular alternados com melhora, causada por bloqueio da junção neuromuscular. Pacientes com MG podem apresentar outras doenças autoimunes, comumente hipo ou hipertiroidismo, e a associação de MG com doenças desmielinizantes é raramente descrita. Relatamos três pacientes brasileiros com MG que desenvolveram doenças desmielinizantes, dois monofásicos e um neuromielite óptica recorrente, vários anos após o diagnóstico de MG e discutimos seus cursos clínicos.

  10. Myasthenia gravis mimicking stroke: a case series with sudden onset dysarthria.

    Science.gov (United States)

    Tremolizzo, Lucio; Giopato, Federico; Piatti, Maria Luisa; Rigamonti, Andrea; Ferrarese, Carlo; Appollonio, Ildebrando

    2015-06-01

    Myasthenia gravis (MG) is an immune-mediated disorder characterized by fluctuating fatigue of skeletal muscles, often involving extrinsic ocular or bulbar districts. Myasthenia gravis in the elderly is an under-recognized condition, sometimes confused with cerebrovascular disease. Here we present a case series of myasthenia patients which onset was characterized by sudden dysarthria, clearly raising this diagnostic dilemma. In the workout of sudden onset isolated dysarthria, MG should be always considered. In fact, even if myasthenia is a rare condition, lacunar stroke only with this clinical presentation is also unusual, and significant risks may arise (e.g., unexpected myasthenic crisis).

  11. Invasive medullary thymoma associated with myasthenia gravis: an unusual case Miastenia gravis em um paciente com timoma medular invasivo: relato de caso

    Directory of Open Access Journals (Sweden)

    JORGE S. REIS FILHO

    2000-12-01

    Full Text Available Thymomas are tumors characterized by a remarkable morphological heterogeneity and variable clinical behavior. This tumor has unique clinical associations, most notably with hematological abnormalities and myasthenia gravis. According with the Müller-Hermelink criteria, there are significant differences between the histological types of thymomas and the association with myasthenia gravis. Among the different histological types, medullary thymoma is the least frequent variant associated with this autoimmune disease. In this report we describe a case of medullary thymoma presenting in a 71-year- old woman with a myasthenic syndrome.Os timomas são tumores caracterizados por grande heterogeneidade morfológica e comportamento clínico variável. Este tumor apresenta associações clínicas singulares, principalmente com doenças hematológicas e com a miastenia gravis. De acordo com a classificação de Müller-Hermelink, existem diferenças significativas entre as variedades histológicas dos timomas e sua associação com a miastenia gravis. Entre os diferentes tipos histológicos, o timoma medular é a variante menos frequentemente associada com esta doença autoimune. Neste relato, nós descrevemos caso de timoma medular em uma paciente de 71 anos de idade com síndrome miastênica.

  12. Malignant thymona with symptoms of myasthenia gravis; Grasiczak zlosliwy z objawami nuzliwosci miesni

    Energy Technology Data Exchange (ETDEWEB)

    Miarzynska, M.; Szlezak, L.; Fibak, J.; Wolski, M.; Lis-Podrzycka, E.; Miarzynski, K. [Szpital im. F. Raszei, Poznan (Poland)

    1994-12-31

    The rare case of malignant tumor of thymus - Thymoma malignum was described. The initial diagnosis was difficult, because of the irregular symptoms of myasthenia gravis. The diagnostic difficulties, treatment and clinical features of this neoplasm were also discussed. (author)

  13. 重症肌无力被动转移大鼠模型的建立及基质金属蛋白酶9表达的改变%Passive transfer of experimental autoimmune myasthenia gravis and the changes of the expression of matrix metalioproteinase 9

    Institute of Scientific and Technical Information of China (English)

    滕银燕; 李佳; 张旭

    2012-01-01

    目的 研究mAb35建立重症肌无力被动转移大鼠模型(passive transfer myasthenia gravis,PTMG)的方法,探讨基质金属蛋白酶9(MMP-9)在PTMG中的表达及意义.方法 用乙酰胆碱受体(acetylcholine receptor,AChR)特异性单克隆抗体mAb35建立PTMG动物模型,应用HE染色观察大鼠腓肠肌炎性细胞浸润情况,应用免疫荧光技术观察大鼠腓肠肌AChR数量改变,用ELISA方法检测血清AChR抗体改变,应用逆转录-聚合酶链反应(reverse transcription-polymerase chain reaction,RT-PCR)法研究MMP-9在PTMG腓肠肌基因水平表达变化.结果 PTMG Lewis大鼠模型成模率为100%.光镜下可见PTMG组肌肉炎性细胞浸润增多,荧光显微镜下可见PTMG组腓肠肌AChR数量相对对照组表达明显下调.PTMG组血清AChR抗体量[(1431±102.9) ng/mL]明显高于健康对照组[(364.8±15.8)ng/mL] (P<0.05).PTMG组腓肠肌MMP-9 mRNA相对表达水平(AMMP-9/AGAPDH=0.693±0.152)高于健康对照组(AMMP-9 /AGAPDH =0.132±0.026)(P<0.01).结论 MMP-9表达增加可能与PTMG发病有关.%Objective To establish experimental autoimmune myasthenia gravis model by passive transferring mAb35 to rats, and then investigate the expression and the effects of matrix metalloproteinase 9 (MMP-9) on passive transfer myasthenia gravis (PTMG). Methods Establish experimental autoimmune myasthenia gravis by passive transfer mAb35 which is acetylcholine receptor (AChR) specific monoclonal antibody. The inflammatory cell infiltration in rat gastrocnemius was observed by HE staining, the AChR changes in the gastrocnemius was observed by immunofluorescence technique, the serum AChRAb was measured by ELISA, the expression of MMP-9 mRNA in the PTMG was detected by RT-PCR. Results Passive transferring mAb35 could successfully establish experimental autoimmune myasthenia gravis, the successful rate was 100%. Light microscopy showed the infiltration of inflammatory cells increased in the PTMG muscle, fluorescence

  14. Acetylcholine receptor binding antibody-associated myasthenia gravis and rhabdomyolysis induced by nivolumab in a patient with melanoma.

    Science.gov (United States)

    Shirai, Takushi; Sano, Tasuku; Kamijo, Fuminao; Saito, Nana; Miyake, Tomomi; Kodaira, Minori; Katoh, Nagaaki; Nishie, Kenichi; Okuyama, Ryuhei; Uhara, Hisashi

    2016-01-01

    We reported an 81-year-old woman with metastatic melanoma, in whom myasthenia gravis and rhabdomyolysis developed after nivolumab monotherapy. The first symptom of myasthenia gravis was dyspnea. Ultrasonography detected hypokinesis of the bilateral diaphragm suggesting myasthenia gravis, although there was no abnormal finding of the lungs in computed tomography images. Acetylcholine receptor binding antibodies were low-titer positive in the preserved serum before administration of nivolumab, strongly suggesting that the myasthenia gravis was a nivolumab-related immune adverse event. Despite the remarkable clinical benefits of immune checkpoint inhibitors for patients with advanced melanoma, it is important to recognize unexpected immune-related adverse events.

  15. The diagnosis of thymoma and thymic atrophy in patients with myasthenia gravis; Dignostikk av tymom og thymusatrofi hos pasienter med myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Sund, K.K.; Skeie, G.O.; Gilhus, N.E.; Aarli, J.A.; Varhaug, J.E. [Haukeland Sykehus, Bergen (Norway)

    1997-11-01

    The authors have compared clinical, immunological and radiological data in 20 patients with myasthenia gravis and thymoma and in 21 patients with myasthenia gravis and thymic atrophy. The median age at onset was 54 years in the thymoma group and 63 years in the thymic atrophy group. The severity of the disease was similar in the two groups, and there was no significant difference in the concentration of acetylcholine receptor antibodies. CA antibodies were demonstrated in 17/20 thymoma patients and in 6/21 with thymic atrophy, while 19/20 thymoma patients had antibodies to titin, compared with 9/21 among those with thymic atrophy. The diagnosis and treatment of patients with myasthenia gravis is based upon an evaluation of clinical, immunological and radiological data. 28 refs., 2 tabs.

  16. Advancement of trentment in myasthenia gravis%重症肌无力的治疗进展

    Institute of Scientific and Technical Information of China (English)

    刘亚蒙; 郑伟刚

    2012-01-01

    Myasthenia gravis is autoimmune diseases which mediated with acetylcholine receptor antibody.Based on the studies in rencent years,treatment of MG has made great progress.Thisreview will discuss several aspects,such as immune tolerance induced by dendritic cells,blocking monoclonal antibodies,hematopoietic stem cell transplantion,and provide new ideas and methods for the treatment of myasthenia gravis.%重症肌无力(myasthenia gravis,MG)是乙酰胆碱受体抗体介导的自身免疫性疾病,随着对MG发病机制研究的深入,MG的治疗取得了巨大进展.该文从树突状细胞诱导的免疫耐受、单克隆抗体阻断、造血干细胞移植疗法等方面进行综述,以便为MG治疗提供依据.

  17. Overcoming challenges in the diagnosis and treatment of myasthenia gravis.

    Science.gov (United States)

    Evoli, Amelia; Iorio, Raffaele; Bartoccioni, Emanuela

    2016-01-01

    In recent years, the discovery of new autoantigens and the use of sensitive assays have expanded the clinical spectrum of myasthenia gravis (MG). In particular, antibodies binding to clustered acetylcholine receptors and to the low-density lipoprotein receptor-related protein 4 have not only bridged a significant gap in diagnosis but also have relevant clinical implications. MG management includes different therapeutic options, from symptomatic agents as the only therapy in mildly affected cases to combined long-term immunosuppression and thymectomy in patients with severe disabling disease. MG biological diversity can influence the response to therapies and should be taken into account when planning treatment. Biologic agents are promising, though their use is currently limited to patients with refractory disease.

  18. Anesthetic management of patient with myasthenia gravis and uncontrolled hyperthyroidism for thymectomy

    Directory of Open Access Journals (Sweden)

    Datt Vishnu

    2010-01-01

    Full Text Available The relationship between myasthenia gravis (MG and other autoimmune disorders like hyperthyroidism is well known. It may manifest earlier, concurrently orafter the appearance of MG. The effect of treatment of hyperthyroidism on the control of MG is variable. There may be resolution or conversely, deterioration of the symptoms also. We present a patient who was diagnosed to be hyperthyroid two and half years before the appearance of myasthenic symptoms. Pharmacotherapy for three months neither improved the myasthenic symptoms nor the thyroid function tests. Thymectomy resulted in control of MG as well as hyperthyroidism. In conclusion, effective control of hyperthyroidism in the presence of MG may be difficult. The authors opine that careful peri-operative management of thymectomy is possible in a hyperthyroid state.

  19. Remarkably increased resistin levels in anti-AChR antibody-positive myasthenia gravis.

    Science.gov (United States)

    Zhang, Da-Qi; Wang, Rong; Li, Ting; Li, Xin; Qi, Yuan; Wang, Jing; Yang, Li

    2015-06-15

    Resistin is a pro-inflammatory cytokine involved in the pathogenesis of autoimmune diseases. To investigate serum resistin levels in patients with myasthenia gravis (MG) and determine if there are associations between resistin levels and disease severity, we measured serum resistin levels in 102 patients with anti-acetylcholine receptor antibody-positive MG (AChR-MG). We further analyzed associations between serum resistin levels and clinical variables in patients with MG. Our findings demonstrate that serum resistin levels are elevated in patients with AChR-generalized MG and AChR-MG with thymoma and are correlated with disease severity. Resistin has potential as a useful serum biomarker for inflammation in AChR-MG.

  20. Serum metabolomic response of myasthenia gravis patients to chronic prednisone treatment.

    Science.gov (United States)

    Sengupta, Manjistha; Cheema, Amrita; Kaminski, Henry J; Kusner, Linda L

    2014-01-01

    Prednisone is often used for the treatment of autoimmune and inflammatory diseases but they suffer from variable therapeutic responses and significant adverse effects. Serum biological markers that are modulated by chronic corticosteroid use have not been identified. Myasthenia gravis is an autoimmune neuromuscular disorder caused by antibodies directed against proteins present at the post-synaptic surface of neuromuscular junction resulting in weakness. The patients with myasthenia gravis are primarily treated with prednisone. We analyzed the metabolomic profile of serum collected from patients prior to and after 12 weeks of prednisone treatment during a clinical trial. Our aim was to identify metabolites that may be treatment responsive and be evaluated in future studies as potential biomarkers of efficacy or adverse effects. Ultra-performance liquid chromatography coupled with electro-spray quadrupole time of flight mass spectrometry was used to obtain comparative metabolomic and lipidomic profile. Untargeted metabolic profiling of serum showed a clear distinction between pre- and post-treatment groups. Chronic prednisone treatment caused upregulation of membrane associated glycerophospholipids: phosphatidylcholine, phosphatidylethanolamine, phosphatidylserine, 1, 2-diacyl-sn glycerol 3 phosphate and 1-Acyl-sn-glycero-3-phosphocholine. Arachidonic acid (AA) and AA derived pro-inflammatory eicosanoids such as 18-carboxy dinor leukotriene B4 and 15 hydroxyeicosatetraenoic acids were reduced. Perturbations in amino acid, carbohydrate, vitamin and lipid metabolism were observed. Chronic prednisone treatment caused increase in membrane associated glycerophospholipids, which may be associated with certain adverse effects. Decrease of AA and AA derived pro-inflammatory eicosanoids demonstrate that immunosuppression by corticosteroid is via suppression of pro-inflammatory pathways. The study identified metabolomic fingerprints that can now be validated as prednisone

  1. Caesarean section in a patient with Myasthenia Gravis: A bigger challenge for the anesthesiologist than the obstetrician

    Directory of Open Access Journals (Sweden)

    Manoj K Sanwal

    2012-01-01

    Full Text Available Myasthenia Gravis (MG is an acquired, autoimmune disorder affecting neuromuscular junction presenting with easy fatigability, progressive weakness, diplopia, difficulty in speaking and swallowing and even ventilatory failure in severe cases. During pregnancy the disease may go into remission or may exacerbate at any time during first, second and third trimesters or postpartum period. We are reporting the case of a 28 year old primigravida, known case of MG, who underwent caesarean section and developed muscular weakness on third postoperative day. Her neonate also had tachypnoea and hypotonia, Both, the mother and the baby were managed aggressively and responded well to therapy.

  2. Anaesthetic Management of a patient with Myasthenia Gravis and Small Bowel Intussusception for Jejuno-Ileal Anastomoses

    Directory of Open Access Journals (Sweden)

    Pawan Kumar Jain

    2016-01-01

    Full Text Available Myasthenia gravis is a chronic autoimmune disease affecting voluntary skeletal muscles. The altered sensitivity of acetylcholine receptors to muscle relaxants and concomitant treatment with anticholinesterase in these patients affect their anaesthetic management. Patients who have undergone bowel anastomosis and are on regular anticholinesterase treatment are susceptible to anastomotic leaks. We report successful anaesthetic management of class I myasthenic patient with coexisting small bowel intussusception operated for jejuno-ileal anastomoses using regional, inhalational and intravenous (i.v anaesthesia based on train of four responses, and avoiding the use of reversal (anticholinesterase.

  3. Correlative study on the cognitive dysfunction of patients with myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    Wenli Chen; Wenmin Wang; Yi Wang

    2006-01-01

    BACKGROUND: Myasthenia gravis (MG) is considered as a peripheral neuromuscular disorder. Some investigations demonstrate that MG affects central nervous system (CNS), and there are disorders in cognitive function, affection, sleep, mental status and others; however, there are also some different standpoints. Some questions are still disputed, such as whether or not MG exists in the CNS and cognitive function is impaired.DATA SOURCES; Using the terms of "myasthenia gravis, neuropsychological test, memory, central, brain,cognitive dysfunction and so on", we searched the Medline database for the articles about CNS involved in MG, nerve and neuropsychology as well as cognitive function, which were published in the English language between January 198,5 and December 2004. Meanwhile, we also searched CNKI database by inputting "myasthenia gravis, neuropsychology and cognitive function" in Chinese for articles about involved neuropsychology and cognitive function which were published between October 1995 and June 2006 in the Chinese language.STUDY SElECTroN: Data were checked in the first trial, and articles about the influences of MG on CNS,neuropsychology, cognitive function and central cholinergic function were selected, then those were obviously unrelated with above criteria were excluded. Articles which expound the influences of MG on neuropsychology and cognitive were included, but those with repeated contents were also excluded.DATA EXTRACTTON: A total of 89 related English articles and 34 Chinese articles were collected, including 43studies on involved CNS and 32 studies on involved central cholinergic function and cholinergic receptor.Sixty-three literatures were about neuropsychology and cognitive function, which were closely related with this article. Seventy-two articles met inclusive criteria and 66 were excluded. Thirty of sevetty-two were chosen for analysis.DATA SYNTHESrs: MG is an autoimmune disease of muscular diseased fatigue induced by transmission

  4. Fusion protein of single-chain variable domain fragments for treatment of myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    Fangfang Li; Fanping Meng; Quanxin Jin; Changyuan Sun; Yingxin Li; Honghua Li; Songzhu Jin

    2014-01-01

    Single-chain variable domain fragment (scFv) 637 is an antigen-specific scFv of myasthenia gravis. In this study, scFv and human serum albumin genes were conjugated and the fusion pro-tein was expressed in Pichia pastoris. The afifnity of scFv-human serum albumin fusion protein to bind to acetylcholine receptor at the neuromuscular junction of human intercostal muscles was detected by immunolfuorescence staining. The ability of the fusion protein to block myas-thenia gravis patient sera binding to acetylcholine receptors and its stability in healthy serum were measured by competitive ELISA. The results showed that the inhibition rate was 2.0-77.4%, and the stability of fusion protein in static healthy sera was about 3 days. This approach suggests the scFv-human serum albumin is a potential candidate for speciifc immunosuppressive therapy of myasthenia gravis.

  5. Diagnosis of Tensilon-Negative Ocular Myasthenia Gravis By Daily Selfie.

    Science.gov (United States)

    Guterman, Elan L; Botelho, James V; Horton, Jonathan C

    2016-09-01

    The initial symptoms of myasthenia gravis are usually ptosis and diplopia. The diagnosis is often confirmed by testing for anti-acetylcholine receptor antibodies or by observing the effects of intravenous edrophonium (Tensilon) injection. However, these standard tests may be negative in patients with isolated ocular findings. We present the case of an 83-year-old woman with negative serologic and Tensilon testing. She was asked to photograph herself daily. The resulting sequence of daily selfies captured striking fluctuations in her ocular alignment and ptosis. Daily selfies may be a useful strategy for confirming the clinical diagnosis of ocular myasthenia gravis.

  6. Myasthenia gravis in children: analysis of 18 patients

    Directory of Open Access Journals (Sweden)

    Morita Maria da Penha A.

    2001-01-01

    Full Text Available Myasthenia gravis (MG in childhood is rare comprising 10 to 20 % of all myasthenic patients. We studied 18 patients with MG whose first symptoms started from 1 to 12 years of age, followed at the Department of Neurology of the UNIFESP-EPM, from January 1983 to August 1997. There were 10 girls and 8 boys (1.2:1. Eleven patients (61% presented moderate or severe generalized disease and 4 (22% had at least one myasthenic crisis. EMG with supramaximal repetitive nerve stimulation was diagnostic in 8 (47% out of 17 patients, and chest CT was normal in 14 patients. Seropositivity to acetylcholine receptor antibodies was found in 81.6% (9 out of 11 tested and the levels had no relation to clinical severity. Nine out of 16 patients (56% worsened with pyridostigmine alone and were treated with prednisone. Four out of those nine continued worsening despite steroids and were subjected to thymectomy (all showed thymic lymphoid follicular hyperplasia. Three patients (75% improved markedly after thymectomy and one (25% worsened, eventually getting better with intravenous immunoglobulin and oral azathioprine. MG treatment, using all resources available, has to be individualized for each child.

  7. Anesthetic implications for video assisted thoracoscopic thymectomy in myasthenia gravis.

    Science.gov (United States)

    El-Dawlatly, Abdelazeem A; Al Kattan, Khaled; Hajjar, Waseem; Essa, Mohamed; Delvi, Bilal; Khoja, Abdulaziz

    2005-06-01

    Thymectomy is an established therapy in the management of generalized myasthenia gravis (MG). However, the optimal surgical approach to thymectomy has remained controversial. There are advocates for transternal, transcervical approaches for "maximal" thymectomy. Video-assisted thoracoscopic thymectomy (VATT) presents new approach to thymectomy. By minimizing chest wall trauma, VATT not only causes less postoperative pain, shortens hospital stay, gives better cosmetic results but also leads to wider acceptance by patients for earlier surgery. Anesthesia for thymectomy in MG is challenging. Currently we are using non-muscle relaxant technique (NMRT) which we adopted in 1994, for maximal thymectomy. In this paper, we present our limited experience with two cases of VATT using two different NMRTs. Two cases of MG underwent VATT under general anesthesia (GA) and one lung ventilation (OLV) using double lumen tube (DLT). In both cases NMRT was used which encompass, light GA plus thoracic epidural analgesia (TEA) in one case and without TEA in the other case. We believe that the use of NMRT provides good operative and postoperative conditions. In this report we have described two different NMRTs, one with TEA and the other without. Further studies are needed on large number of cases to establish an anesthetic protocol for VATT.

  8. Computed tomography of the thymus in myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Tanimura, Shigeo; Sakaguchi, Kozo; Tomoyasu, Hiroshi; Banba, Jiro; Masaki, Mikio; Kurosaki, Atsuko; Matsushita, Hisashi [Toranomon Hospital, Tokyo (Japan)

    1995-01-01

    Comparison of preoperative CT diagnosis and histopathological diagnosis for the thymus were studied in 39 patients with myasthenia gravis. The patients consisted of 10 patients with thymoma and 29 without thymoma confirmed at the operation. CT diagnosis was 11 thymomas, 18 thymic hyperplasias and 10 normal thymuses. Eleven thymomas revealed histopathologically 9 thymomas, one follicular lymphoid hyperplasia (FLH) and one involved thymus. Out of 18 thymic hyperplasias 15 cases were FLH and 3 involved thymus. There were 5 involved thymuses, 4 FLHs and one thymoma in the 10 normal thymuses on CT. The finding of `reticular pattern`, many small nodules scattered reticularly in the thymus, in computed tomography could be regarded as a sign suggesting FLH of the thymus. The accuracy of this finding of CT was 83% for FLH. The finding of thymoma on CT revealed 82% of the accuracy. Therefore, CT was very useful in the diagnosis of the localization of the thymoma but not for the diagnosis of FLH of the thymus. Nevertheless the finding of `reticular pattern` on CT was helpful in the diagnosis of FLH. (author).

  9. Changes in inflammatory cytokine networks in myasthenia gravis

    Science.gov (United States)

    Uzawa, Akiyuki; Kanai, Tetsuya; Kawaguchi, Naoki; Oda, Fumiko; Himuro, Keiichi; Kuwabara, Satoshi

    2016-01-01

    Myasthenia gravis (MG) is an autoimmunological inflammatory disorder of the neuromuscular junction. Inflammation could be a key player for understanding the pathogenesis of MG. We measured the serum levels of 24 inflammatory cytokines in 43 patients with anti-acetylcholine receptor antibody-positive MG and 25 healthy controls. In patients with MG, serum levels of a proliferation-inducing ligand (APRIL), IL-19, IL-20, IL-28A and IL-35 were significantly increased as compared with controls (p IL-35 were significantly decreased after treatment (p < 0.05). In clinical subtype analyses, APRIL and IL-20 were increased in patients with late-onset MG and IL-28A levels were increased in patients with thymoma-associated MG compared with healthy controls (p < 0.01). The results of the present study demonstrate both anti-inflammatory and inflammatory cytokines are upregulated in MG, reflecting the importance of cytokine-mediated inflammation and its regulation in MG pathophysiology. PMID:27172995

  10. Genetics of Myasthenia Gravis: A Case-Control Association Study in the Hellenic Population

    Directory of Open Access Journals (Sweden)

    Zoi Zagoriti

    2012-01-01

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

  11. Diagnostic imaging and treatment of an invasive thymoma in myasthenia gravis. Bildgebende Diagnostik und Therapie eines invasiven Thymoms mit Myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Soetje, G.; Brinkmann, G. (Kiel Univ. (Germany, F.R.). Abt. Radiologie); Striepling, E.; Engemann, R. (Kiel Univ. (Germany, F.R.). Abt. Allgemeine Chirurgie)

    1991-05-01

    A 40-year old woman with an invasive thymoma and myasthenia gravis is described in this article. Chest-x-ray, CT and MRI of the mediastinum could not offer definite results on tumour malignancy. Radical surgical removal was the consequence. This revealed a tumour infiltration of the pleura and pericardium; hence an adjuvant irradiation must have been performed. Mestinon-treatment was afterwards gradually reduced. (orig.).

  12. Robotic thymectomy in patients with myasthenia gravis : neurological and surgical outcomes

    NARCIS (Netherlands)

    Keijzers, Marlies; de Baets, Marc; Hochstenbag, Monique; Abdul-Hamid, Myrurgia; zur Hausen, Axel; van der Linden, Marcel; Kuks, Jan; Verschuuren, Jan; Kessels, Fons; Dingemans, Anne-Marie C.; Maessen, Jos

    2015-01-01

    Thymectomy is frequently used in the treatment of myasthenia gravis (MG). But indication, timing or surgical approach remain controversial. This study reports our experiences with robotic thymectomy and surgical and neurological outcomes in a large cohort of patients with MG. We retrospectively anal

  13. Nonmelanoma skin cancer risk awareness in azathioprine-treated myasthenia gravis patients.

    LENUS (Irish Health Repository)

    McGurgan, Iain J

    2015-10-01

    Increased rates of NMSC (nonmelanoma skin cancer) have recently been reported in people with MG (myasthenia gravis) receiving azathioprine treatment. Guidelines on azathioprine for patients with dermatological and gastrointestinal disorders stress the importance of NMSC risk awareness and prevention. The aim of this study is to assess whether MG patients are being informed of this risk.

  14. What's in a smile? : Quantification of the vertical smile of patients with myasthenia gravis

    NARCIS (Netherlands)

    Weijnen, FG; Wokke, JHJ; Kuks, JBM; van der Glas, HW; Bosman, F

    2000-01-01

    Many patients with myasthenia gravis who experience bulbar symptoms show a vertical smile, which may have a considerable, and often underestimated, impact on social life. Peri-oral muscle function can be quantified by calculating lip-length and snout indices, which indicate the degree to which a per

  15. Predictors of postoperative myasthenic crisis in patients with myasthenia gravis after thymectomy

    Institute of Scientific and Technical Information of China (English)

    CHU Xiang-yang; XUE Zhi-qiang; WANG Ru-wen; TAN Qun-you

    2011-01-01

    Background Thymectomy is considered the most effective treatment in patients with myasthenia gravis. This study aimed to explore the predictors of postoperative myasthenic crisis in patients with myasthenia gravis after thymectomy.Methods Clinical records of 243 patients with myasthenia gravis who underwent thymectomy were reviewed retrospectively. The following factors were analyzed in relation to the occurrence of myasthenic crisis after thymectomy:gender, age, duration of symptoms, Osserman stage, history of myasthenic crisis, concomitant diseases, preoperative pyridostigmine dose, preoperative steroid therapy, operation approach, operation time, presence of thymoma, major postoperative complications.Results Forty-four patients experienced postoperative myasthenic crisis during the first month after thymectomy.Univariate analysis revealed that Osserman stage (RR=0.0976, P=0.000), history of myasthenic crisis (RR=0.2309,P=0.012), preoperative pyridostigmine dose (RR=0.4349, P=0.016), thymoma (RR=0.0606, P=0.000), and major postoperative complications (RR=0.1094, P=0.000) were significantly related to postoperative myasthenic crisis.Multivariate Logistic regression analysis showed that Osserman stage (Ⅱb+Ⅲ+Ⅳ) (RR=0.0953, P=0.000), thymoma (RR=0.0294, P=0.000), and major postoperative complications (RR=0.0424, P=0.000) independently predict postoperative myasthenic crisis.Conclusion Osserman stage (Ⅱb+Ⅲb+Ⅳ), thymoma and major postoperative complications are independent predictors of postoperative myasthenic crisis in patients with myasthenia gravis who underwent thymectomy.

  16. Disability and Functional Profiles of Patients with Myasthenia Gravis Measured with ICF Classification

    Science.gov (United States)

    Leonardi, Matilde; Raggi, Alberto; Antozzi, Carlo; Confalonieri, Paolo; Maggi, Lorenzo; Cornelio, Ferdinando; Mantegazza, Renato

    2009-01-01

    The objective of this study is to describe functional profiles of patients with myasthenia gravis (MG), and the relationships among symptoms, activities and environmental factors (EF), by using WHO's International Classification of Functioning Disability and Health (ICF). Patients were consecutively enrolled at the Besta Institute of Milan, Italy.…

  17. Effect of Gender, Disease Duration and Treatment on Muscle Strength in Myasthenia Gravis

    DEFF Research Database (Denmark)

    Citirak, Gülsenay; Cejvanovic, Sanja; Andersen, Henning;

    2016-01-01

    INTRODUCTION: The aim of this observational, cross-sectional study was to quantify the potential presence of muscle weakness among patients with generalized myasthenia gravis (gMG). The influence of gender, treatment intensity and disease duration on muscle strength and disease progression was also...

  18. Mortality in myasthenia gravis: A nationwide population-based follow-up study in Denmark

    DEFF Research Database (Denmark)

    Hansen, Julie S; Danielsen, Ditte H; Somnier, Finn E;

    2016-01-01

    INTRODUCTION: In previous studies of myasthenia gravis (MG), increased mortality has been reported. The aim of this study was to estimate mortality in patients with acetylcholine receptor antibody-positive (AChR-Ab-seropositive) MG in a nationwide population-based, long-term follow-up study...

  19. Assessment of cognitive function in patients with myasthenia gravis

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    Sherifa A Hamed

    2014-12-01

    Full Text Available Aim: During the past decade, there has been an increasing interest in the evaluation of cognitive function in myasthenia gravis (MG, neuromuscular transmission disorder caused by acetylcholine receptor auto-antibodies. However, the results of previous studies on cognition and MG are inconsistent and controversial. This study aimed to evaluate cognition in patients with mild/moderate grades of MG. Methods: This study included 20 patients with MG with a mean age of 28.45 ± 8.89 years and duration of illness of 3.52 ± 1.15 years. Cognition was tested using a sensitive battery of psychometric testing (Mini-mental State Examination [MMSE], Stanford-Binet Intelligence Scale 4 th edition [SBIS] and Wechsler Memory Scale-Revised [WMS-R] and by recording P300 component of event-related potentials (ERPs, a neurophysiological analog for cognitive function. Results: Compared with healthy subjects (n = 20, patients had lower total scores of cognitive testing (MMSE, SBIS and WMS-R (P = 0.001, higher Beck Depression Inventory 2 nd edition scores (P = 0.0001 and prolonged latencies (P = 0.01 and reduced amplitudes (P = 0.001 of P300 component of ERPs. Correlations were identified between total scores of cognitive testing and age (r = -0.470, P = 0.010, duration of illness (r = -0.788, P = 0.001 and depression scores (r = -0.323, P = 0.045. Using linear regression analysis and after controlling for age and depression scores, a significant correlation was identified between total scores of cognitive testing and duration of illness (β = -0.305, P = 0.045. Conclusion: Patients with mild/moderate MG may have cognitive dysfunction. This is important to determine prognosis and managing patients.

  20. The clinical outcome of thymectomy in myasthenia gravis

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    Mirsharifi R

    2009-02-01

    Full Text Available "nBackground: Myasthenia Gravis (MG is a neuromuscular disorder with weakness of skeletal muscles. Thymectomy is now recognized as a treatment modality in MG. The aim of this study was to evaluate the clinical effect of thymectomy on MG. "nMethods: MG patients with history of thymectomy at a tertiary referral center during twelve year period were included. The medical records were reviewed and telephone survey was conducted to evaluate the effects of thymectomy. "nResults: Sixty MG patients, 46 females and 14 males, aged 30.4±11.1 years, underwent open (n=48 or video-assisted thoracoscopic thymectomy (n=12 during study period. The mean dosage of preoperative pyridostigmine was 235.4±86.2mg/day. This figure reached to 129±18mg/day after thymectomy (p<0.0001. 17 patients (28.3% had complete remission (complete freedom of symptoms without medications. Improve-ment (improved symptoms or less medication requirement was seen in 34 patients (56.6%. There was no response to surgical therapy in six patients (10%. Three patients (5% had experienced progression of disease postoperatively. Overall, benefit of thymectomy was observed in 85% of patients. Age, sex, duration and severity of disease, quantity of preoperative drugs, surgical approach, and presence of thymoma did not affect the outcome. Satisfaction was stated as excellent in 17%, good in 43%, moderate in 35% and poor in 5% of patients after operation. "nConclusion: Thymectomy is an effective treatment for MG which leads to less severity of disease and less drug requirement. It would be considered in all myasthenic patients regardless of age, sex, duration and severity of disease and presence of thymoma.

  1. Sympathetic skin response in patients with myasthenia gravis A comparative analysis

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    BACKGROUND: The examination of sympathetic skin response is an important index for assessing the autonomic nerve function, and patients with myasthenia gravis are always accompanied by dysautonomia.Therefore, it will be important to know whether sympathetic skin response can be used as the index for the clinical evaluation of myasthenia gravis.OBJECTIVE: To investigate the diagnostic value of sympathetic skin response in the damage of autonomic nerve function of patients with myasthenia gravis.DESIGN: A case-controlled comparative observation.SETTING: Department of Neurology and Room of Nerve Electromyogram, the Affiliated Hospital of North Sichuan Medical College.PARTICIPANTS: Thirty outpatients or inpatients with myasthenia gravis were selected from the Department of Neurology, the Affiliated Hospital of North Sichuan Medical College from May 2006 to May 2007, including 9 males and 21 females, aged 8 - 72 years with a mean age of (28±5) years old. They were all accorded with the diagnostic standards of myasthenia gravis, accompanied by different severity of autonomic nerve symptoms, including poor skin nutrition, sweating of hands and feet, pyknocardia,persistent hypotension, abdominal pain, constipation, etc. They all had not taken any drug affecting the autonomic nerve function before the examination. Informed consents were obtained from all the patients.Meanwhile, 30 healthy physical examinees were enrolled as the normal control group, including 10 males and 20 females, aged 10 - 75 years with a mean age of (31±5) years old. Approval was obtained from the hospital ethic committee.METHODS: After admission, the patients were examined with sympathetic skin response using DANTEC keypoint 2.0 electromyography evoked potential apparatus (Danmark). The changes of the latency and wave amplitude of sympathetic skin response were observed. The subjects in the normal control group were examined with the same methods at physical examination. Abnormality was judged by

  2. The role of Thymectomy in Remission of Myasthenia gravis patients with or without Thymoma

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    Manouchehr Aghajanzadeh

    2011-09-01

    Full Text Available Background: Myasthenia gravis is an auto immune disease thymus have important role in this disease which Thymoma is the primary tumor of thymus that is produced by augmentation of epithelial cells inside of Glands. Thymectomy can improved myasthenia gravis disease. The main aim of this study is to assess the remission among the myasthenic patients after thymectomy. Methods: From 1993 to 2004, 68 patients who underwent thymectomy for mysthenia gravis were studied. Myathenia was established by positive electromyography and tensilon tests. These patients were operated by one surgeon at teaching and private hospitals, the specimens were tested by pathologist and patients were under follow up for 12 to 48 months. In following- up time the patient’s answer to remedy was basis to daily use of Mestinone and myasthenia crisis was investigated. Results: The age of the patients was 14 to 60years. The average age of the patients who was 34/08 years old. In 42 of patients was female and 26 was men. Between the men, 11 had complete remission 14 partial remission and one no answer. Between the female, 21 had complete remission. 19 partial remission and 2 no answer. This study had no inhospital mortality, but in follow up we 4/4% mortality seen. In pathology studies, 21 patients had thymoma, 45 had hyperplasia, and 2 had normal thymus. In 21 patients with thymoma, 11 had complete remission, 10 partial remissions. Between 45 patients with hyperplasia, 20 complete remission, 22 partial remission and 3 no answer. From two normal pathology reports, 1 complete and one partial remission. Conclusion: We conclude that thymectomy is a beneficial procedure for myasthenia gravis patients with a complete clinical remission rate in our series. Therefore we conclude thymectomy for myasthenia gravis patients is indicated as early as possible in the course of disease.

  3. Lupus eritematoso sistêmico associado a miastenia gravis: relato de caso Systemic lupus erythematosus and myasthenia gravis: case report

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    MARCIO F. DE CARVALHO

    1998-03-01

    Full Text Available Os autores descrevem o caso de uma mulher branca de 24 anos de idade admitida com lupus eritematoso sistêmico (com 4 anos de evolução de doença e início recente de miastenia gravis. São discutidos os principais diagnósticos diferenciais para a fraqueza muscular e a fadiga apresentadas por esta paciente. Uma revisão de literatura abordando a associação de miastenia gravis e lupus eritematoso é feita, com ênfase às características clínicas desses pacientes e ao papel do timoma e timectomia no desenvolvimento de lupus eritematoso em pacientes previamente miastênicos.We report the case of a 24-year-old white woman admitted with a four year diagnosis of systemic lupus erythematosus and the recent onset of myasthenia gravis discussing the main differential diagnosis of weakness and fatigue in this patient. A review of literature approaching the association of myasthenia gravis and systemic lupus erythematosus is also done with emphasis on the clinical characteristics of these patients and the role of thymoma and thymectomy in the development of systemic lupus erythematosus in myasthenic patients.

  4. Anestesia peridural torácica para cirurgia plástica de mama em paciente portadora de miastenia gravis: relato de caso Anestesia peridural torácica para cirugía plástica de mama en paciente portadora de miastenia gravis: relato de caso Thoracic epidural anesthesia for mammaplasty in myasthenia gravis patient: case report

    Directory of Open Access Journals (Sweden)

    Fabiano Timbó Barbosa

    2005-06-01

    Full Text Available JUSTIFICATIVA E OBJETIVOS: A miastenia gravis é uma doença crônica, auto-imune, caracterizada pela fraqueza da musculatura esquelética em decorrência da diminuição dos receptores de acetilcolina na junção neuromuscular. O objetivo deste relato é mostrar um caso de paciente com miastenia gravis submetida a anestesia peridural torácica para cirurgia plástica de mama. RELATO DO CASO: Paciente com 51 anos, portadora de miastenia gravis foi submetida a anestesia peridural torácica com bupivacaína e fentanil. Não houve sinais de depressão respiratória. A paciente recebeu alta hospitalar após 36 horas. CONCLUSÕES: O presente caso sugere como conduta anestésica para o paciente portador de miastenia gravis a anestesia peridural como técnica única, sem a obrigatoriedade de intubação orotraqueal.JUSTIFICATIVA Y OBJETIVOS: La miastenia gravis es una enfermedad crónica, auto-inmune, caracterizada por la debilidad de la musculatura esquelética resultante de la disminución de los receptores de acetilcolina en la unión neuromuscular. El objetivo de este relato es mostrar el caso de una paciente con miastenia gravis sometida a anestesia peridural torácica para una cirugía plástica de mama. RELATO DEL CASO: Paciente del sexo femenino, 51 años, portadora de miastenia gravis fue sometida a anestesia peridural torácica con bupivacaína y fentanil. No hubo señales de depresión respiratoria. La paciente recibió alta hospitalaria después de 36 horas. CONCLUSIONES: Este actual caso sugiere como conducta anestésica para el paciente portador de miastenia gravis la anestesia peridural como única técnica, sin la obligatoriedad de intubación orotraqueal.BACKGROUND AND OBJECTIVES: Myasthenia gravis is a chronic autoimmune disease characterized by skeletal muscles weakness promoted by decreased acetylcholine receptors in the neuromuscular junction. This report aimed at describing a case of myasthenia gravis patient submitted to thoracic

  5. Gene-Specific Methylation Analysis in Thymomas of Patients with Myasthenia Gravis

    Science.gov (United States)

    Lopomo, Angela; Ricciardi, Roberta; Maestri, Michelangelo; De Rosa, Anna; Melfi, Franca; Lucchi, Marco; Mussi, Alfredo; Coppedè, Fabio; Migliore, Lucia

    2016-01-01

    Thymomas are uncommon neoplasms that arise from epithelial cells of the thymus and are often associated with myasthenia gravis (MG), an autoimmune disease characterized by autoantibodies directed to different targets at the neuromuscular junction. Little is known, however, concerning epigenetic changes occurring in thymomas from MG individuals. To further address this issue, we analyzed DNA methylation levels of genes involved in one-carbon metabolism (MTHFR) and DNA methylation (DNMT1, DNMT3A, and DNMT3B) in blood, tumor tissue, and healthy thymic epithelial cells from MG patients that underwent a surgical resection of a thymic neoplasm. For the analyses we applied the methylation-sensitive high-resolution melting technique. Both MTHFR and DNMT3A promoters showed significantly higher methylation in tumor tissue with respect to blood, and MTHFR also showed significantly higher methylation levels in tumor tissue respect to healthy adjacent thymic epithelial cells. Both DNMT1 and DNMT3B promoter regions were mostly hypomethylated in all the investigated tissues. The present study suggests that MTHFR methylation is increased in thymomas obtained from MG patients; furthermore, some degrees of methylation of the DNMT3A gene were observed in thymic tissue with respect to blood. PMID:27999265

  6. Gene-Specific Methylation Analysis in Thymomas of Patients with Myasthenia Gravis

    Directory of Open Access Journals (Sweden)

    Angela Lopomo

    2016-12-01

    Full Text Available Thymomas are uncommon neoplasms that arise from epithelial cells of the thymus and are often associated with myasthenia gravis (MG, an autoimmune disease characterized by autoantibodies directed to different targets at the neuromuscular junction. Little is known, however, concerning epigenetic changes occurring in thymomas from MG individuals. To further address this issue, we analyzed DNA methylation levels of genes involved in one-carbon metabolism (MTHFR and DNA methylation (DNMT1, DNMT3A, and DNMT3B in blood, tumor tissue, and healthy thymic epithelial cells from MG patients that underwent a surgical resection of a thymic neoplasm. For the analyses we applied the methylation-sensitive high-resolution melting technique. Both MTHFR and DNMT3A promoters showed significantly higher methylation in tumor tissue with respect to blood, and MTHFR also showed significantly higher methylation levels in tumor tissue respect to healthy adjacent thymic epithelial cells. Both DNMT1 and DNMT3B promoter regions were mostly hypomethylated in all the investigated tissues. The present study suggests that MTHFR methylation is increased in thymomas obtained from MG patients; furthermore, some degrees of methylation of the DNMT3A gene were observed in thymic tissue with respect to blood.

  7. Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis

    Directory of Open Access Journals (Sweden)

    P J Lorenzoni

    2011-01-01

    Full Text Available Myasthenia gravis (MG is an immune-mediated disease that compromises the postsynaptic membrane of the neuromuscular junction. Primary sclerosing cholangitis (PSC is considered an immune-mediated cholestatic liver disease. Both MG and PSC include an autoimmune pathogenesis, so there is some evidence that patients with MG or PSC have a higher risk of developing autoantibodies and other immune disorders than normal controls, but the coexistence of these two disorders has never been documented. We report a 40-year-old woman who presented with MG when she was 20 years old and developed PSC 20 years after a thymectomy. Liver biochemistry revealed cholestasis. Magnetic resonance imaging showed multifocal strictures and beads involving the intrahepatic bile ducts. A liver biopsy confirmed sclerosing cholangitis. Serological analysis demonstrated positive autoantibodies (Anti-nuclear antibodies, anti-smooth muscle antibodies. Repetitive stimulation had a decremental response, and antibodies to acetylcholine receptors were detectable. To our knowledge, this is the first case of PSC in a patient with MG. The main characteristics of both MG and PSC combination are discussed.

  8. Profile of upregulated inflammatory proteins in sera of Myasthenia Gravis patients

    Science.gov (United States)

    Molin, Carl Johan; Westerberg, Elisabet; Punga, Anna Rostedt

    2017-01-01

    This study describes specific patterns of elevated inflammatory proteins in clinical subtypes of myasthenia gravis (MG) patients. MG is a chronic, autoimmune neuromuscular disease with antibodies most commonly targeting the acetylcholine receptors (AChRab), which causes fluctuating skeletal muscle fatigue. MG pathophysiology includes a strong component of inflammation, and a large proportion of patients with early onset MG additionally present thymus hyperplasia. Due to the fluctuating nature and heterogeneity of the disease, there is a great need for objective biomarkers as well as novel potential inflammatory targets. We examined the sera of 45 MG patients (40 AChRab seropositive and 5 AChRab seronegative), investigating 92 proteins associated with inflammation. Eleven of the analysed proteins were significantly elevated compared to healthy controls, out of which the three most significant were: matrix metalloproteinase 10 (MMP-10; p = 0.0004), transforming growth factor alpha (TGF-α; p = 0.0017) and extracellular newly identified receptor for advanced glycation end-products binding protein (EN-RAGE) (also known as protein S100-A12; p = 0.0054). Further, levels of MMP-10, C-X-C motif ligand 1 (CXCL1) and brain derived neurotrophic factor (BDNF) differed between early and late onset MG. These novel targets provide valuable additional insight into the systemic inflammatory response in MG. PMID:28045063

  9. B cells produce less IL-10, IL-6 and TNF-α in myasthenia gravis.

    Science.gov (United States)

    Yilmaz, Vuslat; Oflazer, Piraye; Aysal, Fikret; Parman, Yeşim G; Direskeneli, Haner; Deymeer, Feza; Saruhan-Direskeneli, Güher

    2015-06-01

    B cells from myasthenia gravis (MG) patients with autoantibodies (Aab) against acetylcholine receptor (AChR), muscle-specific kinase (MuSK) or with no detectable Aab were investigated as cytokine producing cells in this study. B cells were evaluated for memory phenotypes and expressions of IL-10, IL-6 and IL-12A. Induced productions of IL-10, IL-6, IL-12p40, TNF-α and LT from isolated B cells in vitro were measured by immunoassays. MG patients receiving immunosuppressive treatment had higher proportions of memory B cells compared with healthy controls and untreated patients. With CD40 stimulation MG patients produced significantly lower levels of IL-10, IL-6. With CD40 and B cell receptor stimulation of B cells, TNF-α production also decreased in addition to these cytokines. The lower levels of these cytokine productions were not related to treatment. Our results confirm a disturbance of B cell subpopulations in MG subgroups on immunosuppressive treatment. B cell derived IL-10, IL-6 and TNF-α are down-regulated in MG, irrespective of different antibody productions. Ineffective cytokine production by B cells may be a susceptibility factor in dysregulation of autoimmune Aab production.

  10. B10 Cell Frequencies and Suppressive Capacity in Myasthenia Gravis Are Associated with Disease Severity

    Science.gov (United States)

    Yi, John S.; Russo, Melissa A.; Massey, Janice M.; Juel, Vern; Hobson-Webb, Lisa D.; Gable, Karissa; Raja, Shruti M.; Balderson, Kristina; Weinhold, Kent J.; Guptill, Jeffrey T.

    2017-01-01

    Myasthenia gravis (MG) is a T cell-dependent, B cell-mediated disease. The mechanisms for loss of self-tolerance in this disease are not well understood, and recently described regulatory B cell (Breg) subsets have not been thoroughly investigated. B10 cells are a subset of Bregs identified by the production of the immunosuppressive cytokine, interleukin-10 (IL-10). B10 cells are known to strongly inhibit B- and T-cell inflammatory responses in animal models and are implicated in human autoimmunity. In this study, we examined quantitative and qualitative aspects of B10 cells in acetylcholine receptor autoantibody positive MG (AChR-MG) patients and healthy controls. We observed reduced B10 cell frequencies in AChR-MG patients, which inversely correlated with disease severity. Disease severity also affected the function of B10 cells, as B10 cells in the moderate/severe group of MG patients were less effective in suppressing CD4 T-cell proliferation. These results suggest that B10 cell frequencies may be a useful biomarker of disease severity, and therapeutics designed to restore B10 cell frequencies could hold promise as a treatment for this disease through restoration of self-tolerance. PMID:28239367

  11. Keep An Eye Out For Myasthenia Gravis Patients With An Eye Out

    Directory of Open Access Journals (Sweden)

    A Arturo eLeis

    2014-07-01

    Full Text Available Eye trauma and blindness are common in the United States, with an incidence of over 2 million cases per year and 25 million blind adults, respectively. However, literature is surprisingly scarce on the potential confounding effect of eye trauma or blindness on the diagnosis of myasthenia gravis (MG, an autoimmune neuromuscular disease in which fluctuating ocular symptoms are the most distinguishing feature. We present the case of a 75 year-old man with eye enucleation referred for electrodiagnostic evaluation of the right upper limb after an accidental fall. Neurological examination showed proximal muscle weakness, but MG was not initially considered because the patient lacked the classic ocular symptoms of MG. The delay in diagnosis resulted in worsening of systemic MG symptoms, although in other patients it may have precipitated MG crisis or possibly death. Greater awareness that eye trauma or blindness can prevent expression of ocular symptoms in neuromuscular disorders is needed to avoid morbidity associated with an erroneous or delayed diagnosis.

  12. A Comparison of Epitope Repertoires Associated with Myasthenia Gravis in Humans and Nonhuman Hosts

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    Kerrie Vaughan

    2012-01-01

    Full Text Available Here we analyzed the molecular targets associated with myasthenia gravis (MG immune responses, enabled by an immune epitope database (IEDB inventory of approximately 600 MG-related epitopes derived from 175 references. The vast majority of epitopes were derived from the α-subunit of human AChR suggesting that other MG-associated autoantigens should be investigated further. Human α-AChR was mostly characterized in humans, whereas reactivity primarily to T. californica AChR was examined in animal models. While the fine specificity of T-cell response was similar in the two systems, substantial antibody reactivity to the C-terminus was detected in the nonhuman system, but not in humans. Further analysis showed that the reactivity of nonhuman hosts to the C-terminus was eliminated when data were restricted to hosts tested in the context of autoimmune disease (spontaneous or induced, demonstrating that the epitopes recognized in humans and animals were shared when disease was present. Finally, we provided data subsets relevant to particular applications, including those associated with HLA typing or restriction, sets of epitopes recognized by monoclonal antibodies, and epitopes associated with modulation of immunity or disease. In conclusion, this analysis highlights gaps, differences, and similarities in the epitope repertoires of humans and animal models.

  13. Titin antibodies in "seronegative" myasthenia gravis--A new role for an old antigen.

    Science.gov (United States)

    Stergiou, C; Lazaridis, K; Zouvelou, V; Tzartos, J; Mantegazza, R; Antozzi, C; Andreetta, F; Evoli, A; Deymeer, F; Saruhan-Direskeneli, G; Durmus, H; Brenner, T; Vaknin, A; Berrih-Aknin, S; Behin, A; Sharshar, T; De Baets, M; Losen, M; Martinez-Martinez, P; Kleopa, K A; Zamba-Papanicolaou, E; Kyriakides, T; Kostera-Pruszczyk, A; Szczudlik, P; Szyluk, B; Lavrnic, D; Basta, I; Peric, S; Tallaksen, C; Maniaol, A; Gilhus, N E; Casasnovas Pons, C; Pitha, J; Jakubíkova, M; Hanisch, F; Bogomolovas, J; Labeit, D; Labeit, S; Tzartos, S J

    2016-03-15

    Myasthenia gravis (MG) is an autoimmune disease caused by antibodies targeting the neuromuscular junction of skeletal muscles. Triple-seronegative MG (tSN-MG, without detectable AChR, MuSK and LRP4 antibodies), which accounts for ~10% of MG patients, presents a serious gap in MG diagnosis and complicates differential diagnosis of similar disorders. Several AChR antibody positive patients (AChR-MG) also have antibodies against titin, usually detected by ELISA. We have developed a very sensitive radioimmunoprecipitation assay (RIPA) for titin antibodies, by which many previously negative samples were found positive, including several from tSN-MG patients. The validity of the RIPA results was confirmed by western blots. Using this RIPA we screened 667 MG sera from 13 countries; as expected, AChR-MG patients had the highest frequency of titin antibodies (40.9%), while MuSK-MG and LRP4-MG patients were positive in 14.6% and 16.4% respectively. Most importantly, 13.4% (50/372) of the tSN-MG patients were also titin antibody positive. None of the 121 healthy controls or the 90 myopathy patients, and only 3.6% (7/193) of other neurological disease patients were positive. We thus propose that the present titin antibody RIPA is a useful tool for serological MG diagnosis of tSN patients.

  14. Binding affinities of anti-acetylcholine receptor autoantibodies in myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Bray, J.J.; Drachman, D.B.

    1982-01-01

    Antibodies directed against acetylcholine (ACh) receptors are present in the sera of nearly 90% of patients with myasthenia gravis (MG), and are involved in the pathogenesis of this autoimmune disease. However, the antibody titers measured by the standard radioimmunoassay correspond poorly with the clinical severity of the disease. To determine whether this disparity could be accounted for by differences in the binding affinities of anti-ACh receptor antibodies in different patients, we have measured the binding affinities of these autoantibodies in 15 sera from MG patients. The affinity constants (K/sub o/), as determined by Scatchard analysis, were all in the range of 10/sup 10/ M/sup -1/, comparable to the highest values reported in immunized animals. The affinity constants were truly representative of the population of autoantibodies detected by the radioimmunoassay, as shown by the remarkable linearity of the Scatchard plots (r/sup 2/>0.90) and the close correlation between the antibody titers determined by extrapolation of the Scatchard plots and by saturation analysis (r = 0.99; p < 0.001). There was only a 6-fold variation in affinity constants measured in this series of patients despite widely differing antibody titers and severity of the disease. Factors other than the titer and affinity of anti-ACh receptor antibodies may correlate better with the clinical manifestations of MG.

  15. Dysregulation of B Cell Repertoire Formation in Myasthenia Gravis Patients Revealed through Deep Sequencing.

    Science.gov (United States)

    Vander Heiden, Jason A; Stathopoulos, Panos; Zhou, Julian Q; Chen, Luan; Gilbert, Tamara J; Bolen, Christopher R; Barohn, Richard J; Dimachkie, Mazen M; Ciafaloni, Emma; Broering, Teresa J; Vigneault, Francois; Nowak, Richard J; Kleinstein, Steven H; O'Connor, Kevin C

    2017-02-15

    Myasthenia gravis (MG) is a prototypical B cell-mediated autoimmune disease affecting 20-50 people per 100,000. The majority of patients fall into two clinically distinguishable types based on whether they produce autoantibodies targeting the acetylcholine receptor (AChR-MG) or muscle specific kinase (MuSK-MG). The autoantibodies are pathogenic, but whether their generation is associated with broader defects in the B cell repertoire is unknown. To address this question, we performed deep sequencing of the BCR repertoire of AChR-MG, MuSK-MG, and healthy subjects to generate ∼518,000 unique VH and VL sequences from sorted naive and memory B cell populations. AChR-MG and MuSK-MG subjects displayed distinct gene segment usage biases in both VH and VL sequences within the naive and memory compartments. The memory compartment of AChR-MG was further characterized by reduced positive selection of somatic mutations in the VH CDR and altered VH CDR3 physicochemical properties. The VL repertoire of MuSK-MG was specifically characterized by reduced V-J segment distance in recombined sequences, suggesting diminished VL receptor editing during B cell development. Our results identify large-scale abnormalities in both the naive and memory B cell repertoires. Particular abnormalities were unique to either AChR-MG or MuSK-MG, indicating that the repertoires reflect the distinct properties of the subtypes. These repertoire abnormalities are consistent with previously observed defects in B cell tolerance checkpoints in MG, thereby offering additional insight regarding the impact of tolerance defects on peripheral autoimmune repertoires. These collective findings point toward a deformed B cell repertoire as a fundamental component of MG.

  16. T-lymphocyte-rich thymoma and myasthenia gravis in a Siberian tiger (Panthera tigris altaica).

    Science.gov (United States)

    Allan, K; Masters, N; Rivers, S; Berry, K; Routh, A; Lamm, C

    2014-01-01

    A 10-year-old captive male Siberian tiger (Panthera tigris altaica) presented with acute onset collapse, vomiting and dyspnoea, preceded by a 6-month period of progressive muscle wasting. Following humane destruction, post-mortem examination revealed a large multilobulated mass in the cranial mediastinum, which was diagnosed as a T-lymphocyte-rich thymoma with the aid of immunohistochemistry. Retrospective serology for acetylcholine receptor antibodies (titre 3.90 nmol/l) confirmed a diagnosis of thymoma-associated myasthenia gravis. Thymomas are reported rarely in wild carnivores, but when detected they appear to be similar in morphology to those seen in domestic carnivores and may also be accompanied by paraneoplastic syndromes. The clinical signs of myasthenia gravis in the tiger were consistent with those reported in cats and dogs and the condition is proposed as an important differential diagnosis for generalized weakness in captive Felidae.

  17. A case report of subclinical hypercortisolism due to adrenal incidentaloma complicated by myasthenia gravis after adrenalectomy.

    Science.gov (United States)

    Petramala, Luigi; Marinelli, Cristiano; Giallonardo, Anna Teresa; Concistrè, Antonio; Lucia, Piernatale; Venuta, Federico; Cerbelli, Bruna; Ciardi, Antonio; De Toma, Giorgio; Letizia, Claudio

    2016-11-11

    A 62-year-old woman was admitted for evaluation of an incidentally discovered adrenal mass and hypertension. CT scan revealed a 7 cm mass in the right adrenal gland. After careful examination, the patient was diagnosed with subclinical hypercortisolism (SH). Adrenalectomy was performed. Histopathological examination showed an adrenocortical adenoma. Symptoms and signs of myasthenia gravis appeared 5 months later. CT of the chest showed a solid tissue mass in the mediastinum. The patient underwent a sternotomy with excision of the tumor, which histologically proved to be a type 2B thymoma. We describe a rare case of SH due to an incidentally discovered adrenocortical adenoma in a patient who manifested myasthenia gravis after surgical remission of the cortisol excess.

  18. Myasthenia gravis as a 'stroke mimic'--it's all in the history.

    Science.gov (United States)

    Shaik, Saiffuddin; Ul-Haq, Mian Ayaz; Emsley, Hedley C A

    2014-12-01

    An 85-year-old man presented to hospital as an emergency having difficulties with swallowing and speech. In the emergency department, he was assessed as having acute onset dysphagia and dysarthria in keeping with an acute stroke. Subsequently, it became apparent that although the symptoms were indeed of relatively acute onset, there was a clear description by the patient of fatigability and diurnal variation, prompting a working clinical diagnosis of myasthenia gravis. The patient followed a turbulent clinical course, and interpretation of investigation results proved not to be straightforward in the acute setting. Myasthenia gravis is an uncommon disorder but it is more common in the elderly. This case provides key learning points, particularly highlighting the value of prompt, accurate clinical assessment and the importance of adhering to the clinical diagnostic formulation.

  19. Lúpus Eritematoso Sistêmico com fraqueza muscular por Miastenia Gravis Systemic Lupus Erythematosus with muscle weakness due to Myasthenia Gravis

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    Sâmia Araújo de Sousa Studart

    2011-06-01

    Full Text Available O Lúpus Eritematoso Sistêmico (LES e a Miastenia Gravis (MG são doenças autoimunes cuja associação em um mesmo paciente é raramente descrita. Essas patologias compartilham algumas características como acometimento de mulheres jovens, positividade para anticorpos antinucleares, evolução em períodos de exacerbações e remissões. O presente relato de caso analisa as possíveis hipóteses diagnósticas para um quadro clínico de ptose palpebral e fraqueza muscular proximal em uma paciente portadora de lúpus recente que evoluiu com MG associada.Systemic lupus erythematosus (SLE and myasthenia gravis (MG are autoimmune diseases, whose association in the same patient is rarely reported. Both pathologies share the following characteristics: affect mainly young women; alternate exacerbation and remission periods; and have positive antinuclear antibody (ANA test. This case report assesses possible diagnostic hypotheses for the clinical findings of eyelid ptosis and proximal muscle weakness in a female patient recently diagnosed with SLE, who evolved with associated MG.

  20. Characterization of B cells in muscle-specific kinase antibody myasthenia gravis

    OpenAIRE

    Guptill, Jeffrey T.; Yi, John S.; Sanders, Donald B.; Guidon, Amanda C.; Juel, Vern C; Massey, Janice M.; Howard, James F.; Scuderi, Flavia; Bartoccioni, Emanuela; Evoli, Amelia; Kent J Weinhold

    2015-01-01

    Objective: To characterize B-cell subsets in patients with muscle-specific tyrosine kinase (MuSK) myasthenia gravis (MG). Methods: In accordance with Human Immunology Project Consortium guidelines, we performed polychromatic flow cytometry and ELISA assays in peripheral blood samples from 18 patients with MuSK MG and 9 healthy controls. To complement a B-cell phenotype assay that evaluated maturational subsets, we measured B10 cell percentages, plasma B cell–activating factor (BAFF) levels, a...

  1. Appearance of Systemic Lupus Erythematosus in Patients with Myasthenia Gravis following Thymectomy: Two Case Reports

    OpenAIRE

    2004-01-01

    We report two cases of systemic lupus erythematosus (SLE) in myasthenia gravis (MG) patients who had undergone thymectomy. SLE developed in the patients 3 months or 13 yr after thymectomy, and polyarthritis was the main clinical manifestation of SLE. Both patients fulfilled at least four of the revised criteria for the classification of SLE. In this report, we describe two postthymectomy lupus patients and perform a comparative review of previous cases.

  2. Appearance of systemic lupus erythematosus in patients with myasthenia gravis following thymectomy: two case reports.

    Science.gov (United States)

    Park, Mi Jeong; Kim, Yun A; Lee, Shin Seok; Kim, Byeong Chae; Kim, Myeong Kyu; Cho, Ki Hyun

    2004-02-01

    We report two cases of systemic lupus erythematosus (SLE) in myasthenia gravis (MG) patients who had undergone thymectomy. SLE developed in the patients 3 months or 13 yr after thymectomy, and polyarthritis was the main clinical manifestation of SLE. Both patients fulfilled at least four of the revised criteria for the classification of SLE. In this report, we describe two postthymectomy lupus patients and perform a comparative review of previous cases.

  3. Von Hippel-Lindau disease associated with myasthenia gravis not related to thymoma

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    Paolo Pozzato

    2013-04-01

    Full Text Available BACKGROUND Von Hippel-Lindau disease (VHL is a rare autosomal dominant inherited disorder characterized by an increased risk of tumours in a number of locations (eyes, brain, adrenal gland, pancreas, liver, kidneys, or other areas of the body. It is caused by germline mutation in the VHL gene. The VHL gene is a tumour suppressor gene that has been identified on the short arm of chromosome 3. CASE REPORT We report a case of a 60 year-old female with the clinical diagnosis of VHL type 1 (cerebellar haemangioblastoma, pancreatic cysts with subsequent steatorrhoea, and bilateral renal carcinoma who developed weakness and fatigability of skeletal muscles, left lid ptosis, snarling expression and nasal timbre speech. Acetylcholine receptor antibodies were negative in serum, while the electrodiagnostic test demonstrated an alteration of neuromuscolar junction which was consistent with the diagnosis of myasthenia gravis. Contrast-enhanced TC scan of the anterior mediastinum was performed, which excluded thymus enlargement. VHL gene evaluation in this patient identified a new mutation (c279delC9 and polymorphism c291C>G. At present the patient still suffers from ataxia and dysmetria due to cerebellar involvement in VHL, while fatigue and lid ptosis improved after the treatment with oral pyridostigmine 60 mg tid. DISCUSSION AND CONCLUSIONS To our knowledge this is the first report of a case of VHL associated with myasthenia gravis without thymoma. A case of VHL associated with a form of myasthenia gravis related to thymoma has been recently reported. In our case the absence of acetylcholine receptor antibodies may suggest a genetic origin also for the myasthenia gravis.

  4. Teste do gelo no diagnóstico de miastenia gravis Ice pack test in the diagnosis of myasthenia gravis

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    Nilson Lopes da Fonseca Junior

    2010-04-01

    Full Text Available OBJETIVO: Demonstrar a sensibilidade e especificidade do teste do gelo no diagnóstico diferencial de ptose palpebral por miastenia gravis. MÉTODOS: Estudo prospectivo tipo ensaio clínico com grupo controle. Foi realizado o teste do gelo em pacientes portadores de ptose palpebral. Os pacientes foram divididos em 2 grupos, sendo o grupo I constituído por pacientes com miastenia gravis e o grupo II (controle formado por pacientes portadores de ptose congênita, miogênica não-miastênica ou aponeurótica. RESULTADOS: Todos os pacientes do grupo I tiveram aumento da fenda palpebral de, no mínimo, 3 mm após a aplicação do gelo. Nenhum paciente do grupo II apresentou incremento da fenda palpebral após o teste. CONCLUSÃO: O teste do gelo mostrou-se específico para detecção de ptose palpebral de causa miastênica.PURPOSE: To demonstrate the sensitivity and the specificity of the ice test in the differential diagnosis of ptosis in myasthenia gravis. METHODS: Prospective trial with a control group. The patients were instructed to hold a frozen ice pack on the closed ptotic eyelid. They were divided into 2 groups, with group I consisting of patients with myasthenia gravis and group II (control consisting of patients with congenital, non-myasthenic myogenic or aponeurotic ptosis. RESULTS: All patients in group I had increased palpebral fissure for at least 3 mm after the application of ice pack. No patient in group II showed increased palpebral fissure after the ice test. CONCLUSION: Ice test proved to be specific for the detection of myasthenic ptosis.

  5. TRACHEAL INTUBATION USING McGRATH VIDEO LARYNGOSCOPE IN MYASTHENIA GRAVIS PATIENTS

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    Venkata Sesha Sai Krishna

    2015-07-01

    Full Text Available BACKGROUND : Mc G rath video laryngoscope has been successfully used for managing difficult intubation in various clinical scenarios. In this case series, we aimed to evaluate the success rate and time taken to complete intubation without using muscle relaxants in myasthenia gravis patients coming for thymectomy. METHODS: We prospectively evaluate the use of Mcgrath video laryngoscope for intubation in ten myasthenia gravis patients coming for thymectomy. Intubation time, total time to complete tracheal intubation, laryngoscopic view (Cormack & Lehane grade, and patients tolerance of the procedure were recorded. RESULTS: The procedure was successful in all patients the mean (SD intubation time and total time to complete the tracheal intubation was 4.82 (0.53 and 10.21 (0.81 min, respectively. The laryngeal view was grade I in five and grade II in four patients. CONCLUSION: The Mcgrath Video Laryngoscope allowed a quicker intubation time, fewe r intubation attempts and greater ease of intubation in myasthenia gravis patients coming for thymectomy.

  6. The Anti-Acetylcholine Receptor Antibody Test in Suspected Ocular Myasthenia Gravis

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    Jung Jin Lee

    2014-01-01

    Full Text Available Aim. To estimate the clinical significance of anti-acetylcholine receptor antibody (anti-AChR-Ab levels in suspected ocular myasthenia gravis. Methods. In total, 144 patients complaining of fluctuating diplopia and ptosis were evaluated for serum levels of anti-acetylcholine receptor antibody and their medical charts were retrospectively reviewed. Subjects were classified into three groups: variable diplopia only, ptosis only, and both variable diplopia and ptosis. We investigated serum anti-AChR-Ab titer levels and performed thyroid autoantibody tests. Results. Patients’ chief complaints were diplopia (N=103, ptosis (N=12, and their concurrence (N=29. Abnormal anti-AChR-Ab was observed in 21 of 144 patients (14.1%. Between the three groups, mean age, number of seropositive patients, and mean anti-AChR-Ab level were not significantly different (P=0.224, 0.073, and 0.062, resp.. Overall, 27.5% of patients had abnormal thyroid autoantibodies. Conclusion. The sensitivity of anti-AChR-Ab was 14.1% in suspected ocular myasthenia gravis and seropositivity in myasthenia gravis patients showed a high correlation with the presence of thyroid autoantibodies.

  7. Is thymectomy in non-thymomatous myasthenia gravis of any benefit?

    Science.gov (United States)

    Diaz, Andres; Black, Edward; Dunning, Joel

    2014-03-01

    A best evidence topic in thoracic surgery was written according to a structured protocol. The question addressed was if thymectomy in non-thymomatous myasthenia gravis was of any benefit? Overall, 137 papers were found using the reported search, of which 16 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. The outcome variables were similar in all of the papers, including complete stable remission (CSR), pharmacological remission, age at presentation, gender, duration of symptoms, preoperative classification (Oosterhius, Osserman or myasthenia gravis Foundation of America (MGFA)), thymic pathology, preoperative medications (steroids, immunosuppressants), mortality and morbidity. We conclude that evidence-based reviews have shown that relative rates of thymectomy patients compared with non-thymectomy patients attaining outcome indicate that the former group of patients is more likely to achieve medication-free remission, become asymptomatic and clinically improve (54%, P < 0.01), particularly patients with severe and generalized symptoms (P = 0.007). Patients with generalized myasthenia gravis showed 11% stronger association with favourable outcomes after thymectomy. Some studies show early remission rates (RRs), as early as 6 months post-thymectomy, of 44%. Overall, the reported remission rate for non-thymomatous myasthenia gravis is between 38 and 72% up to 10 years of follow-up. Among these patients, those with thymic hyperplasia show the best complete stable remission rates (42%, P < 0.04) in the majority of studies. Age showed variability across the studies and the cut-off was also different among them. Overall age < 45 years showed a higher probability of achieving complete stable remission during follow-up (81% benefit rate (BR), P < 0.02). Pharmacological improvement is reported between 6 and 42

  8. A Case of Myasthenia Gravis Presenting Solely With Bulbar Palsy Not Associated With Easy Fatigability.

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    Pinank R. Mer

    2015-12-01

    Full Text Available Introduction: Myesthenia Gravis (MG is a neuromuscular disorder characterized by weakness and fatigability of skeletal muscles. The underlying defect is a decrease in number of available acetylcholine receptors (AChRs at neuromuscular junction due to an antibody-mediated autoimmune attack. Case Report: A 26 year old male patient from Dhar (M.P., a tribal district, presented to civil hospital Ahmedabad (CHA with complaints of unable to drink water since 5 months. Patient complained of water coming out from nose. Gradually he developed inability to eat also, with no evidence of vomiting.2 months later patient developed slurring of speech which was followed by total inability to speak. Along with that he developed diplopia on binocular vision. No such symptoms in siblings were there. On examination general examination was normal. On CNS examination he was conscious oriented with normal planter and deep tendon reflexes. On cranial nerve examination 3, 7, 9, 10th nerve palsies seen in the form of absence of medial & upward movement of left eye (3rd, inability to close eyes completely (3rd, reduced power and tone of buccinators muscle (7th, gag reflex absent (9th, presence of dysphagia (10th. Rest of the CNS findings were normal. He was given ryle’s tube feeding and investigated.CBC, LFT, RFT, urine routine-micro were normal.CSF examination showed mild increase of protein.MRI brain was normal. Vitamin B12, TSH, Calcium, HbA1c, ESR, RA factor were normal.ANA was (+ with nuclear speckled pattern. Fundus examination was normal with abnormal ocular movement as described.VEP study was normal.EMG NCV showed reduced amplitude of bilateral facial nerve, involvement of right oculomotor nerve suggesting bulbar myasthenia >generalized myasthenia. Then we did AChR antibody test which came highly positive. He was started T.Pyridostigmine 60mg TDS and T. Prednisolone 5mg/kg and discharged. On follow up we found marked improvement of symptoms. Conclusion: The

  9. Characterization of CD4 and CD8 T cell responses in MuSK myasthenia gravis.

    Science.gov (United States)

    Yi, J S; Guidon, A; Sparks, S; Osborne, R; Juel, V C; Massey, J M; Sanders, D B; Weinhold, K J; Guptill, J T

    2014-08-01

    Muscle specific tyrosine kinase myasthenia gravis (MuSK MG) is a form of autoimmune MG that predominantly affects women and has unique clinical features, including prominent bulbar weakness, muscle atrophy, and excellent response to therapeutic plasma exchange. Patients with MuSK MG have predominantly IgG4 autoantibodies directed against MuSK on the postsynaptic muscle membrane. Lymphocyte functionality has not been reported in this condition. The goal of this study was to characterize T cell responses in patients with MuSK MG. Intracellular production of IFN-gamma, TNF-alpha, IL-2, IL-17, and IL-21 by CD4+ and CD8+ T cells was measured by polychromatic flow cytometry in peripheral blood samples from 11 Musk MG patients and 10 healthy controls. Only one MuSK MG patient was not receiving immunosuppressive therapy. Regulatory T cells (Treg) were also included in our analysis to determine if changes in T cell function were due to altered Treg frequencies. CD8+ T cells from MuSK MG patients had higher frequencies of polyfunctional responses than controls, and CD4+ T cells had higher IL-2, TNF-alpha, and IL-17. MuSK MG patients had a higher percentage of CD4+ T cells producing combinations of IFN-gamma/IL-2/TNF-gamma, TNF-alpha/IL-2, and IFN-gamma/TNF-alpha. Interestingly, Treg numbers and CD39 expression were not different from control values. MuSK MG patients had increased frequencies of Th1 and Th17 cytokines and were primed for polyfunctional proinflammatory responses that cannot be explained by a defect in CD39 expression or Treg number.

  10. Using Xenopus tissue cultures for the study of myasthenia gravis pathogenesis.

    Science.gov (United States)

    Yeo, Hwee Li; Lim, Jorain Yu Ni; Fukami, Yuki; Yuki, Nobuhiro; Lee, Chi Wai

    2015-12-15

    Myasthenia gravis (MG), the most common autoimmune disease of neuromuscular junction (NMJ), is heterogeneous in terms of pathophysiology, which is determined by the pathogenic antigen of autoantibodies targeting to synaptic proteins at the NMJs. Currently, patients suspected with MG are routinely screened for the presence of autoantibodies against acetylcholine receptor (AChR) or muscle-specific kinase (MuSK) using a cell-based assay (CBA) that involves the expression of target synaptic membrane protein in heterologous cell lines. However, some autoantibodies may only show reactivity for binding to densely clustered AChR in the physiological conformation, while AChR clustering is known to involve signaling events orchestrated by over a dozen of postsynaptic proteins. To improve the existing serological diagnosis of MG, this study explored the possibility of using the well-established Xenopus primary culture system as a novel CBA for MG. Here, by examining the pathogenic effects of four MG human plasma samples, we found that the samples from both seropositive and seronegative MG patients effectively induced the disassembly of aneural AChR clusters in cultured Xenopus muscle cells, as well as the nerve-induced AChR clusters in the nerve-muscle co-cultures. Importantly, the disassembly of AChR clusters was spatio-temporally correlated to the disappearance of actin depolymerizing factor (ADF)/cofilin, an actin regulator involved in AChR trafficking and clustering. Taken together, this study develops a reliable CBA using Xenopus primary cultures for screening the pathogenicity of human MG plasma samples, and providing a platform for investigating the pathogenic mechanisms underlying the endocytic trafficking and degradation of AChRs at NMJs in MG patients.

  11. Thymus cells in myasthenia gravis selectively enhance production of anti-acetylcholine-receptor antibody by autologous blood lymphocytes

    Energy Technology Data Exchange (ETDEWEB)

    Newsom-Davis, J.; Willcox, N.; Calder, L.

    1981-11-26

    We investigated the role of the thymus in 16 patients with myasthenia gravis without thymoma by studying the production of anti-acetylcholine-receptor antibody by thymic and blood lymphocytes cultured alone or together. In 10 responders (with the highest receptor-antibody titers in their plasma), cultured thymic cells spontaneously produced measurable receptor antibody. Receptor-antibody production by autologous blood lymphocytes was enhanced by the addition of responder's thymic cells, irradiated to abrogate antibody production and suppression (P<0.01). This enhancement was greater and more consistent than that by pokeweed mitogen; it depended on viable thymic cells, appeared to be selective for receptor antibody, and correlated with the ratio of thymic helper (OKT4-positive or OKT4+) to suppressor (OKT8+) T cells (P<0.01). These results suggest that myasthenic thymus contains cell-bound acetylcholine-receptor-like material or specific T cells (or both) that can aid receptor-antibody production. This may be relevant to the benefits of thymectomy in myasthenia and to the breakdown in self-tolerance in this and other autoimmune diseases.

  12. Thoracic computer tomography for the evaluation of the thymus gland in cases of myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Druschky, K.F.; Stadler, H.W.; Daun, H.

    1981-01-01

    Hyperplasia of the thymus gland is observed in 65% of all patients with myasthenia gravis, while the incidence of thymus tumor is reported to be 8.5-28%. Conventional radiological techniques provide little information in the diagnosis of mediastinal lesions. Even a clearly developed thymus tumor can escape clinical detection. Since March 1978 thoracic computer tomography has been performed in addition to X-rays of the chest in a series of 19 patients with myasthenia ravis, 10 women and 9 men ranging in age from 15-71 years and in 3 patients with suspected thymomas but without myasthenia gravis. These examinations were carried out with a Somatom II (Siemens) since September 1979. On the average 15-20 scans were made at the level of the upper two-third of the sternum. The chest X-rays in 2 planes revealed signs of a thymus tumor in 3 female patients. Thoracic computer tomography showed definite signs of a space-occupying lesion in the anterior mediastinum in 11 cases. At thymectomy 6 patients were found to have hyperplasia of the thymus, 2 patients had a benign thymoma and 3 patients a malignant thymoma. In 6 cases computer tomography showed only slight changes and in 5 patients no pathological findings could be demonstrated in the thymus gland. Thoracic computer tomography is a relatively harmless diagnostic measure without any risk for the patient. It has a high resolution and great accuracy in the evaluation of the thymus gland and is therefore the method of choice for the diagnosis of patients with myasthenia gravis.

  13. Anesthetic Management of Direct Laryngoscopy and Dilatation of Subglottic Stenosis in a Patient with Severe Myasthenia Gravis

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    Hesham A. Elsharkawy

    2012-01-01

    Full Text Available We describe the anesthetic management of a patient with severe myasthenia gravis and tracheal stenosis; the patient was scheduled for direct laryngoscopy and dilatation. The combination of myasthenia gravis and tracheal obstruction presents several difficulties for anesthetic management. The airway is shared; therefore, any complications are also shared by the anesthesiologist and bronchoscopists. The potential for respiratory compromise in patients undergoing the two procedures requires that anesthesiologists be familiar with the underlying disease state, as well as the interaction of anesthetic and nonanesthetic drugs in a case involving myasthenia gravis. We reviewed the literature and report our experience in this case. There is no strong evidence for choosing one approach to general anesthesia over another for bronchoscopy. Careful preoperative planning and experience in airway management and jet ventilation are crucial to prevent an adverse outcome and obtain favorable results.

  14. Comparative analysis of postoperative outcomes of myasthenia gravis with and without thymoma

    Institute of Scientific and Technical Information of China (English)

    WANG Ru-wen; LIN Yi-dan; JIANG Yao-guang; XUE Zhi-qiang; ZHAO Yun-ping; MA Zheng

    2005-01-01

    Objective: To explore the postoperative clinical characteristics and outcomes of myasthenia gravis with and without thymoma. Methods: Two hundred and forty-three patients with myasthenia gravis (MG) surgically treated in our department from 1978 to 2003 were studied retrospectively. The clinical characteristics, complication, remissions and survival rates were compared between MG with and without thymoma. Results: The patients of MG with thymoma were significantly older (P<0. 01), had shorter duration of symptom(P<0.01), and higher myasthenia crisis rates after operation(P<0.01) than those without thymoma. No difference of Osserman classification was found between the 2 groups. The complete remission rates and partial remission rates of MG with thymoma were significantly lower than those without thymoma at 1 and 3 years(P=0. 049, P=0. 000, P=0. 015, P=0. 010), but no differences at 5 years (P= 0. 457, P= 0. 699). The survival rates were lower in MG with thymoma than without thymoma (Log rank = 18.58,P = 0. 000). Conclusion: The clinical characteristics are different between MG with and without thymoma. The remission of symptom of MG- with thymoma is worse than that of MG without thymoma in the near future, but similar in the long future. In a long-term, the death rate of MG with thymoma is significantly higher than that of MG without thymoma.

  15. Familial myasthenia gravis: report of four cases Miastenia grave familial: registro de quatro casos

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    José Lamartine de Assis

    1976-09-01

    Full Text Available Two pairs of siblings with myasthenia gravis, belonging to two different families, are reported. This is the only record of familial myasthenia during the past twenty years, in a total of 145 patients seen at the Neurological Clinic of the São Paulo Medical School. In spite of the fact that myasthenia gravis does not show hereditary characteristics, the peculiar features of the four cases justify the present report. The two pairs of siblings were born from non myasthenic nor consanguineous parents. The disease started at birth showing bilateral partial eyelid ptosis in all patients. The course of the illness has been favorable. There was no thymoma.Os autores registram dois pares de gêmeos com miastenia grave, pertencentes a duas famílias diferentes. Este é o único registro de miastenia familial durante os últimos 20 anos, num total de 145 pacientes examinados na Clínica Neurológica da FMUSP. Apesar do fato de a miastenia grave não ter características hereditárias, os aspectos peculiares dos quatro pacientes justificam o presente registro. Os dois pares de gêmeos nasceram de pais não miastênicos e sem consanguinidade. A doença iniciou-se no nascimento, evoluindo com ptose bilateral parcial da pálpebra superior precocemente em todos os pacientes. O curso da moléstia tem sido favorável. Não havia timoma.

  16. Progressive Outer Retinal Necrosis and Immunosuppressive Therapy in Myasthenia Gravis

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    Solène Coisy

    2014-04-01

    Full Text Available Introduction: Progressive outer retinal necrosis (PORN is a rare but devastating infectious retinitis associated with varicella zoster virus (VZV and responsible for severe visual loss. Case Report: A 59-year-old man treated for generalized myasthenia with oral azathioprine and prednisone presented with severe unilateral necrotizing retinitis. Polymerase chain reaction of the aqueous and vitreous humors was diagnostic for VZV PORN. Conclusion: VZV PORN is a severe potential ocular complication of immunosuppression, prompting urgent diagnosis and appropriate treatment.

  17. Successful treatment of severe myasthenia gravis developed after allogeneic hematopoietic stem cell transplantation with plasma exchange and rituximab.

    Science.gov (United States)

    Unal, Sule; Sag, Erdal; Kuskonmaz, Baris; Kesici, Selman; Bayrakci, Benan; Ayvaz, Deniz C; Tezcan, Ilhan; Yalnızoglu, Dilek; Uckan, Duygu

    2014-05-01

    Myasthenia gravis is among the rare complications after allogeneic hematopoietic stem cell transplantation and is usually associated with chronic GVHD. Herein, we report a 2-year and 10 months of age female with Griscelli syndrome, who developed severe myasthenia gravis at post-transplant +22nd month and required respiratory support with mechanical ventilation. She was unresponsive to cyclosporine A, methylprednisolone, intravenous immunoglobulin, and mycophenolate mofetil and the symptoms could only be controlled after plasma exchange and subsequent use of rituximab, in addition to cyclosporine A and mycophenolate mofetil maintenance. She is currently asymptomatic on the 6th month of follow-up.

  18. Two simultaneous autoimmune processes in a patient presenting with respiratory insufficiency.

    Science.gov (United States)

    Troy, Lauren; Hamor, Paul; Bleasel, Jane; Corte, Tamera

    2014-03-01

    The idiopathic inflammatory myopathies, including dermatomyositis, are uncommon acquired autoimmune diseases, sometimes associated with interstitial lung disease. Myasthenia gravis, a separate autoimmune disorder involving the neuromuscular junction, has some overlapping clinical features but has only rarely been reported to occur simultaneously within the same patient. Here we present the first reported case of concomitant dermatomyositis, myasthenia gravis, and interstitial lung disease.

  19. Juvenile myasthenia

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    Knežević-Pogančev Marija

    2011-01-01

    Full Text Available 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.

  20. Paraneoplastic myasthenia gravis and polymyositis secondary to a thymoma in a young woman

    DEFF Research Database (Denmark)

    Ezzatian-Ahar, Shabnam; Pedersen, Emil Arnspang; Schrøder, Henrik Daa

    2016-01-01

    We present the case of a 33-year-old woman who within weeks developed severe swallowing difficulties and weakness in her limbs to an extent requiring hospitalization. Workup confirmed clinically suspected diagnoses of polymyositis and autoimmune myasthenia. A suspicion of malignant thymoma based...

  1. Anti-musk positive myasthenia gravis and three semiological cardinal signs

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    André P.C. Matta

    2017-01-01

    Full Text Available Myasthenia gravis (MG is a relatively uncommon disorder with an annual incidence of approximately 7 to 9 new cases per million. The prevalence is about 70 to 165 per million. The prevalence of the disease has been increasing over the past five decades. This is thought to be due to better recognition of the condition, aging of the population, and the longer life span of affected patients. MG causes weakness, predominantly in bulbar, facial, and extra-ocular muscles, often fluctuating over minutes to weeks, in the absence of wasting, sensory loss, or reflex changes. The picture of fluctuating, asymmetric external ophthalmoplegia with ptosis and weak eye closure is virtually diagnostic of myasthenia. We report an atypical MG case with three semiological cardinal signs.

  2. The Myotonic Plot Thickens: Electrical Myotonia in Antimuscle-Specific Kinase Myasthenia Gravis

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    Marcus Magnussen

    2015-01-01

    Full Text Available Electrical myotonia is known to occur in a number of inherited and acquired disorders including myotonic dystrophies, channelopathies, and metabolic, toxic, and inflammatory myopathies. Yet, electrical myotonia in myasthenia gravis associated with antibodies against muscle-specific tyrosine kinase (MuSK has not been previously reported. We describe two such patients, both of whom had a typical presentation of proximal muscle weakness with respiratory failure in the context of a significant electrodecrement in repetitive nerve stimulation. In both cases, concentric needle examination revealed electrical myotonia combined with myopathic motor unit morphology and early recruitment. These findings suggest that MuSK myasthenia should be included within the differential diagnosis of disorders with electrical myotonia.

  3. Diurnal and day-to-day variation of isometric muscle strength in myasthenia gravis

    DEFF Research Database (Denmark)

    Vinge, Lotte; Jakobsen, Johannes; Pedersen, Asger Roer;

    2016-01-01

    INTRODUCTION: In patients with myasthenia gravis (MG), muscle strength is expected to decrease gradually during the day due to physical activities. METHODS: Isometric muscle strength at the shoulder, knee, and ankle was determined in 10 MG patients (MGFA class II-IV) who were receiving usual...... strength was higher in patients compared with controls. CONCLUSIONS: Patients with mild to moderate MG did not have increased variation of isometric muscle strength during the day or from day-to-day compared with controls. This suggests that isometric muscle performance can be determined with high...

  4. Thymus and Myasthenia Gravis: what can we learn from DNA microarrays?

    Science.gov (United States)

    Cizeron-Clairac, Géraldine; Le Panse, Rozen; Frenkian-Cuvelier, Mélinée; Meraouna, Amel; Truffault, Frédérique; Bismuth, Jacky; Mussot, Sacha; Kerlero de Rosbo, Nicole; Berrih-Aknin, Sonia

    2008-09-15

    This review is dedicated to John Newsom-Davis, who was an exceptional colleague and friend, always exchanging ideas with respect and consideration. We shall not forget his involvement and passion in search for the truth on the role of thymectomy in the management of Myasthenia Gravis (MG). In this short review, we shall summarize what we learnt from DNA microarrays applied to MG thymus. We shall focus on three main comparisons of the thymic transcriptomes: 1) highly hyperplastic MG patients versus non-MG adults; 2) corticosteroid-treated versus untreated seropositive MG patients; and 3) seronegative versus seropositive MG patients.

  5. Management of Stable Angina with Ivabradine as Safe Alternative to Patients with Myasthenia Gravis

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    Giuliano Ohde Dalledone

    2016-01-01

    Full Text Available Management of cardiac symptoms in myasthenia gravis (MG patients can be challenging. The aim of this report is to describe the safe use of ivabradine for stable angina in MG patients. A 48 y.o. woman, with MG diagnosis, presented stable angina. Therapies choices were reduced considering concomitant disease as well as previous and unsuccessful cardiologic managements. Ivabradine showed unexpected results. The patient presented an improvement of neurological and cardiac symptoms, bringing ivabradine as one more therapeutic option to similar patients. In this report we recommend ivabradine as an effective and safe drug for treatment of stable angina in MG patients.

  6. Major motor-functional determinants associated with poor self-reported health-related quality of life in myasthenia gravis patients.

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    Cioncoloni, David; Casali, Stefania; Ginanneschi, Federica; Carone, Marisa; Veronica, Boni; Rossi, Alessandro; Giannini, Fabio

    2016-05-01

    Myasthenia gravis (MG) is an autoimmune neuromuscular disorder in which disabling muscle weakness may affect health-related quality of life (HRQoL). The aim of this study was to investigate which common motor-functional deficits and corresponding severity are most determinant of poor HRQoL in these patients. In 41 patients, the dichotomized first item of the Italian Myasthenia Gravis Questionnaire (IMGQ), categorizing patients who report "good" and "poor" HRQoL, was chosen as dependent-outcome variable. All items composing the myasthenia gravis-specific scale (MG-ADL), i.e. talking, chewing, swallowing, breathing, impairment of ability to brush teeth or comb hair, impairment of ability to rise from chair, double vision, and eyelid droop were acquired as independent variables and dichotomized. Stepwise backward LR multivariable logistic regression analysis was performed. In addition, the main characteristics of patients were compared. MG-ADL items "chewing" ≥1, i.e. "fatigue chewing solid food", and "breathing" ≥2, i.e. "shortness of breath at rest" proved to be significant determinants. Higher dose of corticosteroid therapy was significantly (p = 0.027; r s  = -0.35), correlated with poor HRQoL. At diagnosis, a decremental response to repetitive nerve stimulation (RNS) from the abductor pollicis brevis was significantly more frequent in patients with poor HRQoL. In conclusion, impaired "chewing" and "breathing" functions indicate the need for careful planning of rehabilitation, re-education and patient management. Moreover, decremental response to RNS at diagnosis may identify patients at risk for poor HRQoL.

  7. Transcervical excision of thymoma and video-assisted thoracoscopic extended thymectomy (VATET) for ectopic cervical thymoma with myasthenia gravis: report of a case.

    Science.gov (United States)

    Kumazawa, Sachiko; Ishibashi, Hironori; Takahashi, Ken; Okubo, Kenichi

    2016-12-01

    Myasthenia gravis is the most common disease associated with thymoma, but it is rarely accompanied by ectopic thymoma. We describe a 47-year-old woman who presented with an ectopic cervical thymoma with myasthenia gravis. She was admitted to our neurology department with ptosis, diplopia, and mandibular muscle fatigue, and was diagnosed with myasthenia gravis. The mass was located posterior to the right lobe of thyroid gland on computed tomography and was diagnosed as ectopic thymoma on fine-needle aspiration biopsy examination. Transcervical excision of thymoma and VATET were performed. The patient has been free of neurological symptoms and has displayed no evidence of recurrent thymoma for 2 years.

  8. Myasthenia Gravis, Lambert-Eaton Myasthenic Syndrome & Congenital Myasthenic Syndromes

    Science.gov (United States)

    ... muscle cells that’s required for has improved dramatically. muscle contraction. MG is an autoimmune disease — a disease that ... an inward flux of electrical current that triggers muscle contraction. These contractions enable someone to move a hand, ...

  9. Leflunomide treatment in corticosteroid-dependent myasthenia gravis: an open-label pilot study.

    Science.gov (United States)

    Chen, Pei; Feng, Huiyu; Deng, Juan; Luo, Yufei; Qiu, Li; Ou, Changyi; Liu, Weibin

    2016-01-01

    Leflunomide is an effective drug used in the treatment of rheumatoid arthritis. Here we report the findings of an open-label pilot study, which found that leflunomide is also an effective treatment for myasthenia gravis (MG). This study recruited 15 corticosteroid-dependent MG patients. For 6 months, leflunomide 20 mg was given to these patients daily along with prednisone. The quantitative myasthenia gravis (QMG) scores and MG activities of daily living (MG-ADL) profiles were measured in these MG patients. After 6 months of treatment, 9 of the 15 patients enrolled in this study showed improvements in both QMG and MG-ADL. The mean QMG scores (13.4 to 8.5) and MG-ADL profiles (5.8 to 2.8) were significantly decreased (P = 0.01, 0.006 respectively). Furthermore, we found that the mean corticosteroid doses were reduced after treatment with leflunomide (24.3 to 12.3 mg per day). Leflunomide is a well-tolerated and efficacious treatment for corticosteroid-dependent MG, which may also enable lower doses of corticosteroids to be administered.

  10. Clinical findings in MuSK-antibody positive myasthenia gravis: a U.S. experience.

    Science.gov (United States)

    Pasnoor, Mamatha; Wolfe, Gil I; Nations, Sharon; Trivedi, Jaya; Barohn, Richard J; Herbelin, Laura; McVey, April; Dimachkie, Mazen; Kissel, John; Walsh, Ronan; Amato, Anthony; Mozaffar, Tahseen; Hungs, Marcel; Chui, Luis; Goldstein, Jonathan; Novella, Steven; Burns, Ted; Phillips, Lawrence; Claussen, Gwendolyn; Young, Angela; Bertorini, Tulio; Oh, Shin

    2010-03-01

    We performed a retrospective chart review on 53 muscle-specific kinase antibody (MuSK-Ab)-positive myasthenia gravis (MG) patients at nine university-based centers in the U.S. Of these, 66% were Caucasian, 85% were women, and age of onset was 9-79 years. Twenty-seven patients were nonresponsive to anticholinesterase therapy. Myasthenia Gravis Foundation of America improvement status was achieved in 53% patients on corticosteroids, 51% with plasma exchange, and in 20% on intravenous immunoglobulin (IVIG). Thymectomy was beneficial in 7/18 patients at 3 years. Long-term (> or =3 years) outcome was very favorable in 58% of patients who achieved remission and/or minimal manifestation status. Overall, 73% improved. There was one MG-related death. This survey reinforces several cardinal features of MuSK-Ab-positive MG, including prominent bulbar involvement and anticholinesterase nonresponsiveness. Facial or tongue atrophy was rare. Most patients respond favorably to immunotherapy. The best clinical response was to corticosteroids and plasma exchange, and the poorest response was to IVIG. Long-term outcome is favorable in about 60% of cases.

  11. Oral and parenteral pyridostigmine in preparing Myasthenia Gravis patients for thymectomy;a randomized Clinical trial

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    Tadjeddein A

    2007-06-01

    Full Text Available Background: Respiratory failure and crisis is one of major complications of thymectomy in myasthenia gravis patients. There are different medication regimes for preparing these patients for surgery and reducing post-operative side effects. The goal of this study is to compare respiratory complications of oral vs. Parenteral preoperative administration of anticholinesterase agents for thymectomy in myasthenia gravis patients. Methods: This randomized controlled trial included 101 patients in class IIA or IIB of myasthenia gravis according to the Osserman classification system. The control group fasted for eight hours before surgery and oral anticholines-terase agents were replaced with parenteral ones. The case group also fasted for 8 hours before surgery, but pyridostigmine was continued at its usual dose until the time of operation and the last dose was given to patients with a small amount of water in the operating room on the operating bed. Results: There was no statistically meaningful difference between the two groups in terms of age, sex and pathologic findings. In comparison, the mean hospital stay for the case group was 3.98 days and 6.34 for the control group (p value = 0.003. There were eight cases of respiratory crisis or failure (16% in the control group but only 1 case (2% was observed in case group (p value = 0.014. Only one patient in the case group required re-intubation after the surgery; however, six patients in control group were re-intubated (p value = 0.053. Plasmapheresis was required for five patients in the control group and one patient in the case group (p value = 0.098. Tracheostomy was performed on two patients in the control group to accommodate prolonged intubation, but none of the case group required this procedure. Conclusion: This study shows that continuing oral anticholinesterase agents up to the time of operation, with the last dose at the operative theater, lowers the incidence of post-operative myasthenia

  12. Myasthenia gravis in Ceará, Brazil: clinical and epidemiological aspects

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    Aline de Almeida Xavier Aguiar

    2010-12-01

    Full Text Available A retrospective chart review was performed on patients diagnosed as having myasthenia gravis in Ceará State, Brazil and who were followed from October 1981 to June 2009. Clinical and epidemiologic aspects were evaluated. In this work, 122 patients were studied, of whom 85 (69.7% were females and 37 (30.3% were males. The disease duration ranged from five months to 50 years (8.9±8.1 years. Age at the first symptoms varied from 0 to 74 years (31.9±14.4 years. The first main symptoms and signs were ptosis, diplopia and limb weakness. Generalized myasthenia was the most common clinical presentation, but 5.1% (n=6 persisted as ocular myasthenia. Thymectomy was performed in 42.6% (n=52 of myasthenic patients. A thymoma was present in 10 patients. Serum acetylcholine receptor (AChR antibodies were present in 80% (n=20 of specimens tested. The data presented are similar to those of studies performed in other countries.

  13. T-cell receptor V sub. alpha. and C sub. alpha. alleles associated with multiple sclerosis and myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Oksenberg, J.R.; Cavalli-Sforza, L.L.; Steinman, L. (Stanford Univ., CA (USA)); Sherritt, M.; Bernard, C.C. (LaTrobe Univ., Victoria (Australia)); Begovich, A.B.; Erlich, H.A. (Cetus Corporation, Emeryville, CA (USA))

    1989-02-01

    Polymorphic markers in genes encoding the {alpha} chain of the human T-cell receptor (TcR) have been detected by Southern blot analysis in Pss I digests. Polymorphic bands were observed at 6.3 and 2.0 kilobases (kb) with frequencies of 0.30 and 0.44, respectively, in the general population. Using the polymerase chain reaction (PCR) method, the authors amplified selected sequences derived from the full-length TcR {alpha} cDNA probe. These PcR products were used as specific probes to demonstrate that the 6.3-kb polymorphic fragment hybridizes to the variable (V)-region probe and the 2.0-kb fragment hybridizes to the constant (C)-region probe. Segregation of the polymorphic bands was analyzed in family studies. To look for associations between these markers and autoimmune diseases, the authors have studied the restriction fragment length polymorphism distribution of the Pss I markers in patients with multiple sclerosis, myasthenia gravis, and Graves disease. Significant differences in the frequency of the polymorphic V{sub {alpha}} and C{sub {alpha}} markers were identified between patients and healthy individuals.

  14. [Desflurane anesthesia without muscle relaxant for a patient with myasthenia gravis undergoing laparoscopic high anterior resection: a case report].

    Science.gov (United States)

    Koda, Kenichiro; Kimura, Haruka; Uzawa, Masashi; Sambe, Norie; Sugano, Takayuki; Kitamura, Takayuki; Tagami, Megumi

    2014-10-01

    Myasthenia gravis (MG) is an autoimmune disease affecting neuromuscular junction, which is characterized by fluctuating muscle weakness and abnormal fatigability. The use of muscle relaxants is major concern in anesthetic management for patients with MG. Muscle relaxant is a practical tool to assure immobilization during surgery under general anesthesia Anesthetic management without muscle relaxants for patients with MG is challenging, because it is difficult to assure immobilization. However, pharmacological effects of muscle relaxants can be prolonged in patients with MG, resulting in the increased incidence of postoperative respiratory support. We, here, describe an anesthetic management of an 82-year-old man with MG undergoing laparoscopic surgery. Anesthesia was induced with propofol and remifentanil Desflurane was administered via a face mask, and the patient was manually ventilated for 10 min, and the trachea was intubated safely without muscle relaxants. Anesthesia was maintained with desflurane and remifentanil. We did not administer muscle relaxants to the patient during surgery. Throughout laparoscopic procedures, no movements of the patient were observed, and there were no problems concerning the laparoscopic view of the operation filed. The surgery was uneventful. The patient emerged from anesthesia smoothly, and was extubated safely. The postoperative course of the patient was also uneventful.

  15. Video-assisted thoracoscopic surgery or transsternal thymectomy in the treatment of myasthenia gravis?

    Science.gov (United States)

    Zahid, Imran; Sharif, Sumera; Routledge, Tom; Scarci, Marco

    2011-01-01

    A best evidence topic in thoracic surgery was written according to a structured protocol. The question addressed was how video-assisted thoracoscopic surgery (VATS) compares to median sternotomy in the surgical management of patients with myasthenia gravis (MG)? Overall 74 papers were found using the reported search, of which 15 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results are tabulated. We conclude that VATS produces equivalent postoperative mortality and complete stable remission (CSR) rates, with superior results in terms of hospital stay, operative blood loss and patient satisfaction at the expense of a doubling of operative time. Six studies comparing VATS and transsternal sternotomy in non-thymomatous myasthenia gravis (NTMG) patients found VATS to have lower operative blood loss (73.8±70.7 vs. 155.3±91.7 ml; P0.05). One study comparing video-assisted thoracoscopic extended thymectomy to transsternal thymectomy in only thymoma-associated myasthenia gravis (T-MG) patients found equivalent CSR (11.3 vs. 8.7%, P=0.1090) at six-year follow-up. Thymoma recurrence rate (9.64%) was not significantly different (P=0.1523) between the two groups. Eight studies comparing VATS and transsternal approach in mixed T-MG and NTMG patients found a lower hospital stay (1.9±2.6 vs. 4.6±4.2 days, P<0.001), reduced need for postoperative medication (76.5 vs. 35.7%, P=0.022), lower intensive care unit stay (1.5 vs. 3.2 days, P=0.018), greater symptom improvement (100 vs. 77.9%, P=0.019) and better cosmetic satisfaction (100 vs. 83, P=0.042) with VATS. In concordance with NTMG and T-MG alone patient groups, VATS and transsternal methods had equivalent complication rates (23 vs. 19%, P=0.765) with no mortalities in either group. Even though VATS has a longer operative time (268±51 vs. 177±92 min, P<0.05), its improved cosmesis, reduced need

  16. Anestesia ambulatorial para radioterapia em paciente portador de miastenia gravis: relato de caso Anestesia ambulatorial para radioterapia en paciente portador de miastenia gravis: relato de caso Outpatient anesthesia for radiotherapy in a patient with myasthenia gravis: case report

    Directory of Open Access Journals (Sweden)

    Raquel Marcondes Bussolotti

    2006-08-01

    , 87 años, 87 kg, estado físico ASA III, con historial de miastenia gravis; accidente vascular encefálico previo (AVE; marcapaso por bloqueo atrio-ventricular total; hipertensión arterial sistémica (HAS. Fueron programadas siete sesiones de radioterapia de 20 minutos para tratamiento del tumor de parótida bajo anestesia general. En la sala de radioterapia fue monitorizado con cardioscopio, monitor de presión arterial no invasiva, oxímetro de pulso y sometido a anestesia general con propofol y sevoflurano. Después de la inducción, se mantuvo en ventilación espontánea con cánula de Guedel y catéter nasofaríngeo con O2 /sevoflurano, para acomodación de la máscara de inmovilización. En la sala de recuperación postanestésica, no presentó complicaciones. CONCLUSIONES: La elección de los anestésicos y el acompañamiento clínico de criterio permitieron la buena evolución del paciente, con AVE previo, cardiopata y anciano, sometido a la anestesia general balanceada para procedimiento de aplicación de radioterapia, en régimen ambulatorial.BACKGROUND AND OBJECTIVES: Myasthenia Gravis (MG is a rare autoimmune disease, characterized by the reduction in the number of nicotinic receptors in the neuromuscular junction, with an incidence of 14/100,000. The objective of this report is to describe the case of a patient with myasthenia gravis who underwent balanced general anesthesia for radiotherapy of a spinocellular carcinoma of the parotid gland as an outpatient. CASE REPORT: Male patient, 87 years old, 87 kg, physical status ASA III, with a prior history of myasthenia gravis; stroke; pacemaker for a third-degree AV block; and hypertension. He was scheduled for seven 20-minutes radiotherapy sessions under general anesthesia. In the radiotherapy room he was monitored with a cardioscope, noninvasive blood pressure, pulse oxymeter, and underwent general anesthesia with propofol and sevoflurane. After the induction, he maintained spontaneous ventilation with a

  17. Myasthenia gravis and myasthenic syndrome: simulation of twitch strength with or without therapy.

    Science.gov (United States)

    Nigrovic, Vladimir; Amann, Anton; Bhatt, Shashi

    2005-12-01

    To examine the quantitative relationship between indirectly evoked twitch and decreases in the number of either postsynaptic receptors or acetylcholine molecules released by a single stimulus, we studied these variables in a computer-simulated model of neuromuscular transmission. Twitch strength decreased if the number of receptors decreased to below 30% of normal or the number of acetylcholine molecules released by a stimulus decreased to below 80%. Inhibition of acetylcholine hydrolysis to 50% restored twitch strength in the presence of a decreased number of receptors. However, twitch strength was more easily restored to normalcy by augmenting the release of acetylcholine, if the release was diminished by disease. The simulations mimic the clinically known therapeutic outcomes in certain disorders of neuromuscular transmission. These results provide useful quantitative insights into the relationship between acetylcholine receptors or the stimulus-induced release of acetylcholine and muscle function in myasthenia gravis or Lambert-Eaton myasthenic syndrome.

  18. VAV1 and BAFF, via NFκB pathway, are genetic risk factors for myasthenia gravis

    DEFF Research Database (Denmark)

    Avidan, Nili; Le Panse, Rozen; Harbo, Hanne F;

    2014-01-01

    OBJECTIVE: To identify novel genetic loci that predispose to early-onset myasthenia gravis (EOMG) applying a two-stage association study, exploration, and replication strategy. METHODS: Thirty-four loci and one confirmation loci, human leukocyte antigen (HLA)-DRA, were selected as candidate genes...... confirmation loci were genotyped in 1177 EOMG patients and 814 controls, from nine European centres. RESULTS: ALLELE FREQUENCY DIFFERENCES WERE FOUND IN FOUR NOVEL LOCI: CD86, AKAP12, VAV1, B-cell activating factor (BAFF), and tumor necrosis factor-alpha (TNF-α), and these differences were consistent in all...... nine cohorts. Haplotype trend test supported the differences in allele frequencies between cases and controls. In addition, allele frequency difference in female versus male patients at HLA-DRA and TNF-α loci were observed. INTERPRETATION: The genetic associations to EOMG outside the HLA complex...

  19. Miastenia grave: aspectos históricos Myasthenia gravis: historical aspects

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    FRANCISCO MARCOS B. CUNHA

    1999-06-01

    Full Text Available Foram pesquisados aspectos históricos da miastenia grave desde as primeiras descrições da doença em 1672, pelo clínico inglês Thomas Willis. São descritas as dificuldades encontradas no manuseio dos primeiros pacientes diagnosticados. Pesquisaram-se fatos históricos ligados à investigação da doença, o tratamento, bem como curiosidades pouco citadas na literatura.We studied historical aspects of myasthenia gravis starting from its first description by the English physician, Sir Thomas Willis, in 1672. We also describe the difficulties in managing triating the first diagnosed patient. Historical facts related to the investigation and the initial treatment of this disorder as well as curiosities seldom mentioned in the literature are part of this paper.

  20. Haptoglobin study in myasthenia gravis Estudo sobre a haptoglobina na miastenia grave

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    Leonardo H. Mendonça Oliveira

    2008-06-01

    Full Text Available OBJECTIVE: A cross-sectional study of haptoglobin (Hp in myasthenia gravis (MG was designed, with the objective to identify its values and correlate them with different disease status. METHOD: 46 patients were enrolled in the study, all having disease severity established according to the quantitative myasthenia gravis strength scores (QMGSS. Based on the functional scale determined by Myasthenia Gravis Foundation of America (MGFA recommendations, patients were classified as having: complete stable remission (CSR; n=10; minimal manifestations-0 (MM0; n=6, minimal manifestations-1 (MM1; n=4; pharmacological remission (PR; n=6. Two other groups participated: thymomatous patients (T; n=10 and patients without imunosuppression or thymectomy, until the assessment for Hp (WIT; n=10. Hp dosage was done by immunonephelometry, blindly to clinical data. Student's t-test, Anova test and linear regression were employed for statistical analyses. RESULTS: Statistically significant differences occurred between CSR+MM0xWIT groups (86.62x157.57, pOBJECTIVO: Desenhou-se estudo transversal sobre a haptoglobina (Hp na miastenia grave (MG com o objetivo de identificar seus valores e correlacioná-los a diferentes condições na doença. MÉTODO: 46 pacientes foram incluídos, todos tendo a gravidade da doença estabelecida segundo escores internacionais (QMGSS. Os pacientes tiveram seu estado funcional determinado de acordo com a Myasthenia Gravis Foundation of América (MGFA e classificados em: remissão completa estável (CSR; n=10; mínima manifestação-0 (MM0; n=6, mínima manifestação-1 (MM1; n=4; remissão farmacológica (PR; n=6. Dois outros grupos participaram: pacientes timomatosos (T; n=10 e pacientes sem imunossupressão ou timectomia, até o momento da inclusão no estudo (WIT; n=10. A dosagem de Hp foi realizada por imunonefelometria, de modo cego quanto à clínica. As análises estatísticas incluíram o teste de Student, Anova e regressão linear

  1. Failure of treatment of myasthenia gravis by cyclosporin-A: a case report

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    Paulo E. Marchiori

    1989-03-01

    Full Text Available Recently, cyclosporin-A (Cy-A has been used in the treatment oy myasthenia gravis (MG. This drug could be employed in some patients refractory to classic treatments or that develop undesirable side effects. It is reported the case of a 22 year-old woman with generalized and severe MG, and diabetes mellitus. She had been submitted to thymectomy and reoperated, to the classic ethiopathogenic methods of therapy, and to total body irradiation. No therapeutical results were observed. Also, she developed transient and slow bone marrow depression, and liver dysfunction. Owing to these limitations and to the absence of response to treatments mentioned, Cy-A use was attempted in this case. Unfortunately, Cy-A did not influence the myasthenic symptomatology. Cy-A also failed in suppressing anti-AChR production, which increased during Cy-A therapy. Results observed in this case are in disagreement with literature data on the subject.

  2. Elevated plasma interleukin-17A in a subgroup of Myasthenia Gravis patients.

    Science.gov (United States)

    Xie, Yanchen; Li, Hai-feng; Jiang, Bin; Li, Yao; Kaminski, Henry J; Kusner, Linda L

    2016-02-01

    To better define the role of IL-17A in myasthenia gravis (MG), we assessed plasma concentrations in 69 adult patients with MG prior to initiation of immunosuppression and monitored their clinical course for the subsequent 2years with quantitative MG scores (QMGS) and Osserman classification. IL-17A was higher among patients than healthy control subjects. Early-onset women without thymoma had greater elevations of IL-17A. Logistic regression analysis indicated that the absence of thymoma rather than women gender or early-onset was the significant determinant associated with IL-17A elevation. Elevated IL-17A levels were associated with more severe MG. In summary, IL-17A has role in the pathogenesis of a subgroup of patients with early-onset women with MG with greater disease severity who are most likely to have thymic hyperplasia. This subgroup may be a target for IL-17 treatments, which are under development.

  3. Computed tomography of the thymus after pneumomediastinography in patients with myasthenia gravis

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    Hirose, Genjiro; Yamamoto, Teiji; Kosoegawa, Hiroshi; Saeki, Mitsuo; Hayashi, Toshiharu (Kanazawa Medical Univ., Ishikawa (Japan))

    1982-09-01

    Nine patients with myasthenia gravis (MG) had plain chest X-ray, tomography, computed tomography (CT) of the thorax, pneumomediastinography (PMG) and post-PMG CT scans. Routine chest radiography detected mediastinal masses in two patients and conventional laminogram provided no additional information. CT yielded anterior mediastinal increased densities in seven patients (78%), whereas post-PMG CT delineated masses (thymoma or thymic hyperplasia) or thymic gland in all cases (100%), but these procedures could not distinguish thymic tumor from nontumorous conditions. PMG and post-PMG CT scans are superior to other radiographic techniques in detecting mediastinal abnormalities with more clear delineation of thymoma or thymic gland. When an abnormal density is present in the mediastinum of a patient with MG, PMG and post-PMG CT scans should be done to outline the mediastinal anatomy, to determine the size, extent, invasiveness of thymoma or thymic gland prior to thymectomy.

  4. The Clinical Outcome and Therapeutic Treatment of a Patient with Double Seronegative Myasthenia Gravis

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    Azanjac Ana

    2014-03-01

    Full Text Available Prikazali smo slučaj 22-godišnjeg muskarca sa kliničkom slikom otežanog gutanja i dvoslika. Bolest myasthenia gravis (MG je dijagnostikovana na osnovu pozitivnog faramakološkog testa, pozitivnog testa neuromišićne transmisije, kompjuterizovane tomografije medijastinuma. Antitela protiv acetilholinskog receptora (AChR i antitela protiv mišićno specifičnog receptora za tirozin kinazu (MuSK. bila su negativna. Zabeleženo je delimično poboljšanje u neurološkom nalazu na ordiniranu terapiju Piridostigminom, Ciklosporinom, kortikosteroidima. Bolesnik je imao povoljan odgovor na izmenu plazme sa povlačenjem svih kliničkih siimptoma što potvrdjuje hipotezu o učešću humoralnih faktora u patogenezi dvostruko negativne MG.

  5. Longitudinal epitope mapping in MuSK myasthenia gravis: implications for disease severity.

    Science.gov (United States)

    Huijbers, Maartje G; Vink, Anna-Fleur D; Niks, Erik H; Westhuis, Ruben H; van Zwet, Erik W; de Meel, Robert H; Rojas-García, Ricardo; Díaz-Manera, Jordi; Kuks, Jan B; Klooster, Rinse; Straasheijm, Kirsten; Evoli, Amelia; Illa, Isabel; van der Maarel, Silvère M; Verschuuren, Jan J

    2016-02-15

    Muscle weakness in MuSK myasthenia gravis (MG) is caused predominantly by IgG4 antibodies which block MuSK signalling and destabilize neuromuscular junctions. We determined whether the binding pattern of MuSK IgG4 antibodies change throughout the disease course ("epitope spreading"), and affect disease severity or treatment responsiveness. We mapped the MuSK epitopes of 255 longitudinal serum samples of 53 unique MuSK MG patients from three independent cohorts with ELISA. Antibodies against the MuSK Iglike-1 domain determine disease severity. Epitope spreading outside this domain did not contribute to disease severity nor to pyridostigmine responsiveness. This provides a rationale for epitope specific treatment strategies.

  6. Disturbed B cell subpopulations and increased plasma cells in myasthenia gravis patients.

    Science.gov (United States)

    Kohler, Siegfried; Keil, Thomas Oskar Philipp; Swierzy, Marc; Hoffmann, Sarah; Schaffert, Hanne; Ismail, Mahmoud; Rückert, Jens Carsten; Alexander, Tobias; Hiepe, Falk; Gross, Christian; Thiel, Andreas; Meisel, Andreas

    2013-11-15

    Whether there is a general perturbation of B and plasma cell subsets in myasthenia gravis (MG) has not been investigated so far. Here we performed a detailed flow cytometric analysis of blood and if available thymic tissue in order to detect MG-specific and therapy-induced changes. We observed significant differences in the distribution of B cell subsets in MG patients, yet these were mainly attributable to medical treatment. Furthermore MG is associated with significantly increased frequencies of plasma cells that were especially activated in purely ocular disease manifestation. In contrast to thymoma, B cell subset distribution in hyperplastic thymus could be distinguished from peripheral blood, however both tissues were not significantly enriched with plasma cells. Thus B cell differentiation in general is not defective in MG, but modified by therapy and enhanced frequencies of plasma cells can be detected in MG patients.

  7. Frequency of myasthenia gravis in multiple sclerosis: Report of five cases from Isfahan, Iran

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    Basiri Keivan

    2009-12-01

    Full Text Available This study was designed to determine the frequency and clinical characteristics of myasthenia gravis (MG in a large cohort of Persian patients with multiple sclerosis (MS living in the province of Isfahan. We reviewed the case records of patients with definite MS (McDonald′s criteria registered in the Isfahan MS Society (IMSS for associated MG. Of the 1,718 patients with MS in the registry, six patients were found to have both MS and MG. The prevalence after excluding one patient with insufficient data, was 291 per 100,000 (0.29%, a higher prevalence than the earlier reports. These results may support the hypothesis that MS and MG share some common immunopathogenic mechanisms.

  8. Patients with ocular symptoms referred for electrodiagnosis: how many of them suffer from myasthenia gravis?

    Science.gov (United States)

    Zambelis, Th; Pappas, V; Kokotis, P; Zouvelou, V; Karandreas, N

    2015-12-01

    The aim of this study was the diagnosis of patients with isolated ocular manifestations (ptosis and/or diplopia) referred for electrophysiological evaluation to the electrodiagnostic laboratory of a University Neurological Department. Examination was performed either in inpatient status or in outpatient basis. We analyzed the clinical, electrophysiological and other laboratory data in 79 subjects. Myasthenia gravis (MG) was diagnosed in 38 %, 45.6 % in other diseases (Graves disease, blepharospasm, IIId cranial verve palsy, multiple sclerosis, stroke, etc.), while in 16.5 %, the cause remained unidentified. Symptoms fluctuation was significantly more frequent in the myasthenic patients, compared to patients with other diseases. The presence of both diplopia and ptosis are more likely due to MG rather than other pathology.

  9. Miastenia gravis análise de 90 casos tratados com timectomia myasthenia gravis

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    Almeida Fábio Henrique Souza

    2000-01-01

    Full Text Available A Miastenia Gravis é uma doença auto-imune caracterizada por auto-anticorpos contra receptores nicotínicos de acetilcolina da placa neural. O tratamento clínico básico para as formas generalizadas é feito com drogas anticolenesterásicas, mas em algumas fases podem ser necessários outras modalidades de tratamento, tais como a corticoterapia e a plasmaferese. O tratamento cirúrgico, a timectomia, é uma forma de abordar a causa base da doença e tem resultados bastante encorajadores, visto que é atribuída ao timo a produção dos auto-anticorpos. Apresentamos neste trabalho o quadro clínico, os resultados cirúrgicos e as alteração anátomo-patológicas dos 90 casos estudados.

  10. Aberrant decrease of microRNA19b regulates TSLP expression and contributes to Th17 cells development in myasthenia gravis related thymomas.

    Science.gov (United States)

    Wang, Zhongkui; Chen, Yuping; Xu, Shengjie; Yang, Yanhua; Wei, Dongning; Wang, Wei; Huang, Xusheng

    2015-11-15

    Myasthenia gravis (MG) is an organ-specific autoimmune disease. The imbalance of T helper type 17 cells (Th17) plays a key role in the pathogenesis of thymomatous MG. But the regulatory mechanism for Th17 cell development in MG-related thymoma remains undefined. Here we demonstrated that thymic stromal lymphopoietin (TSLP) is significantly decreased in thymomas. We also proved that TSLP was post-trancriptionally regulated by microRNA-19b. The expression of microRNA-19b was negatively correlated with the expression of TSLP mRNA and protein in thymomas. This study indicated that the elevation of microRNA-19b suppressed TSLP expression and then influenced T cell development in thymomatous MG.

  11. Acute Respiratory Failure Induced by Magnesium Replacement in a 62-Year-Old Woman with Myasthenia Gravis.

    Science.gov (United States)

    Singh, Paramveer; Idowu, Olakunle; Malik, Imrana; Nates, Joseph L

    2015-10-01

    Magnesium is known to act at the neuromuscular junction by inhibiting the presynaptic release of acetylcholine and desensitizing the postsynaptic membrane. Because of these effects, magnesium has been postulated to potentiate neuromuscular weakness. We describe the case of a 62-year-old woman with myasthenia gravis and a metastatic thymoma who was admitted to our intensive care unit for management of a myasthenic crisis. The patient's neuromuscular weakness worsened in association with standard intravenous magnesium replacement, and the exacerbated respiratory failure necessitated intubation, mechanical ventilation, and an extended stay in the intensive care unit. The effect of magnesium replacement on myasthenia gravis patients has not been well documented, and we present this case to increase awareness and stimulate research. In addition, we discuss the relevant medical literature.

  12. "THE EFFECTS OF ANODAL IONTOPHORESIS OF EPINEPHRINE ON NEUROMUSCULAR RESPONSES IN HEALTHY MEN AND PATIENTS WITH MYASTHENIA GRAVIS "

    OpenAIRE

    Talebian, S.; Abolfazli, R.; G. R. Olyaei S. Hajizadeh

    2005-01-01

    Iontophoresis of epinephrine for assessment of neuromuscular junction response is a new technique that can improve diagnose of neuromuscular dysfunction. The purpose of this study was to investigate the effects of iontophoresis of epinephrine on neuromuscular junction response. Iontophoresis of epinephrine solution (1mg/ml), sodium chloride, calcium gluconate, epinephrine with sodium chloride and distilled water was applied in five groups of healthy men and 7 patients with myasthenia gravis (...

  13. 重症肌无力临床治疗效果观察%Clinical Therapeutic Effect of Myasthenia Gravis

    Institute of Scientific and Technical Information of China (English)

    薛炬君; 陈曦

    2016-01-01

    Objective To analyze the clinical treatment of patients with myasthenia gravis through experimental analysis and observe the clinical effect of records. Methods Colected myasthenia gravis in 100 patients as the research object in our hospital,divided into study group and control group,compared treatment effect. The research group was treated with Chinese medicine treatment scheme of Yiqi Qushi. Patients in the control group were treated with neostigmine. After treatment, compared the two groups of patients with treatment effect.ResultsThe study group patients of effective rate of treatment was significantly higher than the control group patients.Conclusion Myasthenia gravis has more complex condition.In myasthenia gravis patients using YiqiQushi can improve patient's therapeutic effect.%目的:通过实验分析患有重症肌无力的患者有效的临床治疗方案,并观察记录临床效果。方法收集在我院进行治疗的重症肌无力患者100例作为研究对象,分成研究组和对照组,研究组患者实施实施益气祛湿的中药治疗方案,对照组患者使用新斯的明进行对比治疗,治疗完成后对比两组患者治疗效果。结果研究组患者治疗有效率高于对照组患者。结论重症肌无力的临床表现多变,病情较复杂。对重症肌无力的患者实施益气祛湿可以提高患者的治疗效果。

  14. Sevoflurane and thoracic epidural anesthesia for trans-sternal thymectomy in a child with juvenile myasthenia gravis

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    Valluvan Rangasamy

    2014-01-01

    Full Text Available Literature on anesthetic management of juvenile myasthenia gravis (JMG for thymectomy is limited. Recently, use of inhalational agents and total intravenous anesthesia with propofol and remifentanyl has been reported. All these techniques individually or in combination have been tried to avoid the use of muscle relaxant. We report successful use of sevoflurane as sole anesthetic agent for intubation and in combination with thoracic epidural anesthesia for intraoperative anesthetic management in a 5-year-old child with JMG.

  15. Doença de Castleman associada a sarcoma de células dendríticas foliculares e miastenia gravis Castleman's disease associated with follicular dendritic cell sarcoma and myasthenia gravis

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    Fernando Luiz Westphal

    2010-12-01

    Full Text Available A doença de Castleman é um distúrbio linfoproliferativo atípico, de etiologia desconhecida, que pode estar associada a uma série de condições clínicas, inclusive doenças de caráter autoimune e neoplasias malignas. No presente relato, uma paciente de 72 anos foi encaminhada ao serviço de cirurgia torácica do Hospital Universitário Getúlio Vargas, localizado na cidade de Manaus (AM para a ressecção de um tumor de mediastino posterior. Três meses antes, havia sido internada em UTI com um quadro de dispneia intensa, ocasião na qual foi diagnosticada miastenia gravis. Após a ressecção da massa mediastinal, a análise histopatológica revelou doença de Castleman hialino-vascular complicada por sarcoma de células dendríticas foliculares. Até o momento da redação deste estudo, a paciente utilizava um anticolinesterásico e corticoides para o controle da miastenia gravis.Castleman's disease is an atypical lymphoproliferative disorder of unknown etiology, which might be associated with various clinical conditions, including autoimmune diseases and malignant neoplasms. We report the case of a 72-year-old female patient who was referred to the thoracic surgery department of Getúlio Vargas University Hospital, in the city of Manaus, Brazil, for the resection of a posterior mediastinal tumor. Three months prior, the patient had been admitted to the ICU with signs of severe dyspnea, at which time she was diagnosed with myasthenia gravis. After the resection of the mediastinal tumor, the histopathological examination revealed hyaline vascular-type Castleman's disease, complicated by follicular dendritic cell sarcoma. At this writing, the patient was being treated with an anticholinesterase agent and corticosteroids for the control of myasthenia gravis.

  16. Th17细胞在重症肌无力发病机制中作用的研究进展%Progress in the role of Th17 cells in the pathogenesis of myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    李永超; 张临友; 刘春全

    2015-01-01

    新辅助T细胞Th17在分化和功能上与传统的新辅助T细胞Th1和Th2不同,IL-17是其主要的细胞因子.Th17细胞与炎症反应和自身免疫性疾病密切相关,研究Th17细胞的生物学特性、Th17细胞及其细胞因子在重症肌无力(MG)、实验性重症肌无力发病机制中的作用很有意义,通过对Th17细胞的深入研究,有助于认识MG的发病机制及进一步寻找新的治疗靶点.%The new T helper cell Th17 is different from the traditional T helper cells Th1 and Th2 in differentiation and function.IL-17 is the main cytokine of Th17 cells.Th17 cells is closely related to inflammation and autoimmune diseases.Here we review the biological characteristics of Th17 cells,Focusing on the pathogenesis of Th17 cells and cytokines in myasthenia gravis(MG) and experimental autoimmune myasthenia gravis(EAMG).Study on Th17 cells would contribute to understanding the pathogenesis of myasthenia gravis and searching for new therapeutic targets.

  17. The effects of prednisone and steroid-sparing agents on decay accelerating factor (CD55) expression: implications in myasthenia gravis.

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    Auret, Jennifer; Abrahams, Amaal; Prince, Sharon; Heckmann, Jeannine M

    2014-06-01

    Decay accelerating factor (DAF) expression at the muscle endplate is an important defence against complement-mediated damage in myasthenia gravis. Previously we implicated the c.-198C>G DAF polymorphism with the development of treatment-resistant myasthenia-associated ophthalmoplegia by showing that the C>G DAF polymorphism prevented lipopolysaccharide-induced upregulation of lymphoblast DAF. We postulated that drugs used in myasthenia gravis may increase the susceptibility of extraocular muscles to complement-mediated damage and studied their effects on endogenous DAF using patient-derived lymphoblasts as well as mouse myotubes. We show that prednisone repressed C>G DAF expression in lymphoblasts and increased their susceptibility to cytotoxicity. Methotrexate, but not azathioprine or cyclosporine, increased DAF in C>G lymphoblasts. In mouse myotubes expressing wild-type Daf, prednisone also repressed Daf expression. Although cyclosporine, azathioprine, and methotrexate increased muscle Daf levels when used alone, upon co-treatment with prednisone only azathioprine maintained myotube Daf levels close to basal. Therefore, prednisone negatively influences DAF expression in C>G lymphoblasts and in myotubes expressing wild-type Daf. We speculate that myasthenic individuals at risk of developing the ophthalmoplegic complication, such as those with C>G DAF, may have inadequate endogenous levels of complement regulatory protein protection in their extraocular muscle in response to prednisone, increasing their susceptibility to complement-mediated damage.

  18. Clinical manifestation and humoral immuno-function of myasthenia gravis patients with abnormal and normal thymus gland

    Institute of Scientific and Technical Information of China (English)

    Fuhua Peng; Yongqiang Dai; Wei Qiu; Xueqiang Hu

    2006-01-01

    BACKGROUND: Myasthenia gravis (MG) is an autoimmune disease which mainly affects neuromuscular junctions. The ages, modified Osserman classification and clinical manifestation and humoral immunol function of MG with and without thymic abnormality are different.OBJECTIVE: To explore the clinical manifestation and humoral immuno-function of MG with abnormal and normal thymus gland.DESIGN: Contrast observation.SETTING: Department of Neurology, the Third Affiliated Hospital of Sun Yat-sen University.PARTICIPANTS: A total of 49 inpatients with MG were selected from the Third Affiliated Hospital of Sun Yat-sen University from March 2000 to August 2005. All the patients had typical clinical manifestation of MG and positive neostigmine test. All the patients knew and agreed the laboratory examinations. There were 22 males and 27 females of 2-69 years old. Chest MRI or CT scan were performed to reveal thymus gland abnormality. According to whether there was tumor in superior mediastinum, all patients were divided into 2 groups, abnormal and normal groups. Normal thymus gland group (n=30) contained 16 males and 14 famales of 6-43 years old. Abnormal thymus gland group (n=19) contained 6 male and 13 female of 2-69years old.METHODS: ① All patients were questioned about initial symptoms. Meanwhile, main clinical manifestations were recorded at hospital admission. ② 7180A automatic biochemical analyzer and automatic microplate reader were used in detecting seroimmunity index. The levels of C3, C4, IgG, IgA, IgM and CH50 in blood serum were analyzed by nephelometry. ③ Clinical classification is based on modified Osserman classification. The patients with MG were divided into six types: Ⅰ (Ocular myasthenia), Ⅱ a (Mild generalized myasthenia), Ⅱ b (Moderately severe generalized myasthenia), Ⅲ (Acute fulminating myasthenia), Ⅳ (Late severe myasthenia).MAIN OUTCOME MEASURES: ① Differences of initial symptoms and clinical manifestation of two group patients.

  19. Effect of Gender, Disease Duration and Treatment on Muscle Strength in Myasthenia Gravis

    Science.gov (United States)

    Citirak, Gülsenay; Cejvanovic, Sanja; Andersen, Henning; Vissing, John

    2016-01-01

    Introduction The aim of this observational, cross-sectional study was to quantify the potential presence of muscle weakness among patients with generalized myasthenia gravis (gMG). The influence of gender, treatment intensity and disease duration on muscle strength and disease progression was also assessed. Methods Muscle strength was tested in 8 muscle groups by manual muscle testing and by hand-held dynamometry in 107 patients with gMG and 89 healthy age- and gender-matched controls. Disease duration, severity and treatment history were reviewed and compared with muscle strength. Results Patients had reduced strength in all tested muscle group compared to control subjects (p<0.05). Women with gMG were stronger than men (decrease in strength 22.6% vs. 32.7% in men, P<0.05). Regional differences in muscle weakness were also evident, with proximal muscles being more affected. Interestingly, muscle strength did not correlate with disease duration and treatment intensity. Conclusions The results of this study show that in patients with gMG; 1) there is significant muscle weakness, 2) muscle weakness is more pronounced in men than women, 3) shoulder abductors, hip flexors, and neck muscles are the most affected muscle groups and 4) disease duration or treatment intensity alone are not predictors of loss of muscle strength in gMG. PMID:27741232

  20. Diagnostico y tratamiento de la myasthenia gravis estudio de una poblacion hospitalaria

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    Roberto D. Rey

    1990-09-01

    Full Text Available Durante la observacion de 50 pacientes con Myasthenia gravis entre los anos 1974 y 1987 se encontro un predominio del sexo femenino en la proporcion de 2,5 a 1 y mayor frecuencia de presentacion en Ias 3ª y 5ª décadas de la vida. Luego de la evaluacion clínica, el diagnóstico fué confirmado mediante: (1 prueba dei edrofonio, (2 estímulo nervioso repetitivo, (3 dosaje de anticuerpos antirreceptor colinérgico y (4 transferencia pasiva del suero al raton con ulterior niedicion de la amplitud de mepp's. La positividad diagnostica vario entre el 90 y el 100%, segun el tipo de prueba empleada. El estúdio radiológico del timo fué hecho con neumomediastinografía. obteniendose excelente correlación con la descripcion histológica de la glândula, y con tomografia computada, que demostro menor eficiência diagnostica. El tratamiento fué implementado en base a anticolinesterásicos, timectomía y corticoterapia inmunosupresora, evidenciandose con esta última mejores resultados. Nueve pacientes desarrollaron peoria transitória del cuadro muscular al iniciarse el tratamiento esteroideo, 6 de ellos exhibieron posteriormente evolucion desfavorable. Esta observacion aparenta tener valor pronóstico en la evolucion de la MG.

  1. CT-guided percutaneous thymus microwave radiation and local injection of dexamethasone for myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    WANG Pei-jun; YANG Ji-jin; L(U) Tao-zhen; TU Lai-hui; ZUO Chang-jing; ZHANG Ren-qin; CHEN Wei; SHAO Cheng-wei; WANG Min-jie; LIU Dong-ming

    2001-01-01

    Objective: To develop an effective therapy for myasthenia gravis (MG) with little traumatic injury and low morCT-guided percutaneous thymus paracentesis via the upper margin of the sternum incisure and with local dexamethasone injection. Results: The symptoms and signs were relieved within 3 d of treatment in all the 13 MG patients; three had immediate response to the treatment with improved myodynamia. A 3-year follow-up study showed that the symptoms and signs improved significantly in 10 patients and turned for the better in 3. The size of the thymus reduced vertically, transversely and anteroposteriorly by 24.2%, 31.7% and 34.1% respectively. Low-density areas were seen in the thymus due to water gasification and coagulation necrosis of the thymus tissue. Conclusion: The technique has definite therapeutic effect on MG with advantages of easy manipulation, quick response and low morbidity. It is suitable for MG patients with hyperplasia or thymoma, and those who are unwilling to receive thymectomy, or whose thymoma is not resectable, or those who have poor response to radiotherapy.

  2. Intravenous flurbiprofen for post-thymectomy pain relief in patients with myasthenia gravis

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    Su Chunhua

    2012-09-01

    Full Text Available Abstract Background Post-thymectomy pain in myasthenia gravis (MG patients can inhibit breathing and coughing. Inappropriate usage of analgesics may exacerbate respiratory inhibition and even cause myasthenic crisis. Flurbiprofen is a non-steroidal anti-inflammatory drug (NSAID that is commonly used to control moderate postoperative pain and is not associated with respiratory inhibition. We hypothesized that flurbiprofen may provide post-thymectomy pain relief without increasing the risk of complications in MG patients. Methods Two hundred MG patients underwent extended thymectomy from March 2006 to December 2010 and were randomly allocated to a flurbiprofen group (110 patients, 50 mg intravenous flurbiprofen axetil or a control group (90 patients, 100 mg intramuscular tramadol as postoperative analgesia. Visual analog scale (VAS pain score, heart rate, blood pressure, respiratory rate, pulse oximetry (SpO2, and adverse effects were recorded before and up to 24 h after drug administration. Results There were no significant differences in the preoperative clinical characteristics of the flurbiprofen and control (tramadol groups. Both flurbiprofen and tramadol significantly alleviated post-thymectomy pain (p p 2 in either group at all time points. Conclusions Post-thymectomy intravenous administration of flurbiprofen axetil provides safe and effective analgesia for MG patients.

  3. The effect of myasthenia gravis as a prognostic factor in thymoma treatment

    Science.gov (United States)

    Aydemir, Bulent

    2016-01-01

    OBJECTIVE: Thymoma is a standard epithelial tumor. Though it is rare, it constitutes 50% of anterior mediastinal masses. Variety of immunological diseases may accompany thymoma; however, myasthenia gravis (MG) is the most frequently associated paraneoplastic syndrome. Most effective treatment for thymoma is complete surgical resection. In this study, impact of MG on prognosis of thymoma cases was examined. METHODS: Records of 61 patients who underwent surgery with diagnosis of thymoma between January 2003 and September 2016 were retrospectively reviewed. All cases were analyzed for data related to age, gender, complaint, localization of lesion, surgical procedure, histopathological diagnosis, stage, MG, and long-term follow-up results. RESULTS: Total of 58 cases were included in the study. Of those, 37 patients were male and 21 were female. Mean age was 48 years. While 24 cases of thymoma were accompanied by MG, 34 cases were not. Duration of follow-up ranged from 1 month to 155 months. CONCLUSION: It was found that in group with MG, 5-year survival rate was 87.5% while it was 82.4% in group without MG. Despite longer duration of survival in group of thymoma associated with MG, there was no significant statistical difference between groups (p=0.311). PMID:28275751

  4. Association between myasthenia gravis and cognitive function: A systematic review and meta-analysis

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    Zhifeng Mao

    2015-01-01

    Full Text Available The course of myasthenia gravis (MG is complicated by increased reports of cognitive defects in both human and animal models, which suggests potential central nervous system (CNS damage. We conducted a systematic review of the relationships between MG and cognitive function. This systematic review followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA guidelines. Major databases were searched to examine the neuropsychological studies of adults with MG. Weighted effect sizes were pooled by cognitive domain. Eight studies representing 300 subjects were included. Eight cognitive domain categories were identified: (i Mini-Mental State Examination (MMSE, (ii language, (iii processing speed, (iv verbal learning and memory, (v visual learning and memory, (vi attention span, (vii response fluency, and (viii motor performance. Nine (cognitive domain categories, MMSE, language, processing speed, verbal learning and memory (except for delayed recall memory, and motor performance of 16 cognitive tasks revealed significant moderate effect sizes. Verbal logical-delayed memory, finger tapping with the preferred hand, and the Symbol Digit Modalities Test showed a greater magnitude relationship to cognitive function than did other specific cognitive domains. Verbal learning and memory seems to be the most significant affected according to cognitive domain categories. For MG, the ability of attention, response fluency, visual learning, and memory seems to be reserved. The MG patients seem to perform significantly worse than the non-MG controls in a range of cognitive domains. Our findings should be interpreted with caution because of the clinical and methodological heterogeneity of included studies.

  5. Electrocardiography as the First Step for the Further Examination of Cardiac Involvement in Myasthenia Gravis

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    Takao Kato

    2016-01-01

    Full Text Available Introduction. Cardiac involvement of myasthenia gravis (MG accompanies a poor prognosis. In the present study, we aimed to investigate the relationship between ECG abnormality and cardiac involvement. Methods. Of 178 patients diagnosed with MG between 2001 and 2013 at our hospital, we retrospectively analyzed consecutive 58 patients who underwent both ECG and echocardiography and without underlying cardiovascular disease. ECG abnormalities were defined by computer-assigned Minnesota-codes. Cardiac damage was defined as either (1 ejection fraction (EF 8 on echocardiography. Results. Thirty-three patients (56.8% had ECG abnormality. An elevated E/e′ was observed in patients with ECG abnormality compared to those without ECG abnormality (11.2±3.2, 8.7±2.2, resp., p=0.03. Among patients with ECG abnormality, 14 of 15 patients showed cardiac damage. Among patients without ECG abnormality, 6 of 33 patients showed cardiac damage (p=0.003. Reduced EF was observed in five patients (8.6% with ECG abnormality and none in patients without ECG abnormality. Conclusions. ECG may aid as the first step for the further examination of cardiac damage in patients with MG.

  6. Thymic lesions and myasthenia gravis. Diagnosis based on mediastinal imaging and pathological findings

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    Pirronti, T.; Rinaldi, P.; Marano, P. [Univ. Cattolica del S. Cuore, Roma (Italy). Inst. of Radiology; Batocchi, A.P.; Evoli, A.; Di Schino, C. [Univ. Cattolica del S. Cuore, Roma (Italy). Inst. of Neurology

    2002-07-01

    Purpose: To achieve a better understanding of the role of CT and MR imaging in the study of the mediastinum in patients with myasthenia gravis (MG). Material and Methods: Mediastinal CT and MR findings were correlated with the histopathological results in 104 thymectomized MG patients. Results: CT was performed in 104 patients; in 11 of them, MR was also carried out. 44 patients had hyperplasia at histology. On CT, thymic hyperplasia was confirmed in 16 cases, thymoma was diagnosed in 10 and a normal thymus in 18 (sensitivity 36%, specificity 95%). Of 52 patients with thymoma at histology, CT showed thymoma in 46, hyperplasia in 1, and normal thymus in 5. CT showed 88.5% sensitivity and 77% specificity for thymoma. In 10 patients with invasive thymoma, CT was indiscriminate, while invasiveness was detected in 7 cases at MR (70% sensitivity) and at CT in 1 case. Both CT and MR detected tumor recurrence in 5 cases, but the exact localization and degree of invasion were best defined by MR. Conclusion: In MG patients CT is a sensitive, specific and efficient modality for detecting thymoma, but is less so for detecting thymic hyperplasia. MR was shown to be accurate in detecting invasive thymoma both preoperatively and in postoperative follow-up.

  7. The usefulness of MRI for detection of the thymus gland in myasthenia gravis

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    Hokezu, Youichi; Kaseda, Syun; Arimura, Kimiyoshi; Osame, Mitsuhiro; Baba, Kuniaki (Kagoshima Univ. (Japan). Faculty of Medicine); Ohkubo, Koichi; Hagiwara, Hiroshi

    1989-08-01

    Seven patients with myasthenia gravis (MG) were examined to find thymus or thymoma employing chest radiographs, computed tomography (CT), pneumomediastinography (PMG), computed tomography after pneumomediastinography (PMG-CT) and magnetic resonance imaging (MRI). X-ray CT examination could reveal thymus only in half out of 6 cases scanned. On the other hand, MRI confirmed thymus or thymoma in 6 out of 7 patients. PMG and PMG-CT confirmed thymus or thymoma clearly in all of the 4 cases studied. PMG and PMG-CT examinations revealed thymus or thymoma more clearly than MRI. MRI is, however, an examination causing no pain to the patients and also more superior to X-ray CT in distinguishing between a thymus and mediastinal fat or vascular structure. In addition, MRI could reveal even capsules in thymoma which were never revealed by X-ray CT. We concluded that MRI could be an alternative method to CT and PMG in detection of thymus or thymoma in MG. (author).

  8. Rare association of thymoma, myasthenia gravis and sarcoidosis : a case report

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    Kurukumbi Mohankumar

    2008-07-01

    Full Text Available Abstract Introduction The association of thymoma with myasthenia gravis (MG is well known. Thymoma with sarcoidosis however, is very rare. We presented an interesting case with coexisting thymoma, MG and sarcoidosis. Case presentation A 59-year-old female patient with a history of sarcoidosis was admitted to the hospital with a one-day history of sudden onset of right-sided partial ptosis and diplopia. Neurosarcoidosis with cranial nerve involvement was considered, but was ruled out by the clinical findings, and MG was confirmed by the positive tensilon test, electrophysiological findings and positive acetylcholine receptor binding antibodies. On further evaluation, a CT chest scan showed a left anterior mediastinal mass and bilateral lymphadenopathy. Post surgical diagnosis confirmed the thymoma and sarcoidosis in the lymph nodes. Conclusion When two or more diseases of undetermined origin are found together, several interesting questions are raised. It is important to first confirm the diagnoses individually. Immunologic mechanisms triggering the occurrence of these diagnoses together, are difficult to address. Although the coexistence of thymoma, MG and sarcoidosis may be coincidental, it is noteworthy to report this case because of the multiple interesting features observed as well as the rarity of occurrence.

  9. Skeletal-muscle CT, with special reference to polymyositis and myasthenia gravis

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    Higashi, Yasuto; Ono, Shimato; Yasuda, Takeshi; Morimoto, Kenji; Terao, Akira; Shirabe, Teruo; Yokobayashi, Tsuneo (Kawasaki Medical School, Kurashiki, Okayama (Japan))

    1984-10-01

    We here report on skeletal-muscle CT at the thigh level as studied using a whole-body CT scanner, with special reference to polymyositis (PM) and myasthenia gravis (MG). Early diseased muscles appeared homogenous and were likely to be almost normal. The first sign of muscular atrophy was the appearance of small, patchy or linear, low-density tissues in several muscles. These low-density tissues gradually increased in number until finally the diseased muscles were totally replaced by low-density tissue. These pathological findings were more severe in PM than in MG. There was a maldistribution of low-density tissue in several cases of PM. According to these findings, skeletal-muscle CT was thought to be of great help for the recognition of the general condition of muscles and for the follow-up on the patients. We think skeletal-muscle CT has a very practical application for the better selection of suitable muscular biopsy and EMG sites and for the better clinical interpretation of these findings.

  10. Visualization of the thymus in myasthenia gravis. Comparison between pneumomediastinography and CT of the anterior mediastinum

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    Komatsu, Midori; Tanaka, Makoto; Morimatsu, Mitsunori; Hirai, Shunsaku; Heshiki, Atsuko (Gunma Univ., Maebashi (Japan). School of Medicine)

    1982-12-01

    We investigated whether CT had any advantage over pneumomediastinography (PMG) for the visualization of thymus in twenty-one patients with myasthenia gravis (MG). In two cases of thymoma which had been suspected with chest radiography, CT demonstrated an anterior mediastinal tumor distinguishable from other mediastinal organs, and for these cases PMG was not performed. Excluding three patients who were submitted to PMG only, CT of the anterior mediastinum was carried out in sixteen patients of MG, revealing thymic shadows in seven (44%). PMG followed by conventional tomography was done subsequently in three of these seven cases, for whom thymectomy was indicated because of uncontrollable myasthenic symptoms, and in all patients finger-like thymic shadows were disclosed. The vertical extension of thymus was more easily demonstrable by PMG than CT. PMG was carried out in six of nine patients in whom CT was negative, and in all cases thymic shadows were obvious with subsequent conventional tomography. Consequently, false negative rate of CT was at least 38% (6/16) with regard to the visualization of the nontumorous thymus. Although CT of the anterior mediastinum is useful as a screening method because of its non-invasiveness, its negative result does not rule out an absence of the pathologic thymus in view of its high false negative rate. In this regard, PMG is still necessary for the final determination of the thymic configuration in the MG patients.

  11. Radiographical diagnosis of the thymus in myasthenia gravis. Comparison of pneumomediastinography and computed tomography

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    Tanimura, Shigeo; Banba, Jiro; Masaki, Mikio; Irimoto, Masahiro; Matsushita, Akira

    1987-11-01

    Comparison of radiographical findings of the thymus between pneumomediastinography and computed tomography were studied in 35 patients with myasthenia gravis. The patients consisted of 10 patients with thymoma and 25 without thymoma confirmed at the operations. Pneumomediastinography was very useful to discern whether the thymoma was invasive or noninvasive, but not contributory to know whether the thymus was composed of folicular lymphoid hyperplasia or normal thyimic tissues. Computed tomography was also useful to dertermine the localization and the invasiveness of the thymoma, but not helpful to know whether the thymus was of follicular lymphoid hyperplasia or normal tissues. However, the finding of ''reticular pattern''-many small nodules scattered resicularly in the thymus-in computed tomography could be regarded as a sign suggesting follicular lymphoid hyperplasia of the thymus. Therfore, both pneumomediastinography and computed tomography were very useful in the diagnosis of the localization and the invasiveness of the thymoma but not for the diagnosis of follicular lymphoid hyperplasia of the thymus. Neverethless the finding of ''reticular pattern'' computred tomography was helpful in the diagnosis of follicular lymphoid hyperplasia.

  12. Preoperative High-Dose Steroid Has Long-Term Beneficial Effects for Myasthenia Gravis

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    Syuichi Tetsuka

    2013-01-01

    Full Text Available Previous studies addressing preoperative steroid treatment have revealed that control of myasthenia gravis (MG with steroids prior to surgery appeared to stabilize postoperative status. The purpose of our study was to clarify the clinical benefits of the preoperative programmed high-dose steroid treatment on the long-term outcomes of MG patients. We retrospectively reviewed the records of 171 MG patients who were followed up after undergoing thymectomy in our hospital between 1988 and 2006. One hundred and thirteen patients in the programmed treatment group had received preoperative steroid treatment, while 58 patients received no steroid treatment during the preoperative period. Clinical remission, which was defined as the achievement of the modified pharmacologic remission (PR for at least 1 year, and clinical benefits were compared between the two groups. With regard to the remission after thymectomy, Kaplan-Meier life-table curves for patients in the preoperative steroid treatment group versus those for patients in the no steroid preoperative treatment group revealed a significantly higher probability of the PR in the preoperative steroid treatment group (log-rank test, P<0.01. This study might be the first, as per our knowledge, to indicate that preoperative programmed high-dose steroid treatment has long-term beneficial effects for MG patients.

  13. Association between Glucocorticoid-Induced Osteoporosis and Myasthenia Gravis: A Cross-Sectional Study.

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    Shingo Konno

    Full Text Available To investigate the association between glucocorticoid-induced osteoporosis and myasthenia gravis (MG using a cross-sectional survey in Japan.We studied 363 patients with MG (female 68%; mean age, 57 ± 16 years who were followed at six Japanese centers between April and July 2012. We evaluated the clinical information of MG and fractures, bone markers, and radiological assessment. Quality of life was measured using an MG-specific battery, MG-QOL15.Glucocorticoids were administered in 283 (78% of 363 MG patients. Eighteen (6% of 283 MG patients treated with prednisolone had a history of osteoporotic fractures. The duration of glucocorticoid therapy, but not the dose of prednisolone, was associated with the osteoporotic fractures in MG patients. Bone mineral density was significantly decreased in the MG patients with fractures. The multivariate analyses showed that the total quantitative MG score was the only independent factor associated with osteoporotic fractures (OR = 1.30, 95% CI 1.02-1.67, p = 0.03. MG patients who had experienced fractures reported more severe difficulties in activities of daily living.Glucocorticoid-induced osteoporosis aggravates quality of life in patients with MG.

  14. Two patients with a neuroendocrine tumour of the small intestine and paraneoplastic myasthenia gravis

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    Hermans, M A W; Stelten, B M L; Haak, H R; de Herder, W W; Dercksen, M W

    2014-01-01

    Summary This paper reports on two patients with a long-standing diagnosis of an ENETS stage IV neuroendocrine tumour (NET) of the small intestine who developed neurological symptoms. The first patient only had bulbar symptoms and tested positive for acetylcholine receptor antibodies. The second patient had more classical symptoms of fatigable diplopia and muscle weakness of the legs, but no detectable antibodies. The diagnosis of paraneoplastical myasthenia gravis (MG) was postulated. Both patients were treated with pyridostigmine for MG and octreotide for the NETs. Interestingly, treatment of the NETs resulted in improvement of myasthenic symptoms. Paraneoplastic MG has been described to occur with certain malignancies, mainly thymoma. Herein, we prove that the association with gastrointestinal NETs, however, rare, is also one to be considered by clinicians dealing with either of these diseases. The pathogenesis has yet to be elucidated. Learning points NETs are rare malignancies with a wide variety of symptoms.Paraneoplastic MG can occur with various types of malignancies.Herein, we provide evidence of paraneoplastic MG in association with a grade IV NET of the small intestine.Treatment of the NETs resulted in remission of myasthenic symptoms in one patient. PMID:24839548

  15. Thymic pathologies in myasthenia gravis: a preoperative assessment of CAT scan and nuclear based imaging.

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    Jordan, Berit; Kellner, Juliane; Jordan, Karin; Bähre, Manfred; Behrmann, Curd; Zierz, Stephan

    2016-04-01

    Precise diagnostic work up of a suspected thymic pathology in patients with myasthenia gravis (MG) is very important for potential surgical implications and further disease course. In this study the diagnostic value of combined preoperative radiological (CAT scan) and nuclear based imaging (octreotide and thallium scintigraphy) in patients with MG was evaluated. Twenty four patients were included. Histopathology revealed thymoma in nine patients, thymic carcinoma (TC) in one patient, lymphofollicular hyperplasia in seven patients, and involuted thymus in another seven patients. Diagnostic sensitivity for detecting thymoma/TC was 80 % in CAT scan as well as in somatostatin scintigraphy; the combination of both procedures reached 90 %. However, the diagnostic specifity to exclude thymoma in CAT scan was 100 % and in octreotide scintigraphy 85.7 %. Semiquantitative octreotide uptake significantly correlated with histological grading of thymoma/TC (r = 0.764) and histological proliferation rate Ki67 (r = 0.894). Thallium scintigraphy was positive only in one out of four thymoma cases. In this study, somatostatin scintigraphy has been shown to be a useful additional diagnostic technique in detecting thymic malignancies in patients with MG. These results might be especially helpful in patients with late onset MG as these patients are in general no candidates for thymectomy.

  16. Clinical fluctuations in MuSK myasthenia gravis are related to antigen-specific IgG4 instead of IgG1

    NARCIS (Netherlands)

    Niks, E H; van Leeuwen, Y; Leite, M I; Dekker, F W; Wintzen, A R; Wirtz, P W; Vincent, A; van Tol, M J D; Jol-van der Zijde, C M; Verschuuren, J J G M

    2008-01-01

    We studied the longitudinal relation between disease severity and titers of antigen-specific IgG subclasses in sera of patients with myasthenia gravis and antibodies to Muscle Specific Kinase (MuSK MG). Six patients were included of whom 55 samples had been collected during 2.5-13.4 years. Anti-MuSK

  17. Response to suxamethonium during propofol-fentanyl-N2O/O2 anaesthesia in a patient with active myasthenia gravis receiving long-term anticholinesterase therapy.

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    Vanlinthout, L E; Robertson, E N; Booij, L H

    1994-06-01

    We describe the effect of repeated suxamethonium doses during propofol-fentanyl-N2O/O2 anaesthesia in a 29-year-old woman with active myasthenia gravis receiving chronic pyridostigmine therapy. Despite adequate pre-operative pseudocholinesterase activity, suxamethonium resistance occurred. Neither bradycardia nor residual neuromuscular block were seen after repeated doses of suxamethonium.

  18. Clinical significance of detection of antibodies to fetal and adult acetylcholine receptors in myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    Qi-Guang Shi; Zhi-Hong Wang; Xiao-Wei Ma; Da-Qi Zhang; Chun-Sheng Yang; Fu-Dong Shi; Li Yang

    2012-01-01

    Objective To evaluate the frequency,distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor (AChR) in patients with myasthenia gravis (MG).Methods AChR antibodies were detected by cell-based assay in the serum of ocular MG (OMG) (n =90) and generalized MG (GMG) patients (n =110).The fetaltype (2α∶ β∶ γ∶ δ) and adult-type (2α∶ β∶ ε∶ δ) AChR were used as antigens,and their relevance to disease presentation was assessed.Results The overall frequencies of anti-adult and anti-fetal AChR antibodies were similar in all 200 patients examined,with 14 having serum specific to the AChR-γ subunit,and 22 to the AChR-ε subunit.The overall sensitivity when using the fetal and adult AChR antibodies was higher than that when using the fetal AChR antibody only (P =0.015).Compared with OMG patients,the mean age at disease onset and the positive ratio of antibodies to both isoforms of the AChR were significantly higher in patients who subsequently progressed to GMG.Older patients and patients with both anti-fetal and anti-adult AChR antibodies had a greater risk for developing generalized disease [odds ratio (OR),1.03;95% confidence interval (CI),1.01-1.06 and OR,5.09;95% CI,2.23-11.62].Conclusion Using both fetal-and adulttype AChRs as the antigens may be more sensitive than using either subtype.Patients with serum specific to both isoforms are at a greater risk of progressing to GMG.Patients with disease onset at an advanced age appear to have a higher frequency of GMG conversion.

  19. Validation of the MG-DIS: a disability assessment for myasthenia gravis.

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    Raggi, Alberto; Leonardi, Matilde; Schiavolin, Silvia; Antozzi, Carlo; Brenna, Greta; Maggi, Lorenzo; Mantegazza, Renato

    2016-05-01

    This paper is aimed to present the validation of the myasthenia gravis disability assessment (MG-DIS), a MG-specific patient-reported disability outcome measure. Consecutive MG patients were enrolled, followed-up for 12 months and administered the SF-36, the WHO disability assessment schedule (WHODAS 2.0) and the preliminary 31-item MG-DIS addressing impairments and activity limitations. Factor structure and metric properties were assessed. In total, 109 patients were enrolled: 76 were females, mean age 50, mean MG duration 10.4 years, 86 were AChR-positive. The MG-DIS was reduced to 20 items, explaining 70.6 % of the original questionnaire variance, four subscales (generalized impairment-related problems; bulbar function-related problems; mental health and fatigue-related problems; vision-related problems) and an overall disability index. The MG-DIS has good metric properties (Cronbach's alpha ranging between .808 and .930), is stable, showed to be more sensitive than the WHODAS 2.0 and SF-36 to detect group differences and longitudinal changes and was well correlated with the MG-composite (.642). The MG-DIS includes items representing ocular, generalized, bulbar and respiratory symptoms, and is therefore well-built around MG-specific features. MG-DIS can be used in clinical trials as well as in observational or epidemiological studies to characterize patients' disability level and address the amount of improvement in disability. Further studies are needed to explore the possibility of a shorter disability scale.

  20. Intravenous flurbiprofen for post-thymectomy pain relief in patients with myasthenia gravis

    Science.gov (United States)

    2012-01-01

    Background Post-thymectomy pain in myasthenia gravis (MG) patients can inhibit breathing and coughing. Inappropriate usage of analgesics may exacerbate respiratory inhibition and even cause myasthenic crisis. Flurbiprofen is a non-steroidal anti-inflammatory drug (NSAID) that is commonly used to control moderate postoperative pain and is not associated with respiratory inhibition. We hypothesized that flurbiprofen may provide post-thymectomy pain relief without increasing the risk of complications in MG patients. Methods Two hundred MG patients underwent extended thymectomy from March 2006 to December 2010 and were randomly allocated to a flurbiprofen group (110 patients, 50 mg intravenous flurbiprofen axetil) or a control group (90 patients, 100 mg intramuscular tramadol) as postoperative analgesia. Visual analog scale (VAS) pain score, heart rate, blood pressure, respiratory rate, pulse oximetry (SpO2), and adverse effects were recorded before and up to 24 h after drug administration. Results There were no significant differences in the preoperative clinical characteristics of the flurbiprofen and control (tramadol) groups. Both flurbiprofen and tramadol significantly alleviated post-thymectomy pain (p < 0.05 for both), but patients in flurbiprofen group had significantly lower VAS pain scores at 0.5 h, 2 h, 4 h, and 8 h after surgery (p < 0.05 for all times). There were no significant post-thymectomy changes of heart rate, respiratory rate, mean arterial blood pressure, or SpO2 in either group at all time points. Conclusions Post-thymectomy intravenous administration of flurbiprofen axetil provides safe and effective analgesia for MG patients. PMID:23020939

  1. TUMOR NECROSIS FACTOR-ALPHA POLYMORPHISM AND SECRETION IN MYASTHENIA GRAVIS

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    Yu-zhou Guan; Li-ying Cui; Yan-feng Li; Jun-bao Zhang

    2005-01-01

    Objective To analyze the relationship between tumor necrosis factor-alpha (TNFo) gene promoter -308 polymorphism and myasthenia gravis (MG) in Chinese and analyze secretion of TNFo in peripheral blood mononuclear cells (PBMC) in MG patients.Methods A biallelic polymorphism at position -308 in the promoter of TNFα gene was screened by PCR amplification and NcoI recognition site. One hundred and twenty-three MG cases and 115 healthy controls were included in this study. MG patients were classified to different groups according to clinical type, age at onset, and sex respectively. PBMC were isolated from 20 patients and 20 healthy controls, and then cultured in the presence or absence of phytohemagglutinin (PHA) and acetycholine receptors (AchR). The supernatants were harvested after incubation and stored until TNFαwas assayed by enzyme-linked immunosorbent assay.Results The frequency of TNFα-308 allele 2 (A) was found significantly increase in MG patients and showed a trend especially in late onset (≥ 40 years) and male patients (P < 0.05). The allele A had no relationship with thymic pathogenesis in MG patients. But frequency of allele A was significantly higher in general type than in ocular type (P < 0.05). MG patients had a higher inducible level of TNFα by PHA and AchR, and could be down regulated after treatment.Conclusion Polymorphism in TNFα gene promoter -308 is associated with onset of MG. The microsatellite allele TNFα2 confer risk for the development of MG in Chinese patients. MG patients have a higher inducible level of TNFα.

  2. Evaluation of the quality of guidelines for myasthenia gravis with the AGREE II instrument.

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    Zhenchang Zhang

    Full Text Available BACKGROUND: Clinical practice guidelines (CPGs are systematically developed statements to assist practitioners in making decisions about appropriate healthcare in specific clinical circumstances. The methodological quality of CPGs for myasthenia gravis (MG are unclear. OBJECTIVE: To critically evaluate the methodological quality of CPGs for MG using AGREE II instrument. METHOD: A systematical search strategy on PubMed, EMBASE, DynaMed, the National Guideline Clearinghouse (NGC and the Chinese Biomedical Literature database (CBM was performed on September 20th 2013. All guidelines related to MG were evaluated with AGREE II. The software used for analysis was SPSS 17.0. RESULTS: A total of 15 CPGs for MG met the inclusion criteria (12 CPGs in English, 3 CPGs in Chinese. The overall agreement among reviews was moderate or high (ICC >0.70. The mean scores (mean ± SD for al six domains were presented as follows: scope and purpose (60.93% ± 16.62%, stakeholder involvement (40.93% ± 20.04%, rigor of development (37.22% ± 30.46%, clarity of presentation (64.26% ± 16.36%, applicability (28.19% ± 20.56% and editorial independence (27.78% ± 28.28%. Compared with non-evidence-based CPGs, evidence-based CPGs had statistically significant higher quality scores for all AGREE II domains (P0.05. The quality scores of CPGs developed by NGC/AAN were higher than the quality scores of CPGs developed by other organizations for all domains. The difference was statistically significant for all domains with the exception of clarity of presentation (P = 0.07. CONCLUSIONS: The qualities of CPGs on MG were generally acceptable with several flaws. The AGREE II instrument should be adopted by guideline developers, particularly in China.

  3. Social disadvantages associated with myasthenia gravis and its treatment: a multicentre cross-sectional study

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    Nagane, Yuriko; Murai, Hiroyuki; Imai, Tomihiro; Yamamoto, Daisuke; Tsuda, Emiko; Minami, Naoya; Suzuki, Yasushi; Kanai, Tetsuya; Uzawa, Akiyuki; Kawaguchi, Naoki; Masuda, Masayuki; Konno, Shingo; Suzuki, Hidekazu; Aoki, Masashi; Utsugisawa, Kimiaki

    2017-01-01

    Objectives To clarify the social disadvantages associated with myasthenia gravis (MG) and examine associations with its disease and treatment. Design Cross-sectional study. Setting and participants We evaluated 917 consecutive cases of established MG seen at 13 neurological centres in Japan over a short duration. Outcome measures All patients completed a questionnaire on social disadvantages resulting from MG and its treatment and a 15-item MG-specific quality of life scale at study entry. Clinical severity at the worst condition was graded according to the MG Foundation of America classification, and that at the current condition was determined according to the quantitative MG score and MG composite. Maximum dose and duration of dose ≥20 mg/day of oral prednisolone during the disease course were obtained from the patients' medical records. Achievement of the treatment target (minimal manifestation status with prednisolone at ≤5 mg/day) was determined at 1, 2 and 4 years after starting treatment and at study entry. Results We found that 27.2% of the patients had experienced unemployment, 4.1% had been unwillingly transferred and 35.9% had experienced a decrease in income, 47.1% of whom reported that the decrease was ≥50% of their previous total income. In addition, 49.0% of the patients reported feeling reduced social positivity. Factors promoting social disadvantages were severity of illness, dose and duration of prednisolone, long-term treatment, and a depressive state and change in appearance after treatment with oral steroids. Early achievement of the treatment target was a major inhibiting factor. Conclusions Patients with MG often experience unemployment, unwilling job transfers and a decrease in income. In addition, many patients report feeling reduced social positivity. To inhibit the social disadvantages associated with MG and its treatment, greater focus needs to be placed on helping patients with MG resume a normal lifestyle as soon as

  4. Factors predicting surgical outcome of thymectomy in myasthenia gravis: A 16-year experience

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    Nilkamal Kumar

    2011-01-01

    Full Text Available Aim: To assess the surgical outcome of myasthenia gravis (MG following thymectomy and to determine the outcome predictors to such therapeutic approach. Materials and Methods: This study is a retrospective review of 80 consecutive thymectomies performed for MG over a 16-year period. Results: There were 41 females and 39 males (mean age, 34.32 years with mean disease duration of 17.45 months prior to surgery. Stagewise distribution of the patients revealed 2.5% in stage I, 48.7% in stage IIA, 33.8% in stage IIB, 8.7% in stage III, and 6.3% in stage IV. The surgical approach was either trans-sternal (n=67 or video-assisted thoracoscopic route (n=13. Follow-up was obtained in 91.2% (n=73 of patients with mean duration of 67.7 months. At their last follow-up, 26.0% were in complete remission, 35.6% were asymptomatic on decreased medications, and 17.8% had clinical improvement on decreased medications. Overall, 79.4% of patients benefited from surgery, 8.2% had unchanged disease status, and 12.3% worsened clinically. Factors influencing favorable outcome include sex, disease stage, gland weight, and preoperative medication with anti-cholinesterase (P<0.05. There was one death in the perioperative period due to septicemia. Two patients died at fourth and seventh month following thymectomy. Conclusion: Thymectomy for MG is safe and effective. Certain influencing factors may shape treatment decisions and target higher risk patients.

  5. 重症肌无力辨证治疗探微%Differential Treatment of myasthenia gravis Exploration

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    马广斌

    2014-01-01

    目的:中医中药辨证治疗重症肌无力的临床疗效观察。方法运用中医理论辨证论治58例重症肌无力患者10个月。结果痊愈 8例,显效21例,好转24例,无效5例,总有效率为91.4%;其中中气不足型、脾肾阳虚型疗效最佳,总有效率达100%。结论中医辨证论治可以阻止 MG 病变发展,改善症状,并收到良好效果。%Objective to observe the clinical curative effect of tcM syndrome differentiation and treatment of myasthenia gravis. Methods using the theory of tcM syndrome differentiation and treatment of 58 patients with myasthenia gravis in 10 months. Results 8 cases were cured, 21 cases markedly effective, 24 cases improved, 5 cases ineffective, the total efficiency of 91.4%; the effect of qi deficiency, spleen kidney yang deficiency type best, the total effective rate was 100%. Conclusion tcM syndrome differentiation and treatment may prevent the development of MG lesions, improve symptoms, and received good results.

  6. Long-Term Respiratory Muscle Endurance Training in Patients with Myasthenia Gravis: First Results after Four Months of Training

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    Beate Rassler

    2011-01-01

    Full Text Available Myasthenia gravis (MG is characterized by reduced muscle endurance and is often accompanied by respiratory complications. Improvement of respiratory function is therefore an important objective in MG therapy. A previous study demonstrated that respiratory muscle endurance training (RMET over four weeks increased respiratory muscle endurance of MG patients to about 200% of baseline. The purpose of the present study was to establish an appropriate maintenance training and to test its effects over four months. Ten patients with mild to moderate MG participated in this study. During the first month, they performed five training sessions per week. For the following 3 months, training frequency was reduced to five sessions per two weeks. Myasthenia score, lung function, and respiratory endurance were determined prior to training, after the first month, and after 4 months. Myasthenia score improved from 0.71±0.1 to 0.56±0.1 (P=0.007. Respiratory endurance time increased from 6.1±0.8 to 20.3±3.0 min (P<0.001. In conclusion, this RMET maintenance program is feasible and is significantly beneficial for MG patients.

  7. A functional SNP in the regulatory region of the decay-accelerating factor gene associates with extraocular muscle pareses in myasthenia gravis

    KAUST Repository

    Heckmann, J M

    2009-08-13

    Complement activation in myasthenia gravis (MG) may damage muscle endplate and complement regulatory proteins such as decay-accelerating factor (DAF) or CD55 may be protective. We hypothesize that the increased prevalence of severe extraocular muscle (EOM) dysfunction among African MG subjects reported earlier may result from altered DAF expression. To test this hypothesis, we screened the DAF gene sequences relevant to the classical complement pathway and found an association between myasthenics with EOM paresis and the DAF regulatory region c.-198CG SNP (odds ratio8.6; P0.0003). This single nucleotide polymorphism (SNP) results in a twofold activation of a DAF 5?-flanking region luciferase reporter transfected into three different cell lines. Direct matching of the surrounding SNP sequence within the DAF regulatory region with the known transcription factor-binding sites suggests a loss of an Sp1-binding site. This was supported by the observation that the c.-198CG SNP did not show the normal lipopolysaccharide-induced DAF transcriptional upregulation in lymphoblasts from four patients. Our findings suggest that at critical periods during autoimmune MG, this SNP may result in inadequate DAF upregulation with consequent complement-mediated EOM damage. Susceptible individuals may benefit from anti-complement therapy in addition to immunosuppression. © 2010 Macmillan Publishers Limited. All rights reserved.

  8. A functional SNP in the regulatory region of the decay-accelerating factor gene associates with extraocular muscle pareses in myasthenia gravis.

    Science.gov (United States)

    Heckmann, J M; Uwimpuhwe, H; Ballo, R; Kaur, M; Bajic, V B; Prince, S

    2010-01-01

    Complement activation in myasthenia gravis (MG) may damage muscle endplate and complement regulatory proteins such as decay-accelerating factor (DAF) or CD55 may be protective. We hypothesize that the increased prevalence of severe extraocular muscle (EOM) dysfunction among African MG subjects reported earlier may result from altered DAF expression. To test this hypothesis, we screened the DAF gene sequences relevant to the classical complement pathway and found an association between myasthenics with EOM paresis and the DAF regulatory region c.-198C>G SNP (odds ratio=8.6; P=0.0003). This single nucleotide polymorphism (SNP) results in a twofold activation of a DAF 5'-flanking region luciferase reporter transfected into three different cell lines. Direct matching of the surrounding SNP sequence within the DAF regulatory region with the known transcription factor-binding sites suggests a loss of an Sp1-binding site. This was supported by the observation that the c.-198C>G SNP did not show the normal lipopolysaccharide-induced DAF transcriptional upregulation in lymphoblasts from four patients. Our findings suggest that at critical periods during autoimmune MG, this SNP may result in inadequate DAF upregulation with consequent complement-mediated EOM damage. Susceptible individuals may benefit from anti-complement therapy in addition to immunosuppression.

  9. Feasibility of full and rapid neuromuscular blockade recovery with sugammadex in myasthenia gravis patients undergoing surgery – a series of 117 cases

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    Vymazal T

    2015-10-01

    Full Text Available Tomas Vymazal,1 Martina Krecmerova,1 Vladimír Bicek,1 Robert Lischke2 1Department of Anaesthesiology and ICM, 2nd Faculty of Medicine, 23rd Surgical Department of 1st Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic Purpose: Myasthenia gravis (MG is an autoimmune disease interfering with neuromuscular transmission. Patients are at risk of postoperative residual curarization (PORC if nondepolarizing muscle relaxants are used. Clinically inapparent insufficient muscle strength may result in hypoventilation and postoperative bronchopneumonia. We describe a cohort of 117 cases in which sugammadex was used in MG patients undergoing surgery with muscle relaxation with rocuronium.Methods and patients: We anesthetized 117 patients with MG using rocuronium and sugammadex as neuromuscular blockade reversal agent. One hundred five patients underwent surgical thymectomy and 12 underwent cholecystectomy (five laparotomic and seven laparoscopic. We measured time from sugammadex administration to recovery and to extubation, using the TOF-Watch® (series of four consecutive electrical impulses [the train-of-four] >0.9. We tracked peripheral capillary oxygen saturation (SpO2 <95%, elevation of partial pressure of carbon dioxide (pCO2 >10% above baseline, number of reintubations within the first 48 hours, and number of pneumonias within 120 hours, postoperatively. Results were processed as average, minimum, and maximum values.Results: The period needed to reach train-of-four of 0.9 following sugammadex administration was on average 117 seconds (minimum of 105 seconds/maximum of 127 seconds and differed within deviation <10%. The time to extubation following sugammadex administration was on average 276 seconds (minimum of 251 seconds/maximum of 305 seconds and differed minimally among patients as well. We observed no SpO2 <95%, no pCO2 elevation >10% above a baseline, no emergent reintubation within the

  10. 胸腺瘤相关重症肌无力发病的免疫学机制研究进展%Immune mechanism of thymomas in the development of myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    王卫

    2013-01-01

    Myasthenia gravis is an acquired autoimmune disorder which is cellular-immunity-dependent,humoral-immunitymediated and complement participated.It is still not clear which initial factor causes the immune response.The characteristic of myasthenia gravis is that many of the patients have an abnormality in their thymus.So it is speculated that the immune response is initiated in thymus.The thymus is the central organ of the immune system,playing the key role in T cell generation,diversification and maturation.T cells are positively selected for their recognition of antigens associated with MHC expressed in the thymus,and negatively selected according to their response to the self-antigens.The result of these selection is that the T cell use the MHC expressed on the other cells of the body for recognition of foreign antigens but do not react with self-antigens.Perhaps the most important role of thymus is the induction of immune self-tolerance that functions to prevent self-harm or autoimmunity.Thymomas are functional tumors originated from epithelial of the thymus,which have the capacity to induce T cell to differentiate and mature.What mechanism is myasthenia gravis associated thymoma initiated by? This review will summarize the existing evidence of thymus,thymoma and myasthenia gravis and try to clarify the immune mechanism of initiation of myasthenia gravis associated thymomas.%重症肌无力(MG)是一种细胞免疫依赖、体液免疫介导、补体参与的获得性自身免疫疾病,但其引起免疫应答的始动环节仍不十分清楚.由于绝大多数MG患者伴随胸腺异常,故推测导致MG发生的免疫反应起始部位可能在胸腺.胸腺是T细胞分化、发育、成熟的场所,后者在此经历阳性选择和阴性选择,从而获得识别外来抗原的能力,但同时也去除了对自身抗原的反应性,因此胸腺的重要作用之一在于诱导自身免疫耐受,从而避免自身免疫性疾病的发生.在伴胸腺增生的MG

  11. Comparison of diagnosis value for new onset myasthenia gravis by many clinical auxiliary examinations

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    BACKGROUND: The clinical values of neostigmine test, clinical electrophysiologic study and acetylcholine receptor antibody detection in diagnosing myasthenia gravis (MG) found newly are unclear in China.OBJECTIVE: To investigate the reference value of common clinical diagnosis parameters in correctly diagnosing untreated MG found newly.DESIGN: Retrospective case analysis.SETTING: Department of Neurology, Beijing Hospital, Ministry of Health.PARTICIPANTS: Totally 156 outpatients with MG admitted to Department of Neurology, Beijing Hospital,Ministry of Health between January 1999 and December 2002. The involved patients, 72 males and 84 females, were aged 2 - 79 years. They were classified according to Osserman's criteria: ⅡA 72, ⅡB 76, Ⅲ3 and Ⅳ 5. They were all subjected to being inquired of disease history, neostigmine test, and acetylcholine receptor antibody detection, met the diagnosis criteria of Neuroimmunology Committee of China, and confirmed by clinical electrophysiologic detections; Informed consents were obtained from all the involved subjects.METHODS: ①After admission, every patient was intramuscularly injected with 1.5 mg neostigmine; If the patient was a child, the injection dose was decreased according to his/her age. If his/her score of any observation index after injection was improved ≥ 50% as compared with before injection , his positive index was Set as positive. Positive neostigmine test was set if there was one positive index. ②Repetitive nerve stimulation and single fiber electromyography were performed with Dantec Keypoint electromyogram (EMG) apparatus. ③Acetylcholine receptor antibody was detected by ELISA method.MAIN OUTCOME MEASURES: Clinical absolute and relative scores of MG, acetylcholine receptor antibody level, and repetitive nerve stimulation and single fiber electromyography examination results.RESULTS: The positive rates of neostigmine test, repetitive nerve stimulation and single fiber electromyography examination

  12. Frequency of myasthenic crisis in relation to thymectomy in generalized myasthenia gravis: A 17-year experience

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    Shalmani Hamidreza

    2004-09-01

    Full Text Available Abstract Background Myasthenic crisis is the most serious life-threatening event in generalized myasthenia gravis (MG patients. The objective of this study was to assess the long-term impact of thymectomy on rate and severity of these attacks in Iranian patients. Methods We reviewed the clinical records from 272 myasthenic patients diagnosed and treated in our neurology clinic during 1985 to 2002. Fifty-three patients were excluded because of unconfirmed diagnosis, ocular form of MG, contraindication to surgery, concomitant diseases and loss to follow-up. The Osserman classification was used to assess the initial severity of the disease. Frequency and severity of the attacks were compared between two groups with appropriate statistical tests according to the nature of variables. Multivariate logistic regression analysis was used to assess the predictors of myasthenic crisis in the group of patients without thymoma. Results 110 patients were in thymectomy group and the other 109 patients were on medical therapy. These two groups had no significant differences with respect to age at onset, gender, Osserman score in baseline and follow up period. 62 patients (28.3% of all 219 patients had reported 89 attacks of myasthenic crisis. 20 patients of 62 (32% were in thymectomy group and 42 (68% were in the other group. There was significant difference between the two groups in number of patients with crisis (P = 0.001; odds ratio = 2.8 with 95% CI of 1.5 to 5.2. In addition, these attacks were more severe in group of non-thymectomized patients as the duration of ICU admission was longer and they needed more ventilatory support during their attacks. Regression model showed thymectomy and lower age at onset as two predictors of decrement in myasthenic crisis rate in non-thymomatous MG patients. Conclusions It is suggested that frequency and severity of myasthenic attacks as important endpoints in evaluation of MG patients. Thymectomy seems to have a

  13. Shenqi Fuzheng Injection Alleviates the Transient Worsening Caused by Steroids Pulse Therapy in Treating Myasthenia Gravis

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    Guo-Yan Qi

    2013-01-01

    Full Text Available Purpose. To evaluate the treatment effect and side effect of Shenqi Fuzheng Injection (SFI on alleviating transient worsening of myasthenia gravis (MG symptoms caused by high-dose steroids pulse therapy. Methods. Sixty-six consecutive patients with MG were randomly divided into two groups: the treatment group treated with SFI and methylprednisolone pulse therapy (MPT and the control group treated with MPT alone. The severity of MG before, during, and after MPT and the duration of transient worsening (TW were evaluated and compared with the clinical absolute scoring (AS and relative scoring (RS system. Results. Twenty-nine patients experienced TW in each group. At TW, the AS was significantly increased (P<0.000 in both groups compared with baseline data, with the AS increase in the treatment group (16.8 ± 2 significantly smaller (P<0.05 than in the control group (24.9 ± 2.5. At the end of the treatment course, the AS for the treatment group was significantly decreased (7.5 ± 0.9 compared with at TW, although no significant difference compared with the control (9.7 ± 1.1. The TW lasted 1–6 days (mean 3.7 for the treatment group, significantly shorter (P<0.05 than 2–12 days (mean 7.8 for the control. The RS for the treatment group at the end of treatment was 43.8%–100% (mean 76.8% ± 2.6%, significantly better than the control group: 33.3%–100% (mean 67.2 ± 3.6%. Slight side effects (18.75% included maldigestion and rash in the treatment group. Conclusion. SFI has a better treatment effect and few side effects and can alleviate the severity and shorten the duration of the transient worsening of MG during steroids pulse therapy.

  14. Detection and clinical significance of serum autoantibodies in patients with myasthenia gravis

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    Yun LIU

    2016-10-01

    Full Text Available Objective To investigate the expressions and clinical significance of serum anti - acetylcholine receptor antibodies (AChR-Ab, anti-Titin antibodies (Titin-Ab, and anti-Ryanodine receptor antibodies (RyR-Ab in patients with myasthenia gravis (MG. Methods Serum AChR-Ab, Titin-Ab and RyR-Ab were detected with enzyme-linked immunosorbent assay (ELISA in 182 MG patients, 105 patients of other neurological diseases (OND and 62 normal controls. Results Serum AChR-Ab, Titin-Ab and RyR-Ab were detected positive respectively in 68.13% (124/182, 64.29% (117/182 and 67.03% (122/182 of patients in MG group. The positive rates of those antibodies in MG group were significantly higher than those in OND group (P = 0.000, for all and control group (P = 0.000, for all. When 3 antibodies coexisted, the sensitivity in the diagnosis of MG was 41.21%, with 99.40% of specificity. There was no significant difference in the positive rate of serum AChR-Ab, Titin-Ab and RyR-Ab between early-onset MG subgroup and late-onset MG subgroup (P > 0.05, for all. The positive rates of AChR-Ab were significantly higher in MG patients with thymoma than in those without thymoma (P = 0.004. There was no significant difference in the positive rate of Titin-Ab and RyR-Ab between MG with thymoma subgroup and MG without thymoma subgroup (P > 0.05, for all. The prevalence of AChR-Ab in generalized MG (GMG atients (Ⅱa and Ⅱb was higher than those in ocular MG (OMG patients (typeⅠ; P = 0.005, 0.012. There was no significant difference in the positive rate of Titin-Ab and RyR-Ab between GMG subgroup and OMG subgroup (P > 0.05, for all. Conclusions Serum AChR-Ab, Titin-Ab and RyR-Ab can be used as ndicators of the diagnosis of MG. Patients with 3 coexisted positive antibodies are highly suspected as MG. Higher AChR-Ab level in serum of OMG patients indicates the possibility of progressing to GMG. DOI: 10.3969/j.issn.1672-6731.2016.10.007

  15. Decrease of 25 kD protein component in the muscle of myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    Hui-Min Ren; Zhi-Gang; Zhou Xiang-Jun; Chen Jun Huang; Chuan-Zhcn Lu

    2000-01-01

    Objective To explore whether the protein components have difference between normal and myasthenia gravis (MG) skeletal muscles and the differential components of protein was associated with muscle contraction components. Methods Proteins were extracted from 10 cases of normal muscles and 17 cases of MG skeletal muscles with PBS and Gubstraub solution, respectively. The components of protein were analyzed by SDS-PAGE in common and micro methods in double blind. Composition of the differential protein band was discovered by two-dimensional electrophoresis. Results SDS-PAGE patterns showed that concentration of the protein band with mass of about 25 kD in the MG muscles were much lower than that in the normal muscles. The density of the protein band in the PBS extraction, in the common method, was 4.20±2.31 and 1.40±0.47 in the normal and MG muscles, respectively (p<0.01), in the micro method, it was 4.62±1.94 and 1.66±0.56 in the normal and MG muscles. respectively (p<0.001). The value of density for 25 kD protein band in the Gubstraub solution extraction, in the common analysis was 4.14 ± 1.33 and 2.02 ±1.08 in the normal and MG muscles, respectively (p<0.001), and it in the micro analysis was 4.26±2.58 and l.34±0.79 in the normal and MG muscles, respectively (p<0.001).The pattern of two-dimensional electrophoresis demonstrated that the differential 25 kD protein band consisted of two components at least with adjacent isoelectric point on the alkaline side in the gel, and they were proved to be irrelated to the components of myosin light chains. Conclusion It was suggested that 25 kD protein from skcletal muscle could be associated with the pathogeny or developing of MG.

  16. Myasthenia gravis and thymoma: evaluation of 41 patients Miastenia grave e timoma: avaliação de 41 pacientes

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    JOSÉ LAMARTINE DE ASSIS

    1999-03-01

    Full Text Available We evaluated the epidemiological, clinical, laboratory and therapeutical aspects of 41 patients with thymomatous myasthenia gravis. Thirty five patients (85.36% were submitted to thymectomy. Follow-up ranged from two to 18 years. Diagnosis of thymoma was based upon clinical investigations and CT scan of the anterior mediastinum and in 11 patients supported by immunological tests of anti-striated muscle antibodies with a positive result in more than 80% of cases. Histopathologic examination of all thymomectomized patients confirmed the diagnosis of thymoma. There was a significant predominance of benign over malignant thymoma. Occurred higher prevalence of male patients and of patients over 40 years of age. The therapeutical strategy to control myasthenic clinical findings was the same as that for non-thymomatous myasthenia gravis. The corticosteroids associated to cytotoxic drugs were less often used. Radiotherapy of the anterior mediastinum was more often used in patients having invasive tumors submitted to surgery or not. With regard to survival and control of myasthenia gravis, especially in younger patients and in those submitted to early surgery, results of treatment were surprisingly favorable.Avaliamos 41 pacientes com miastenia grave timomatosa sob os aspectos epidemiológico, clínico e terapêutico. Trinta e cinco pacientes (85,36% foram timectomizados. O seguimento clínico variou de dois meses até 18 anos. O diagnóstico do timoma foi fundamentado no estudo de imagem do mediastino (tomografia axial computadorizada e, em 11 pacientes, complementado com a determinação sérica de anticorpos para músculo estriado com resultado positivo em mais de 80% dos casos e confirmado pelo exame anátomo-patológico do timo realizado em todos os pacientes operados. Ocorreu predomínio significante de timomas benignos sobre timomas malignos, forma clínica generalizada severa, frequente envolvimento do sexo masculino e, em pacientes com mais de

  17. Progressive bilateral ophthalmoparesis--a case of simultaneous autoimmunity: balancing Graves' ophthalmoparesis and ocular myasthenia.

    Science.gov (United States)

    Canepa, Carlo; Venu, Maya

    2016-01-04

    A 44-year-old woman with no medical history presented with a 1-year history of horizontal diplopia, bilateral exophthalmos and progressive asymmetrical ophthalmoparesis, with no pupillary dysfunction or ptosis. Within 3 months of her initial presentation, she noticed paresis of right eye abduction, followed after 1 month with paresis of left eye abduction. Initial investigations revealed positive antiperoxidase antibodies for Graves' disease and positive AChR for myasthenia gravis. MRI of the brain showed increased intensity in bilateral inferior rectus muscles and CT of the chest showed thymic hyperplasia. Treatment with carbimazole and pyridostigmine was started, with complete resolution after 1 month.

  18. Morphological and Molecular Characterization of Exophiala polymorpha sp. nov. Isolated from Sporotrichoid Lymphocutaneous Lesions in a Patient with Myasthenia Gravis.

    Science.gov (United States)

    Yong, Lee K; Wiederhold, Nathan P; Sutton, Deanna A; Sandoval-Denis, Marcelo; Lindner, Jonathan R; Fan, Hongxin; Sanders, Carmita; Guarro, Josep

    2015-09-01

    Exophiala species are capable of causing cutaneous and subcutaneous infections in immunocompromised patients. An Exophiala isolate was cultured from a biopsy specimen of a lesion on the forearm of a patient with myasthenia gravis. The patient also had lesions on the palm and distal aspects of the hand, which were successfully treated with a long-term course of itraconazole. A detailed morphological and molecular characterization of the isolate was undertaken. Phylogenetic analysis of the internal transcribed spacer region and portions of the β-tubulin and translation elongation factor 1-alpha genes indicated that the isolate was a novel species closely related to but genetically distinct from species within the Exophiala spinifera clade; the name Exophiala polymorpha sp. nov. is proposed. Morphologically, E. polymorpha most closely resembles E. xenobiotica but it differs in possessing phialides bearing prominent, wide collarettes, and it does not produce chlamydospores.

  19. Disease specific enrichment of circulating let-7 family microRNA in MuSK+ myasthenia gravis.

    Science.gov (United States)

    Punga, Tanel; Bartoccioni, Emanuela; Lewandowska, Marta; Damato, Valentina; Evoli, Amelia; Punga, Anna Rostedt

    2016-03-15

    Myasthenia gravis (MG) patients with antibodies against the muscle specific tyrosine kinase (MuSK+) have predominantly involvement of cranio-bulbar muscles and do not display thymus pathology, as do acetylcholine receptor antibody seropositive (AChR+) MG patients. In search of novel biomarkers for MuSK+ MG, we evaluated circulating serum microRNAs. Four analyzed microRNAs were specifically elevated in MuSK+ MG patient serum samples: let-7a-5p, let-7f-5p, miR-151a-3p and miR-423-5p. The circulating microRNA profile in MuSK+ MG differs from the profile previously observed in the serum of AChR+ MG, thus indicating the etiological difference between these two entities. We propose that the identified microRNAs could serve as potential serum biomarkers for MuSK+ MG.

  20. Treatment of myasthenia gravis with dropped head: a report of 2 cases and review of the literature.

    Science.gov (United States)

    Tamai, Michihiro; Hashimoto, Takao; Isobe, Takashi; Sato, Hiromasa; Doden, Tadashi; Nakano, Takeshi

    2015-05-01

    We report two patients with myasthenia gravis (MG) who showed dropped head as an early myasthenic manifestation. They had elevated anti-acetylcholine receptor antibody and showed improvement of the symptoms after intravenous injection of edorophonium chloride. One patient had thymoma and developed myasthenic crisis two weeks after thymectomy. The patient recovered from the crisis after a combination of immunoadsorption plasmapheresis (IAPP) and initiation of steroid and tacrolimus. The other patient without thymoma initiated treatment with steroid, tacrolimus and IAPP and showed complete recovery one month later. Dropped head in MG can recover well with immunosuppression therapy using steroid, and IAPP is helpful in getting a rapid improvement of dropped head as well as recovery from myasthenic crisis. When we consider treatment for MG with dropped head, we should take into account that MG of this type can develop myasthenic crisis and use the same treatment strategy as that for generalized MG.

  1. Immunological pathogenic mechenism of myasthenia gravis%重症肌无力的免疫学发病机制

    Institute of Scientific and Technical Information of China (English)

    蒋莉; 蔡方成

    2001-01-01

    @@ 重症肌无力(myasthenia gravis,MG)是神经肌肉接头处突触后膜上乙酰胆碱受体(acetycholine receptor,AchR)自身致敏后,受体遭到破坏所致的自身免疫性疾病.抗AchR抗体(anti-AchR antibody,AchR-Ab)介导的体液免疫和T细胞介导的细胞免疫是其主要发病机制.胸腺则是激活和维持MG自身免疫反应的重要因素,同时某些遗传及环境因素也与MG的发病密切相关.

  2. Myasthenia gravis and thymus: long-term follow-up screening of thymectomized and non-thymectomized patients

    Directory of Open Access Journals (Sweden)

    Paulo Jose Lorenzoni

    2013-07-01

    Full Text Available Thymoma screening is recommended at the onset of myasthenia gravis (MG or when patients with MG present with clinical deterioration or a progressive increase of anti-acetylcholine receptor antibody. However, it is unknown if it is necessary to repeat the screening of thymoma at fixed intervals, even in the absence of MG deterioration, when the initial screening is negative. We analyzed the recurrence rate and incidence of new thymoma in a series of patients with well-controlled MG. The sample consisted of 53 patients, aged 17 to 72 years, and the follow-up varied between 75 and 472 months. The chest computerized tomography detected thymus abnormalities in eight patients at the initial screening and no abnormalities in all patients at a second screening after five years. The findings of this study support the classical opinion that screening for thymoma should be recommended only if there is clinical deterioration due to the disease.

  3. Long-term efficacy and side effects of low-dose tacrolimus for the treatment of Myasthenia Gravis.

    Science.gov (United States)

    Tao, Xiaoyong; Wang, Wei; Jing, Feng; Wang, Zhongkui; Chen, Yuping; Wei, Dongning; Huang, Xusheng

    2017-02-01

    The study evaluated the efficacy of low-dose tacrolimus for treating Myasthenia Gravis (MG). Data were collected from 97 patients treated with low-dose tacrolimus from February 2011 to April 2015. Metabolic analysis was performed to determine more accurate tacrolimus dosing and patients were followed-up within clinic every 6 months for up to 4 years. The myasthenia gravis-specific activities of daily living scale was used to assess MG symptoms and their effects on patients' daily activities. All side effects and adverse reactions were thoroughly documented. At the end of follow-up, 6 patients were in complete stable remission, 17 patients were in pharmacological remission, 26 patients were in minimal manifestation status, 32 patients were improved, 2 patients were unchanged, 11 patients had worsening symptoms, and 3 patients died. Side effects were reported and/or observed in 24 patients, of which 7 patients experienced elevated blood glucose, 2 patients developed neoplasms, 3 patients developed gastrointestinal symptoms, 3 showed mild increases in aminotransferases, 3 patients suffered from bone marrow suppression, 2 patients suffered from skin rashes and erythema, and 1 patient required discontinuation of therapy. Transient renal insufficiency was also observed in 1 patient and 3 other patients had minor miscellaneous side effects. This study adds some knowledge on the efficacy and side effects of low-dose tacrolimus in the treatment of MG. Tacrolimus immunotherapy is a valid option for the management of MG, and can be gradually reduced in dose once symptoms are improved until complete withdrawal is achieved.

  4. A functional SNP in the regulatory region of the decay-accelerating factor gene associates with extraocular muscle pareses in myasthenia gravis

    OpenAIRE

    Heckmann, J M; Uwimpuhwe, H; Ballo, R; Kaur, M.; Bajic, V.B.; Prince, S.

    2009-01-01

    Complement activation in myasthenia gravis (MG) may damage muscle endplate and complement regulatory proteins such as decay-accelerating factor (DAF) or CD55 may be protective. We hypothesize that the increased prevalence of severe extraocular muscle (EOM) dysfunction among African MG subjects reported earlier may result from altered DAF expression. To test this hypothesis, we screened the DAF gene sequences relevant to the classical complement pathway and found an association between myasthe...

  5. Misdiagnosis of myasthenia gravis:a case report%重症肌无力误诊一例

    Institute of Scientific and Technical Information of China (English)

    葛同军; 周涌涛

    2016-01-01

    重症肌无力是一种神经-肌肉接头传递功能障碍性疾病,主要临床特征为受累骨骼肌易疲劳,休息后减轻,晨轻暮重。该病多隐袭起病,眼外肌最先受累。该文报道1例胸腺瘤所致的重症肌无力患者,其虽有眼外肌最先受累的表现,但为急性起病,且有不洁饮食史,无晨轻暮重现象,故曾被误诊为吉兰-巴雷综合征、肉毒杆菌中毒。经检测,其乙酰胆碱受体抗体阳性,重频神经电刺激低频电刺激波幅有明显递减现象,高频未见递增递减现象,新斯的明试验阳性,胸腺 CT 提示为胸腺瘤。其后于胸腔镜下行胸腺瘤摘除术,术后病理学检查示 B3型胸腺瘤,最终明确诊断为胸腺瘤所致的重症肌无力。术后患者恢复良好。因此,对于出现周围神经特别是颅神经支配区受累症状的患者,应考虑到肌肉疾病、神经-肌肉接头疾病的可能。%Myasthenia gravis is a disease of neuro-muscular dysfunction.Main clinical characteristics include the fatigue of affected skeletal muscle,which can be relieved after resting.The symptom is slight in the morning and becomes aggravated in the evening.The onset of myasthenia gravis is elusive and extraocular mus-cle is the predisposing affected muscle.Here we reported one case of myasthenia gravis caused by thymoma. Although extraocular muscular involvement was the initial presentation,the patient presented with acute episode and had a history of eating contaminated food,whereas had no phenomenon of slight symptom in the morning and severe in the evening.The patient was misdiagnosed as Guillain-Barrésyndrome and botulism infection. The acetylcholine receptor antibody was detected positive.The low-frequency rather electrical stimulation am-plitude of heavy-frequency nerve electrical stimulation was declined,whereas no decrease was detected in high-frequency electrical stimulation amplitude.Neostigmine test yielded positive

  6. Confiabilidade do teste da caminhada de seis minutos em pacientes com miastenia gravis generalizada Reliability of the six-minute walk test in patients with generalized myasthenia gravis

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    Vanessa Regiane Resqueti

    2009-09-01

    Full Text Available Este estudo objetivou determinar a confiabilidade do teste da caminhada de seis minutos (TC6M como um teste de capacidade funcional em pacientes com miastenia gravis generalizada (MG. Foram selecionados 11 pacientes com MG - 5 homens, 6 mulheres - com idade de 55±9 anos, avaliados inicialmente quanto à função fulmonar, que se submeteram a três TC6M em dias diferentes. Durante e/ou após cada teste foram medidas freqüência cardíaca e saturação de oxigênio (por oxímetro portátil, sensação de dispnéia (pela escala de Borg e distância percorrida. Nos três testes as distâncias percorridas foram 498 m, 517 m e 520 m (respectivamente 99%, 103% e 104% do valor predito. Em média, a freqüência cardíaca, dispnéia e saturação de oxigênio mostraram comportamento constante nos três testes. Foram encontradas alta confiabilidade relativa, com coeficiente de correlação interclasse maior que 0,90 entre os testes (TC6M1-TC6M2, 0,960; TC6M1-TC6M3, 0,945; e TC6M2-TC6M3, 0,970 e confiabilidade absoluta de 4%, 3,5% e 4,8%, com reprodutibilidade de 11%, 9,8% e 13,4%, respectivamente para o primeiro, segundo e terceiro testes. Os limites superiores e inferiores de concordância e o valor médio das médias das diferenças (bias calculados pelo teste de Bland-Altman mostraram-se clinicamente aceitáveis. Conclui-se que o TC6M se mostrou seguro, confiável e reprodutível, podendo ser aplicado para avaliação e seguimento da tolerância ao exercício em pacientes com MG generalizada.The purpose of this study was to assess the reliability of the six minutes walking test (6MWT as a functional capacity test for patients with generalized myasthenia gravis (MG. Eleven patients with generalized MG (5 men, six women, aged 55±9 years, were first assessed as to pulmonary function and then submitted to three 6MWT in different days. Heart rate and oxygen saturation were measured (by means of portable oxymeter during, and dyspnea (by the Borg scale and

  7. CLINICAl AND IMMUNOPATHOLOGICAL.OGICAI. STUDY ON THE THYMUS IN MYASTHENIA GRAVIS

    Institute of Scientific and Technical Information of China (English)

    Lai-Hui Tu; Ren-Qin; Tao Wu

    2000-01-01

    Objective Directing towards the thymus and emphasizing mainly on the clinical and immunopathofogical study to probe its actior of the diagnosis,treatmeant and pathogcnesis cfmyasthema gravis(MG). Results tnd Dicusslons I .Diagnosis of MG with thymus pathologic lesions: I.MG with thymus hyetplasta It was shown by midstemum. CT scanning Germinal center arises from B ceil was determined by immunohistologic stain(Tol 5 marked); 2MG with thymoma It usualy occures at midaged man and apt to relapses on myasthenic crises. Positive rate of serum CAE-Ab(anti-citric acid extract antibody of human skeletal musie) was used tc find small thymoma early Rabbit Anti-serum against human CAE was labeled to determine thymoma AgNOR(argyrophilic stain of nuclear organizer region)was used to differentiate bemgn and maligant thymoma .As Muller-Hermlink' s classfication.thymoma of cortical type occurred more common and reliable to infiltration Ⅱ Treatment of MG with thymus pathoiogic lestons Except for symptomatic treatment with anti-acytlecholine drugs.MPSS or CTX intravenous perfusion had effect to relieve the myasthenic cnsis Using thymus radiation therapy to 134 cases, the complete remission and remarkable improvement rates were 66.5%.Rhe long-term effect followed up was rather stable. Using percutanous paracentesis into thymus gland for intervention treatment to 13 cases, all of them showed effect within one week.10 cases followed up more than 3 years,9 cases were complete remission or remarkable improvement Thymectmy is still a basic therapy for MG.In 102 cases with thymectomy,the complete remission and remarkable improvement rates were 59.8%,mortanty was 176%.Continual observation of CAE-Ab could contribute to determine the pateint' s prognosis,9 patients in severe condition followed up within 3 years,their serum CAE-Ab antibody titers persisted at high lever,3 Of them died,but 7 patients followed up within I year,those of their serum antibodies decreased quickly,5 cases of them

  8. Resultado da timectomia ampliada no tratamento de pacientes com Miastenia gravis Extended thymectomy for treating patients with Myasthenia gravis (MG

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    Raul Lopes Ruiz Jr

    2004-04-01

    Full Text Available INTRODUÇÃO: Diversas variações da timectomia podem ser realizadas, dentre elas a transesternal ampliada. A literatura sugere que, quanto mais extenso o procedimento para ressecção da glândula e tecidos do mediastino anterior, melhores os resultados e prognóstico. OBJETIVO: Avaliar retrospectivamente a resposta à timectomia ampliada em portadores de Miastenia gravis. MÉTODO: Foram avaliados 46 portadores de Miastenia gravis, submetidos à plasmaferese pré-operatória e à timectomia ampliada, entre agosto de 1992 e janeiro de 2003, divididos em três grupos, segundo o tempo decorrido desde o início dos sintomas: menor que 12 meses, 13 a 24 meses e maior que 25 meses. RESULTADOS: Trinta e um pacientes eram do sexo feminino e 15 do masculino. A média de idade foi de 30 anos. O tempo médio de evolução da doença foi de 26,3 meses. O acompanhamento ambulatorial pós-operatório foi em média de 26,6 meses. Quanto ao grau de resposta à timectomia, 89% dos pacientes tiveram boa resposta, sendo que 50% apresentaram remissão completa. Ocorreu um óbito nesta série. O exame anatomopatológico demonstrou que a hiperplasia tímica foi o achado mais freqüente. Apenas 3 pacientes (6,5% apresentaram timomas benignos. Em 5 pacientes (10,8% encontramos tecido tímico extraglandular: na gordura peritímica em 2 deles, na gordura pericárdica em 1, junto ao nervo frênico esquerdo em outro e na janela aorto-pulmonar em outro. CONCLUSÃO: A timectomia ampliada para tratamento da Miastenia gravis mostrou-se segura, eficiente, e apresentou alta porcentagem de remissão completa. Houve a detecção de tecido tímico extraglandular em alguns pacientes. Tão logo seja feito o diagnóstico, está indicada como terapêutica associada à plasmaferese pré-operatória e à medicamentosa, independentemente da idade, patologia tímica, e início dos sintomas.BACKGROUND: Extended thymectomy is one of several types of thymectomy. Literature suggests that the

  9. Perioperative and long-term outcome of thymectomy for myasthenia gravis: comparison of surgical approaches and prognostic analysis

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    LIU Cheng-wu; LUO Meng; MEI Jian-dong; ZHU Yun-ke; PU Qiang; MA Lin; CHE Guo-wei

    2013-01-01

    Background Thymectomy is an established treatment for myasthenia gravis (MG),and video-assisted thoracoscopic surgery (VATS) thymectomy has become an acceptable surgical procedure.This study aimed to compare the results of VATS thymectomy and open thymectomy and to identify the prognostic factors after thymectomy.Methods The clinical data of 187 consecutive thymectomies performed between July 2000 and December 2009 were retrospectively reviewed; 75 open thymectomies and 112 VATS thymectomies.Clinical efficacy and variables influencing outcome were assessed by Kaplan-Meier survival curves and Cox proportional hazards regression analysis.Results The operative blood loss in the VATS group was significantly less than that in the open group ((62.14±55.43)ml vs.(137.87±165.25) ml,P <0.05).The postoperative crisis rate increased with the severity of preoperative MG and the prescription dose of anticholinesterase.Complete follow-up information of patients more than 12 months after the thymectomy was obtained on 151 cases,89 cases from the VATS group and 62 cases from the open group,with a mean follow-up period of 59.3 months,range from 12 to 117 months.Complete stable remission (CSR) was the end point for evaluation of the treatment results.The overall five-year CSR rate was 57.5% Two good prognostic factors were identified; preoperative prescription of anticholinesterase alone (P=0.035) and non-thymomatous MG (P=0.003).The five-year CSR rate of the ocular type of MG reached a high level of 67.4%.Conclusions Thymectomy can achieve good long-term CSR in MG,and VATS is an ideal alternative method.High-dose prescription of anticholinesterase and the advanced stage by Myasthenia Gravis Foundation of America (MGFA) classification have higher risks of postoperative crisis.Preoperative prescription of anticholinesterase alone and non-thymomatous MG are good prognostic factors.Thymectomy should also be considered for the ocular type of MG.

  10. Increased expression of Toll-like receptors 7 and 9 in myasthenia gravis thymus characterized by active Epstein-Barr virus infection.

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    Cavalcante, Paola; Galbardi, Barbara; Franzi, Sara; Marcuzzo, Stefania; Barzago, Claudia; Bonanno, Silvia; Camera, Giorgia; Maggi, Lorenzo; Kapetis, Dimos; Andreetta, Francesca; Biasiucci, Amelia; Motta, Teresio; Giardina, Carmelo; Antozzi, Carlo; Baggi, Fulvio; Mantegazza, Renato; Bernasconi, Pia

    2016-04-01

    Considerable data implicate the thymus as the main site of autosensitization to the acetylcholine receptor in myasthenia gravis (MG), a B-cell-mediated autoimmune disease affecting the neuromuscular junction. We recently demonstrated an active Epstein-Barr virus (EBV) infection in the thymus of MG patients, suggesting that EBV might contribute to the onset or maintenance of the autoimmune response within MG thymus, because of its ability to activate and immortalize autoreactive B cells. EBV has been reported to elicit and modulate Toll-like receptor (TLR) 7- and TLR9-mediated innate immune responses, which are known to favor B-cell dysfunction and autoimmunity. Aim of this study was to investigate whether EBV infection is associated with altered expression of TLR7 and TLR9 in MG thymus. By real-time PCR, we found that TLR7 and TLR9 mRNA levels were significantly higher in EBV-positive MG compared to EBV-negative normal thymuses. By confocal microscopy, high expression levels of TLR7 and TLR9 proteins were observed in B cells and plasma cells of MG thymic germinal centers (GCs) and lymphoid infiltrates, where the two receptors co-localized with EBV antigens. An increased frequency of Ki67-positive proliferating B cells was found in MG thymuses, where we also detected proliferating cells expressing TLR7, TLR9 and EBV antigens, thus supporting the idea that EBV-associated TLR7/9 signaling may promote abnormal B-cell activation and proliferation. Along with B cells and plasma cells, thymic epithelium, plasmacytoid dendritic cells and macrophages exhibited enhanced TLR7 and TLR9 expression in MG thymus; TLR7 was also increased in thymic myeloid dendritic cells and its transcriptional levels positively correlated with those of interferon (IFN)-β. We suggested that TLR7/9 signaling may be involved in antiviral type I IFN production and long-term inflammation in EBV-infected MG thymuses. Our overall findings indicate that EBV-driven TLR7- and TLR9-mediated innate immune

  11. Dandy-Walker syndrome in adult mimicking myasthenia gravis Síndrome de Dandy-Walker em adulto simulando miastenia gravis

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    Juliana Cardoso

    2007-03-01

    Full Text Available The Dandy-Walker syndrome (DWS is a rare posterior fossa malformation. The DWS can occur associated with other brain or systemic malformations, but ocular abnormalities in this disease are rare and clinical findings mimicking myasthenia gravis have not been described to date. We report a 23-year-old woman who presented mild limitation of the ocular movements with progressive palpebral ptosis, which changed in intensity during the day. The investigation showed negative anti-acetylcholine receptor antibody, repetitive nerve stimulation and "Tensilon test", but the brain magnetic resonance image reveals DWS with hydrocephalus associated with calosal dysgenesis. The characteristic of disease, clinical manifestations and pathologic features, specially the clinical evaluation of ocular abnormalities in suspicion of DWS, including the MG in differential diagnosis are discussed.A síndrome de Dandy-Walker (DWS é uma rara malformação da fossa posterior que pode ocorrer associada com outras malformações cerebrais ou sistêmicas. As alterações oculares são raras e as manifestações clínicas, simulando miastenia gravis (MG, não foram descritas até o momento. Descrevemos uma mulher de 23 anos apresentando discreta limitação da movimentação ocular com progressiva ptose palpebral que mudava de intensidade durante o dia. A investigação mostrou negativos o anticorpo anti-receptor de acetilcolina, a estimulação nervosa repetitiva e o "teste do Tensilon", porém a ressonância magnética de crânio revelou DWS com hidrocefalia associada à disgenesia de corpo caloso. As características da doença, manifestações clínicas e patológicas, especialmente a avaliação clínica de anormalidade ocular na suspeita de DWS serão discutidas, incluindo a MG no diagnóstico diferencial.

  12. Biologics and other novel approaches as new therapeutic options in myasthenia gravis: a view to the future.

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    Dalakas, Marinos C

    2012-12-01

    Myasthenia gravis (MG) is caused by complement-fixing antibodies against the acetylcholine receptors (AChR). Regulatory T cells (T(reg) cells), Th17 cells, and recognition of AChR epitopes by CD4(+) T cells are fundamental. Novel biological agents now in the offing, offer the potential for specific treatment options in MG by targeting the following: (1) T cell intracellular signaling pathways, costimulation, and transduction molecules; (2) B cells, against CD20 molecules, or the B cell trophic factors BAFF and APRIL; (3) complement, against C5 that intercepts the formation of MAC; (4) cytokines and cytokine receptors targeting interleukin (IL)-6, IL-17, and the Janus tyrosine kinases (JAK)1 and JAK3; and (5) cellular adhesion and T cell migration molecules. Reengineering of pathogenic antibodies (i.e., molecular decoys) by constructing recombinant antibodies that block the complement binding of the pathogenic AChR antibodies is an additional approach. The promising therapeutic profile of these agents should be weighted against excessive cost and rare complications necessitating the need for controlled trials to secure efficacy and balance benefit against risks.

  13. Clinical profile of patients with myasthenia gravis followed at the University Hospital, Federal University of Minas Gerais

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    Aline Mansueto Mourão

    2015-04-01

    Full Text Available Summary Objective: to determine the clinical profile of patients with myasthenia gravis (MG; followed at the Neuromuscular Diseases Clinic of the University Hospital, Federal University of Minas Gerais, Brazil, and to compare it with other Brazilian case series. Methods: sociodemographic and clinical data were collected from patients, and a systematic literature review performed, focusing on national studies on the clinical profile of MG patients. Results: sixty nine patients were enrolled in the study. Fifty five (91% subjects were female and the mean age (SD was 37.6 (±11.4 years. The mean disease duration was 14.1 years. Regarding treatment, prednisone was the most used strategy (64%, followed by the use of azathioprine (43%. There was no difference between thymectomized (42 and non-thymectomized (27 patients regarding disease severity and medication use. Conclusion: clinical and socio-demographic features of this MG sample from a University-based clinic resemble those reported in other Brazilian series and in the international literature.

  14. [Administration of sugammadex to a patient with myasthenia gravis with fade of the train-of-four ratio].

    Science.gov (United States)

    Sugawara, Ami; Sasakawa, Tomoki; Hasegawa, Naoyoshi; Takahata, Osamu; Iwasaki, Hiroshi

    2011-09-01

    A 50-year-old man (weight 87 kg, height 171 cm) with myasthenia gravis (MG) was scheduled for extended thymectomy under general anesthesia. His preanesthetic train-of-four ratio (T4/T1) was 59%. The first twitch of the train-of-four (T1) was 130% after calibration. We administered rocuronium 10 mg (0.11 mg x kg(-1)) for tracheal intubation. Maximal suppression was achieved in 50 seconds. During the operation, we did continuous infusion of rocuronium to maintain T1 at 10%. We discontinued rocuronium infusion before the end of surgery. In patients with MG, deep levels of neuromuscular block can be achieved with less rocuronium. We hypothesized that the requirement of sugammadex in a patient with MG is less than that in normal patients. Therefore, we administered 0.5 mg x kg(-1) of sugammadex. After 5 min, T4/T1 had reached 54%, but T1 had not reached the control value; therefore, we administered additional 1.5 m x kg(-1) of sugammadex. Subsequently, T1 reached 120%. Patients with MG with fade on T4/T1 require a full dose of sugammadex, identical to the dose administered to normal patients.

  15. Preoperative Anxiety in Patients With Myasthenia Gravis and Risk for Myasthenic Crisis After Extended Transsternal Thymectomy: A CONSORT Study.

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    Zou, Jianyong; Su, Chunhua; Lun, Xueping; Liu, Weibing; Yang, Weiling; Zhong, Beilong; Zhu, Haoshuai; Lei, Yiyan; Luo, Honghe; Chen, Zhenguang

    2016-03-01

    A thymectomy can ameliorate the symptoms of myasthenia gravis (MG) and prevent the progression of ocular MG (OMG) to generalized MG (GMG). However, postoperative myasthenic crisis (POMC) is a serious post-thymectomy complication. Preoperative anxiety (POA) is common but typically neglected in MG patients. The association of POA with POMC has not yet been examined.From June 2007 to December 2013, 541 cases of MG were admitted to the First Affiliated Hospital of Sun Yat-sen University (Guangzhou, China). All cases underwent extended transsternal thymectomy (ETT). The clinical and pathological characteristics of these patients, including POA and POMC, were analyzed.A total of 179 patients experienced POA and 67 patients experienced POMC. Patients with POA were more likely to have POMC, a thymoma, and an ectopic thymus. Univariate analysis showed that POMC correlated with POA, presence of an ectopic thymus, dose of pyridostigmine bromide (PYR), presence of a thymoma, MGFA stage, preoperative myasthenic crisis, and postoperative pneumonia. Multivariate logistic regression analysis showed that the independent risk factors for POMC were POA, preoperative myasthenic crisis, higher dose of PYR, and postoperative pneumonia.Our results suggest that clinicians should consider the risk factors for POMC-especially preoperative anxiety-before performing a thymectomy in patients with MG.

  16. Brazilian cross-cultural translation and adaptation of the "Questionnaire of Life Quality Specific for Myasthenia Gravis - 15 items"

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    Aline Mansueto Mourao

    2013-12-01

    Full Text Available Objective To translate and to perform the cross-cultural adaptation of the “Questionnaire of Life Quality Specific for Myasthenia Gravis - 15 items” (MG-QOL15. Method The original English version of the questionnaire was translated into Portuguese. This version was revised and translated back into English. Later, both English versions were compared and the divergences were corrected in the Portuguese text. At a second stage, ten patients with MG followed at the Neuromuscular Diseases Clinic from the University Hospital, Universidade Federal de Minas Gerais answered the questionnaire. The authors analyzed the difficulties and misunderstandings in the application of the questionnaire. Results The questions 8, 13 and 15 were considered difficult to understand and were modified in the final Portuguese version. Most patients (70% had a total score above 25, and the statements 3, 8 and 9 showed the highest scores. Conclusion The Brazilian version of the questionnaire MG-QOL15 seems to be a promising tool for the assessment of Brazilian patients with MG.

  17. Both Treg cells and Tconv cells are defective in the Myasthenia gravis thymus: roles of IL-17 and TNF-α.

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    Gradolatto, Angeline; Nazzal, Dani; Truffault, Frédérique; Bismuth, Jacky; Fadel, Elie; Foti, Maria; Berrih-Aknin, Sonia

    2014-08-01

    Myasthenia gravis (MG) is an autoimmune disease in which the thymus frequently presents follicular hyperplasia and signs of inflammation and T cells display a defect in suppressive regulation. Defects in a suppressive assay can indicate either the defective function of Treg cells or the resistance of Tconv cells to suppression by Treg cells. The aim of this study was to determine which cells were responsible for this defect and to address the mechanisms involved. We first performed cross-experiment studies using purified thymic Treg cells and Tconv cells from controls (CTRL) and MG patients. We confirmed that MG Treg cells were defective in suppressing CTRL Tconv proliferation, and we demonstrated for the first time that MG Tconv cells were resistant to Treg cell suppression. The activation of MG Tconv cells triggered a lower upregulation of FoxP3 and a higher upregulation of CD4 and CD25 than CTRL cells. To investigate the factors that could explain these differences, we analyzed the transcriptomes of purified thymic Treg and Tconv cells from MG patients in comparison to CTRL cells. Many of the pathways revealed by this analysis are involved in other autoimmune diseases, and T cells from MG patients exhibit a Th1/Th17/Tfh signature. An increase in IL-17-related genes was only observed in Treg cells, while increases in IFN-γ, IL-21, and TNF-α were observed in both Treg and Tconv cells. These results were confirmed by PCR studies. In addition, the role of TNF-α in the defect in Tconv cells from MG patients was further confirmed by functional studies. Altogether, our results indicate that the immunoregulatory defects observed in MG patients are caused by both Treg cell and Tconv cell impairment and involve several pro-inflammatory cytokines, with TNF-α playing a key role in this process. The chronic inflammation present in the thymus of MG patients could provide an explanation for the escape of thymic T cells from regulation in the MG thymus.

  18. Treatment of cervical dystonia with botulinum toxin in a patient with myasthenia gravis Tratamento de distonia cervical com toxina botulínica em uma paciente com miastenia gravis

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    MARCIA RUBIA R. GONÇALVES

    1999-09-01

    Full Text Available We report the case of a 49-year-old woman who has the rare combination of myasthenia gravis and cervical dystonia. She was treated with botulinum toxin type A with good response and no evidence of deterioration of the myasthenic symptoms. We therefore conclude that it is possible to use botulinum toxin in the presence of defective neuromuscular transmission.Relatamos o caso de uma mulher de 49 anos com rara combinação de miastenia gravis e distonia cervical tratada com toxina botulínica tipo A, apresentando boa resposta e nenhuma evidência de piora do quadro miastênico. A partir dessas observações concluimos que é possível o uso de toxina botulínica na presença de doença da transmissão neuromuscular.

  19. 重症肌无力的围术期处理%Treatment of Myasthenia Gravis before and after the Operation

    Institute of Scientific and Technical Information of China (English)

    刘明林

    2003-01-01

    @@ 重症肌无力(Myasthenia gravis MG)行胸腺扩大切除时,由于本病的特点和复杂性,国手术期并发症多,甚至还可出现肌无力危象,如果处理不当可致病人死亡.199年2月~2003年2月,我们手术治瘵MG46例,在围术期处理方面做了积极的改进,疗效良好,无并发症发生,报告如下.

  20. 重症肌无力患者规范化护理的应用%Application of Standard of Care in Patients with Myasthenia Gravis

    Institute of Scientific and Technical Information of China (English)

    廖晓琴

    2014-01-01

    目的探讨重症肌无力患者规范化护理的效果。方法随机将我院40例重症肌无力患者分为对照组和观察组各20例,对照组采用常规护理,观察组采用规范的护理方法,比较两组患者脱机时间,危象的发生率,并发症的发生率。结果患者脱机时间明显短于对照组(<0.05),危象的发生率,并发症发生率低于对照组(<0.05)。结论应用规范重症肌无力患者的护理可有效提高临床疗效,重症肌无力危象的抑制和并发症的发生率,提高患者的生活质量。%Objective To investigate the ef ect of the standardized nursing in patients with myasthenia gravis. Methods To divide randomly 40 cases of myasthenia gravis patients in our hospital as control group and observation group with 20 cases in each group, the control group received routine nursing care, observation group adopted standardized nursing method, compared two groups of patients, the incidence rate of of -line time crisis, the machine rate and incidence of complications. Results Patients with of line time was shorter than the control group ( <0.05), the incidence of the crisis, and complication rates were lower than the control group ( < 0.05). Conclusion To application specification in myasthenia gravis patients nursing ef ectively enhance the clinical curative ef ect, inhibition of myasthenia gravis crisis and the incidence of complications, improve the quality of life of patients.

  1. Distinguishing Myasthenia Exacerbation from Severe Preeclampsia: A Diagnostic and Therapeutic Challenge

    Science.gov (United States)

    Sikka, Pooja; Aggarwal, Neelam; Suri, Vanita; Bhagat, Hement

    2015-01-01

    Myasthenia gravis is an acquired, autoimmune neuromuscular disorder characterized by voluntary muscle weakness. Pregnant patients may have disease exacerbation, respiratory failure, crisis, adverse drug reaction, surprisingly enough remission at any trimester or postnatal period. Concurrence of myasthenia gravis with severe preeclampsia is a dreadful condition raising diagnostic and management issues. We hereby discuss a case of myasthenic woman who developed severe preeclampsia during pregnancy and presented in last trimester with clinical features mimicking signs of impending eclampsia. Keeping in mind the history of myasthenia gravis, urgent neurology review taken and diagnosis of myasthenic exacerbation was entertained. She responded well to injection neostigmine and in this way inadvertent use of magnesium sulphate was avoided. PMID:26436003

  2. Association of myasthenia gravis with polymorphisms in the gene of histamine N-methyltransferase

    DEFF Research Database (Denmark)

    Kellermayer, Blanka; Polgar, Noemi; Pal, Jozsef

    2013-01-01

    Histamine N-methyltransferase (HNMT) is the main metabolizing enzyme of histamine. Histamine modulates immune responses and plays a role in the pathogenesis of autoimmune disorders.......Histamine N-methyltransferase (HNMT) is the main metabolizing enzyme of histamine. Histamine modulates immune responses and plays a role in the pathogenesis of autoimmune disorders....

  3. Immunotherapy of Myasthenia Gravis%重症肌无力的免疫治疗

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    廖建湘; 李成荣

    2005-01-01

    重症肌无力(myasthenia gravis,MG)是一种T细胞依赖、抗体介导的自身免疫病。患者血清中抗乙酰胆碱受体(AChR)抗体等自身抗体滴度升高;MG免疫治疗目前主要有两大类,一是对免疫系统有广泛作用的免疫疗法如皮质激素等;二是针对MG发病有关的特异性免疫细胞或细胞因子等,而对全身其他免疫功能影响较小的免疫治疗方法。

  4. Thymic Germinal Centers and Corticosteroids in Myasthenia Gravis: an Immunopathological Study in 1035 Cases and a Critical Review.

    Science.gov (United States)

    Truffault, Frédérique; de Montpreville, Vincent; Eymard, Bruno; Sharshar, Tarek; Le Panse, Rozen; Berrih-Aknin, Sonia

    2017-02-01

    The most common form of Myasthenia gravis (MG) is due to anti-acetylcholine receptor (AChR) antibodies and is frequently associated with thymic pathology. In this review, we discuss the immunopathological characteristics and molecular mechanisms of thymic follicular hyperplasia, the effects of corticosteroids on this thymic pathology, and the role of thymic epithelial cells (TEC), a key player in the inflammatory thymic mechanisms. This review is based not only on the literature data but also on thymic transcriptome results and analyses of pathological and immunological correlations in a vast cohort of 1035 MG patients without thymoma. We show that among patients presenting a thymic hyperplasia with germinal centers (GC), 80 % are females, indicating that thymic follicular hyperplasia is mainly a disease of women. The presence of anti-AChR antibodies is correlated with the degree of follicular hyperplasia, suggesting that the thymus is a source of anti-AChR antibodies. The degree of hyperplasia is not dependent upon the time from the onset, implying that either the antigen is chronically expressed and/or that the mechanisms of the resolution of the GC are not efficiently controlled. Glucocorticoids, a conventional therapy in MG, induce a significant reduction in the GC number, together with changes in the expression of chemokines and angiogenesis. These changes are likely related to the acetylation molecular process, overrepresented in corticosteroid-treated patients, and essential for gene regulation. Altogether, based on the pathological and molecular thymic abnormalities found in MG patients, this review provides some explanations for the benefit of thymectomy in early-onset MG patients.

  5. Effective treatment of a 13-year-old boy with steroid-dependent ocular myasthenia gravis using tacrolimus.

    Science.gov (United States)

    Mori, Tatsuo; Mori, Kenji; Suzue, Masashi; Ito, Hiromichi; Kagami, Shoji

    2013-05-01

    Over the past several years, tacrolimus has attracted attention as a new therapeutic drug for myasthenia gravis (MG), but few reports have considered its use for MG in pediatric patients, and most of these have focused on severe systemic MG. In this case report, we used tacrolimus to successfully treat a 13-year-old boy with ocular MG who had suffered from severe steroid complications, including a failure of thrive and osteoporosis. He first showed symptoms of ocular MG at age 2 years 3 months. At age 13 years, he was receiving PSL (3.75 mg/day), but the symptoms of ocular MG recurred. We increased the dosage of oral PSL up to 30 mg/day, and three courses of mPSL pulse therapy were applied, but these therapies had only limited effect, and his symptoms worsened. Tacrolimus was started at 0.4 mg/day (0.011 mg/kg/day), and every 2 weeks the dose was gradually increased by 0.2 mg/day. His symptoms of MG began to improve 3 weeks after the initial administration of tacrolimus. Approximately 3 months after the start of tacrolimus administration, PSL was discontinued. Currently, at 1 year and 4 months after the start of tacrolimus administration, while slight ptosis is observed in the evening, it does not influence his daily life, and his condition remains comparable to that when he stopped taking PSL. No adverse effects of tacrolimus have been recognized. In pediatric patients with steroid-dependent ocular MG without thymectomy, tacrolimus may be a safe and effective alternative to steroid and thymectomy.

  6. Establishment of a simple predictive score for general myasthenia gravis in patients with ocular myasthe- nia gravis%预测眼肌型重症肌无力预后的临床评分方法

    Institute of Scientific and Technical Information of China (English)

    王莉莉; 谢琰臣; 贺茂林

    2014-01-01

    目的:建立预测眼肌型起病的重症肌无力患者(ocular myasthenia gravis, OMG)预后的临床评分方法。方法回顾性分析113例眼肌型起病的重症肌无力(myasthenia gravis, MG)患者临床资料,根据起病2年时病情进展情况将患者分为单纯眼肌型组(ocular myasthenia gravis, OMG)和进展为全身型组(general myasthenia gravis, GMG),应用Logistic回归分析筛选影响OMG预后的影响因素,并根据影响因素的回归系数建立量化的预测OMG预后的临床评分方法。受试者工作曲线(receiver operator characteristic curve, ROC)验证评分方法的有效性并确定诊断界点。结果Logistic回归分析显示胸腺瘤(OR=4.005,95%CI 1.493~10.742)、甲状腺功能(OR=6.640,95%CI 2.553~17.25)是影响OMG预后的危险因素,评分方法总分数值为3分。ROC曲线下面积为76.6%,界点为1分,患者大于该分数值有进展为GMG的风险。界点为1分时该评分方法预测OMG预后的灵敏度为1,特异度为0.455,阳性预测值为73.9%,阴性预测值为59.1%。结论该评分方法在预测眼肌型MG预后方面切实有效,量化评分1分以上OMG患者2年内有进展为GMG的可能。%Objective To establish a simple scale system to predict early risk of general myasthenia gravis (GMG) in patients with ocular myasthenia gravis (OMG). Methods We retrospectively analyzed clinical data of the patients with general myasthenia gravis with a definite ocular myasthenia gravis origin (n=113). The patients with OMG were divided into two groups (OMG and GMG) according to the disease progression within two years after onset of OMG. Logistic re-gression analysis was used to screen the influencing factors.The score was developed according to regression coefficients. The receiver operating characteristic (ROC) curve was first plotted. The ROC was then used to determine sensitivities and specificities of prediction. Results Logistic regression analysis

  7. [Premature birth in patient with cervix incompetence and history of myasthenia gravis].

    Science.gov (United States)

    Fuentealba, Maximiliano; Troncoso, Miguel; Vallejos, Joaquin; Ponce, Sebastian; Villablanca, Nelson; Melita, Pablo

    2013-09-01

    Cervical incompetence it's a dilatation of the cervix during the third trimester of pregnancy that ends with the interruption of it. The incidence in Chile is about 0.1-2% of the total pregnancies and it's one of the causes of preterm birth. A 34 years old pregnant patient. Timectomized at age 18 to treat her miastenia gravis, previously trated with medication, had 4 previous preterm labours all of them under 25 weeks and vaginal births. All fetuses died postpartum. A cerclage was made during the third, fourth and fifth pregnancies. She didn't present hypertension during the gestation and no cervical diameter under 15mm. Since the fourth gestation the following tests are taken: Antifosfolipidic antibodies, APTT,PT. All the results are either normal or negative. Microbial cultures were negative. No amniocentesis was made. A McDonald cervical cerclage was made during pregnancies number 3, 4 and 5 on the 16th week to delay the labor. Also oral micronized progesterone, on a 400mg/24 hours dosis, was administered to avoid preterm birth. On the 24th week the pharmacological treatment started including Intramuscular Betamethasone, 12 mg/24 hours (2 doses), to induce lung maturity on the fetus. It is thought that the administration of progesterone could have improved the situation of the patient, because it acts as a labour repressants. The use of cerclage could have helped, but the factors that may influence the effectiveness of this method are unknown. Perhaps there is some immunologic factor associated with the miastenia gravis that alters the normal course of pregnancy.

  8. Evaluation of repetitive stimulation test (RST in 30 patients with Myasthenia Gravis, who were previously confirmed by clinical sign and tensilon test 1996-99

    Directory of Open Access Journals (Sweden)

    "Ghabaee M

    2001-07-01

    Full Text Available est (RST is the most commonly used electrodiagnostic test to asses the defect of neuromuscular transmission, which is reported to be positive in the diffuse and restricted ocular forms 60-95% and 14-50%, respectively. In a cross-sectional study, to determine the efficacy of repetitive stimulation test in myasthenia gravis, we evaluated the results in 30 cases who were hospitalized in Imam Khomeini Hospital during 1996-1999. Patients were first selected clinically and then confirmed by Tensilon test.Various clinical types including generalized and restricted ocular forms with different severity and duration were entered in this study. Considering the fact that the positiveness of the test is enhanced by assessment of more muscle groups, we evaluated decremental response in the facial, proximal and distal muscles of limbs. 90% of patients had the generalized form of the disease, whereas ocular myasthenia gravis was seen only in 10% of the cases. 74% of females and 73% of males showed positive response (overall: 73.3%. No significant association was found between the positive response, and age and sex. Peaks of incidences of the disease for the males were in fourth and sixth decades and for the females in thired decades

  9. Successful treatment on 1 case of myasthenia gravis pregnancy%成功救治妊娠合并重症肌无力患者1例

    Institute of Scientific and Technical Information of China (English)

    于启霞

    2014-01-01

    Myasthenia gravis and pregnancy is a rare disease,and lack of progress in the treatment of this disease.The disease easily leads to complications,and seriously threat to the life and health of pregnant women and fetus.In this paper we analysis the successful treatment in our hospital of 1 pregnancy with myasthenia gravis,in order to investigate the prevention and treatment of this disease.%妊娠合并重症肌无力是一种罕见疾病,目前尚且缺乏关于该病的治疗方法和治疗进展的介绍,并且该病容易引起并发症,严重威胁孕妇及胎儿的生命健康。本文对本院成功救治的1例妊娠合并重症肌无力患者的临床治疗情况进行分析,探讨该病的预防和处理方法。

  10. 锁骨骨折术后格林巴利综合征合并重症肌无力一例报道%Guillain Barré Syndrome Combined with Myasthenia Gravis after Operation for Clavicle Fracture:A Case Report

    Institute of Scientific and Technical Information of China (English)

    王成谋; 费松柏; 马祥铭; 徐蔚; 陈小霞

    2013-01-01

    Guillain Barre syndrome and myasthenia gravis are two different diseases. This paper reports a case of guillain Barre syndrome complicated by myasthenia gravis after operation for clavicle fracture and reviews pertinent literatures to analyze its reasons. Immune factor plays an important role in the occurrence of the two diseases. Surgical procedures induce peripheral nerve autoimmunity. Cross - reactive antibodies may be the cause of damages of peripheral nerve myelin proteins and neuro-muscular junction acetylcholinesterase receptor.%格林巴利综合征和重症肌无力是两种不同的疾病,本文报道1例锁骨骨折术后出现格林巴利综合征合并重症肌无力患者,并查阅相关文献分析其原因.免疫因素在两者的发病中起重要作用,外科手术操作诱发周围神经的自身免疫反应,交叉性反应抗体可能是周围神经髓磷脂蛋白和神经肌肉接头乙酰胆碱受体受损的原因.

  11. EXPRESSION AND SUBCELLULAR LOCALIZATION OF P9-ZFD PROTEIN IN PATIENTS WITH MYASTHENIA GRAVIS

    Institute of Scientific and Technical Information of China (English)

    Ming-shan Ren; Chuan-zhen Lu; Jian Qiao; Hui-min Ren; Ren Xu; Ren-bao Gan

    2004-01-01

    Objective To express and purify the protein coded by the TRAF-type zinc finger domain ofmyasthenia gravis (MG)-related gene P9 ( P9-ZFD ) and to prepare P9-ZFD antiserum for detecting expression and subcellular distribution of P9-ZFD protein in the skeletal muscles of patient with MG.Methods The cDNA encoding P9-ZFD was amplified by RT-PCR. The cloned P9-ZFD cDNA was ligated into pET-24a, and the P9-ZFD recombinant protein was induced via E. coli. BL21 (DE3) and purified by histidine affinity chromatography. P9-ZFD antiserum was prepared and its fiter and specificity were determined by ELISA and Western blot. Expression and subcellular distribution of P9-ZFD protein in the skeletal muscles of MG and control were studied.Results The molecular weight of purified P9-ZFD protein was about 30 kD. Its purity was more than 95%. Antiserum specific for P9-ZFD was excellent. P9-ZFD protein is fully confined to the cytoplasm membrane of skeletal muscle cell of MG, obvious immunostaining was absent in the A, I, and Z bands of cytoplasm and no immunoreactivity was observed in the skeletal muscle cell of control.Conclusion P9-ZFD protein is expressed as a cytoplasm membrane-bound protein and has obvious distribution difference in the skeletal muscle cells of patient with MG and normal control.

  12. The molecular anatomy of human Hsp60 and its similarity with that of bacterial orthologs and acetylcholine receptor reveal a potential pathogenetic role of anti-chaperonin immunity in myasthenia gravis.

    Science.gov (United States)

    Gammazza, Antonella Marino; Bucchieri, Fabio; Grimaldi, Luigi M E; Benigno, Arcangelo; de Macario, Everly Conway; Macario, Alberto J L; Zummo, Giovanni; Cappello, Francesco

    2012-08-01

    Heat-shock protein 60 (Hsp60) is ubiquitous and highly conserved being present in eukaryotes and prokaryotes, including pathogens. This chaperonin, although typically a mitochondrial protein, can also be found in other intracellular sites, extracellularly, and in circulation. Thus, it can signal the immune system and participate in the development of inflammation and immune reactions. Both phenomena can be elicited by human and foreign Hsp60 (e.g., bacterial GroEL), when released into the blood by infectious agents. Consequently, all these Hsp60 proteins become part of a complex autoimmune response characterized by multiple cross reactions because of their structural similarities. In this study, we demonstrate that Hsp60 proteins from humans and two common pathogens, Chlamydia trachomatis and Chlamydia pneumoniae, share various sequence segments of potentially highly immunogenic epitopes with acetylcholine receptor α1 subunit (AChRα1). The structural data indicate that AChRα1 antibodies, implicated in the pathogenesis of myasthenia gravis, could very well be elicited and/or maintained by self- and/or bacterial Hsp60.

  13. Differential Cytokine Changes in Patients with Myasthenia Gravis with Antibodies against AChR and MuSK.

    Directory of Open Access Journals (Sweden)

    Vuslat Yilmaz

    Full Text Available Neuromuscular transmission failure in myasthenia gravis (MG is most commonly elicited by autoantibodies (ab to the acetylcholine receptor or the muscle-specific kinase, constituting AChR-MG and MuSK-MG. It is controversial whether these MG subtypes arise through different T helper (Th 1, Th2 or Th17 polarized immune reactions and how these reactions are blunted by immunosuppression. To address these questions, plasma levels of cytokines related to various Th subtypes were determined in patients with AChR-MG, MuSK-MG and healthy controls (CON. Peripheral blood mononuclear cells (PBMC were activated in vitro by anti-CD3, and cytokines were quantified in supernatants. In purified blood CD4+ T cells, RNA of various cytokines, Th subtype specific transcription factors and the co-stimulatory molecule, CD40L, were quantified by qRT-PCR. Plasma levels of Th1, Th2 and Th17 related cytokines were overall not significantly different between MG subtypes and CON. By contrast, in vitro stimulated PBMC from MuSK-MG but not AChR-MG patients showed significantly increased secretion of the Th1, Th17 and T follicular helper cell related cytokines, IFN-γ, IL-17A and IL-21. Stimulated expression of IL-4, IL-6, IL-10 and IL-13 was not significantly different. At the RNA level, expression of CD40L by CD4+ T cells was reduced in both AChR-MG and MuSK-MG patients while expression of Th subset related cytokines and transcription factors were normal. Immunosuppression treatment had two effects: First, it reduced levels of IL12p40 in the plasma of AChR-MG and MuSK-MG patients, leaving other cytokine levels unchanged; second, it reduced spontaneous secretion of IFN-γ and increased secretion of IL-6 and IL-10 by cultured PBMC from AChR-MG, but not MuSK-MG patients. We conclude that Th1 and Th17 immune reactions play a role in MuSK-MG. Immunosuppression attenuates the Th1 response in AChR-MG and MuSK-MG, but otherwise modulates immune responses in AChR-MG and Mu

  14. 重症肌无力危象44例循证护理分析%Evidence-based nursing for patients with 44 myasthenia gravis crisis cases

    Institute of Scientific and Technical Information of China (English)

    乔娟; 王佳

    2015-01-01

    Objective: To investigate the influence of evidence-based nursing on complications and prognosis for patients with myasthenia gravis crisis. Methods:The research method of evidence - based medicine was used to the patients with myasthenia gravis crisis. Forty-four cases in intervention group were treated with evidence-based care,and another 38 patients in control group were just given routine nursing care. The incidence of complications and indicators of health economics were evaluated between the two groups. Results:With Evidence-based nursing,the incidences of pulmonary infection and affective disorder were lower than those in control group(P < 0. 05 and P < 0. 01). In addition,the length of stay and the hospitalization expenses were decreased in the intervention group. Conclusions:Evidence-based nursing can lower incidence of complications and gain better prognosis after myasthenia gravis crisis,which suggested that evidence-based nursing should be practiced on an early stage.%目的::分析循证护理对重症肌无力危象、并发症及预后的影响。方法:应用循证方法分析重症肌无力危象患者,确定循证的护理问题,寻找循证支持。干预组44例应用循证护理措施,对照组38例应用常规护理。比较2组并发症发生率及卫生经济学指标。结果:干预组患者呼吸道感染和情感障碍发生率均低于对照组(P <0.05和 P <0.01),住院时间、住院费用明显少于对照组(P <0.01)。结论:循证护理能减少重症肌无力危象并发症发生,对重症肌无力危象患者应早期应用循证护理措施。

  15. Corticosteroides no tratamento da miastenia grave. Estudo de 12 casos com revisão da literatura Corticosteroids in the management of myasthenia gravis

    Directory of Open Access Journals (Sweden)

    José Lamartine de Assis

    1975-03-01

    Full Text Available Foram estudados os resultados do tratamento da miastenia grave em 12 pacientes, sendo 10 com a forma generalizada severa, um com a forma generalizada moderada e um com miastenia acentuada associada a polimiosite. Sete pacientes foram previamente timectomizados e somente um apresentava timoma. Todos estavam em uso de doses elevadas de drogas anticolinesterásicas com resposta terapêutica pobre ou nula. Um paciente foi tratado com dexametaxona, outro com dexametazona seguida de prednisona e os restantes com prednisona, sempre em doses altas inicialmente (100 mg por dia ou em dias alternados e com redução gradativa ulterior até doses de manutenção. Os corticosteróides foram administrados sempre pela via oral, por períodos que variaram de duas semanas até dois anos e meio. Em conclusão, 75% de 12 pacientes com miastenia grave generalizada foram influenciados favoravelmente com este método terapêutico. Deste grupo apenas um apresentava timoma e não respondeu a qualquer tratamento. Outro paciente apresentava polimiosite associada e teve excelente resposta terapêutica. Dois miastênicos sem timoma tiveram a sintomatologia exacerbada durante o tratamento. Nenhum efeito colateral importante foi observado, mesmo nos pacientes tratados a longo prazo.The results of treatment of myasthenia gravis in 12 patients are reported. There were 10 cases with severe generalized form and two cases with moderate generalized form, one of which associated with polymyositis. Seven patients had prior thymectomy and one of them had a thymoma. All the patients were receiving anticholinesterase drugs with poor response or without any response. One patient received a short, intensive course of dexametazone, and other patient used prednisone after a more prolonged dexametazone course. The remaining patients received prednisone, always beginning with high (100 mg alternate day oral single-doses. This therapy has been maintained for a period thought to be sufficient

  16. Autoimmune Disease

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    2008391 Prognostic factors related to recurrence after extended thymectomy in patients with myasthenia gravis. MEN Lina(门丽娜), et al. Dept Neurol, 1st Affili Hosp, Sun Yat-sen Univ, Guangzhou 510080. Natl Med J China 2008;88(2):1446-1449. Objective To identify the recurrence-related factors in patients with myasthenia gravis (MG) after extended thymectomy.

  17. Associação familiar de miastenia grave e síndrome miotônica Familial association of myasthenia gravis and myotonic syndrome

    Directory of Open Access Journals (Sweden)

    Sylvio Saraiva

    1969-12-01

    Full Text Available É relatada uma associação familiar incomum, incidindo em mãe e filha, a primeira com síndrome miotônica e, a segunda, com miastenia grave, forma ocular. Um terceiro membro da família, irmão da paciente com miotonia, teria também manifestações dessa síndrome. São comentados aspectos clínico-laboratoriais dos casos estudados.An uncommon association of myasthenia gravis in a child and a myotonic syndrome in her mother is reported. A brother of the second patient probably had myotonic symptomatology. Clinical and laboratorial aspects are discussed.

  18. Effect of intravenous immunoglobulin in Guilain-Barre syndrome, myasthenia gravis and chronic idiopathic demyelinative polyneuropathy, A survey in Imam Khomeini Hospital

    Directory of Open Access Journals (Sweden)

    Qaffarpoor M

    1999-09-01

    Full Text Available With retrospective evaluation of 44 patients suffering from Guilan-Barre Syndrome (GBS, Chronic Idiopathic Demtyelinative Polyradiculoneuropathy (CIDP and Myasthenia Gravis (MG treated with intravenous immunoglobulin, we found following results: 1 Initial symptoms of improvement on forth or fifth days. 2 Maximum recovery for CIDP and MG were after 16-24 and 3-11 days, respectively. 3 No major complication, but mild side effects in 32% of patients. 4 In patients with GBS one grade improvement achieved after 8-30 days. 5 Intravenous immunoglobulin (IVIG plus plasmapheresis had no advantages over IVIG alone. 6 No reasonable conclusion about relapsing rate and duration of response due to follow up restrictions.

  19. 小儿重症肌无力的治疗现状及进展%Current Status and Progress of the Treatment of Myasthenia Gravis in Children

    Institute of Scientific and Technical Information of China (English)

    李东景; 黄志

    2012-01-01

    重症肌无力(myasthenia gravis,MG)是累及神经-肌肉接头处突触后膜上的烟碱型乙酰胆碱受体( nicotinic acetylcholine receptor,nAChR),由乙酰胆碱受体抗体介导、细胞免疫依赖、补体参与的自身免疫性疾病.对于MG的治疗包括:对症治疗、免疫抑制剂、血浆置换、免疫球蛋白、胸腺切除等.随着MG发病机制的深入研究及新疗法的开展,使该病的临床疗效明显提高,病死率明显下降.本文就小儿MG的治疗现状及进展作一综述.

  20. Diagnosis and treatment of thymoma with myasthenia gravis%重症肌无力胸腺瘤的诊断与治疗

    Institute of Scientific and Technical Information of China (English)

    张其刚; 胡永校; 芦伟

    2006-01-01

    @@ 胸腺瘤发病率占纵隔肿瘤的25%~30%,重症肌无力(myasthenia gravis,MG)是其常见的并发症.我们统计国内外相关文献报道[1-3],MG胸腺瘤平均占胸腺瘤的31.7%.MG胸腺瘤与胸外科其他疾病相比有许多特点和不同之处,深入探讨其诊断和治疗手段及方法,对于提高诊治效果、改善预后具有重要意义.

  1. Significance of Fas and bcl-2 gene in the pathogenesis and treatment of myasthenia gravis%Fas和bcl-2基因与重症肌无力的发生与治疗

    Institute of Scientific and Technical Information of China (English)

    赖成虹; 李作孝

    2005-01-01

    OBJECTIVE: At present the function of Fas and Bcl-2 gene in the pathogenesis of myasthenia gravis has gained more attention and more studies have been extensively made inside and outside, to investigate the modulation of Fas and Bcl-2 gene expression is of extremely significance for exploring the pathogenesis and treatment of myasthenia gravis.DATA SOURCE: The related articles published in English from January 1990 to December 2004 were computer searched in Medline database using the terms of "Fas", "Bcl-2", "Myasthenia Gravis" and "gene".STUDY SELECTION: The data were identified preliminarily and literatures on the relationship between Fas and Bcl-2 gene and myasthenia gravis, as well as on Fas and Bcl-2 gene and genotherapy were remained for searching the full content.DATA EXTRACTION: A great deal of literatures about Fas and Bcl-2 gene were searched, 21 of which were studies on Fas and Bcl-2 gene and myasthenia gravis, and 32 on myasthenia gravis related genotherapy. A total of 20 representative articles were selected for analysis in this study.DATA SYNTHESIS: Eight out of 20 literatures were about the relationship between Fas gene and myasthenia gravis, 5 about the Bcl-2 gene and myasthenia gravis, 7 about the genotherapy in the treatment of myasthenia gravis and immune diseases.CONCLUSION: Abnormal Fas and Bcl-2 gene modulation and uncontrollable expression were closely associated with apoptosis defect of T lymphocyte and thymocytes, and even closely related to the pathogenesis of myasthenia gravis. To explore pathogenesis of myasthenia gravis at gene level by inducing cell apoptosis and depressing self-antibody production, would provide new means for the treatment of myasthenia gravis.%背景:目前Fas和Bcl-2基因在重症肌无力发病机制中的作用越来越受到重视,国内外已做了较多的研究,阐明调控Fas和Bcl-2基因的表达对重症肌无力发生及其疾病过程中的治疗均具有密切的关系.资料来源:

  2. The role of γ-interferon in the pathogenesis of myasthenia gravis%干扰素-γ在重症肌无力发病机制中的作用

    Institute of Scientific and Technical Information of China (English)

    谢琰臣; 孙兆林; 李海峰

    2002-01-01

    @@ 重症肌无力(myasthenia gravis,MG)是由乙酰胆碱受体抗体(acetylcholine receptor antibody,AChRAb)介导、细胞免疫依赖性补体参与的自身免疫性疾病,主要累及神经肌肉接头处突触后膜上的乙酰胆碱受体.

  3. Experience of Professor LIU Fu-you to Treat Ovular Type of Myasthenia Gravis%刘福友教授治疗眼肌型重症肌无力经验

    Institute of Scientific and Technical Information of China (English)

    王强

    2013-01-01

    刘福友教授善于从脾肾论治,运用补脾益肾法治疗眼肌型重症肌无力,配合瘫瘘胶囊取得满意临床疗效.%Professor LIU Fu-you Liu is good at treating ocular myasthenia gravis through the spleen and kidney treatment, with the Tanwei capsules to achieve a-satisfactory clinical efficacy.

  4. Employees with Myasthenia Gravis

    Science.gov (United States)

    ... Fatigue/Weakness: Reduce or eliminate physical exertion and workplace stress Schedule periodic rest breaks away from the workstation ... an accommodation. An administrator was having problems handling stress and ... person in the workplace, and provided a reader as an accommodation. A ...

  5. Antibodies to autoantigen targets in myasthenia and their value in clinical practice

    Directory of Open Access Journals (Sweden)

    S. I. Dedaev

    2014-01-01

    Full Text Available Myasthenia gravis is a classic autoimmune disease, which clinical manifestations in the form of weakness and abnormal muscle fatigue, due to the damaging effect of polyclonal antibodies to different structures of the neuromuscular synapse and muscles. The study of autoimmune substrate with myasthenia is routine in many clinics dealing with the problems of neuromuscular pathology, and the identification of high concentration of serum antibodies to a number of antigenic structures is the gold standard in diagnosis.Determination of serum antibodies to various autoimmune targets is an important tool in clinical practice. The majority of patients shows the high concentration of antibodies to AchR that gives the opportunity to use it as an important diagnostic criterion. The specificity of changes in the concentration of AchR-antibodies due to pathogenetic treatment allows to objectify the suppression of autoimmune aggression and evaluate the reliability of remission. However, the absence of AchR-antibodies when there are clear clinical and electromyography signs of myasthenia gravis suggests an autoimmune attack against a number of other targets, the most studied of which is the MuSK. On the contrary, patients with myasthenia gravis associated with thymoma, almost always have a higher level of AchR-antibodies. The presence of thymoma is accompanied by the generation of antibodies to titin and RyR, which is also observed in persons with late-onset myasthenia without thymoma. High concentration of antibodies to these structures can be interpreted as a reliable sign of thymoma in patients younger than 60 years.

  6. Current immune therapies of autoimmune disease of the nervous system with special emphasis to multiple sclerosis.

    Science.gov (United States)

    Vass, Karl

    2012-01-01

    Autoimmune diseases of the nervous system such as myasthenia gravis, inflammatory demyelinating polyneuropathies, multiple sclerosis and others are still not curable. Yet the introduction of modern immune therapies could significantly improve the prospects of many patients affected by these disorders. In addition to steroids and immunosuppression i.v. immunoglobulins are used for treatment of myasthenia gravis and chronic inflammatory demyelinating polyneuropathy. Interferons, glatiramer acetate, natalizumab and fingolimod are applied in multiple sclerosis. The ever-improving efficacy of the drugs has to be balanced against the increasing risk of possible severe adverse effects.

  7. Coexistence of autoimmune diseases and autoantibodies in patients with myasthenia gravis

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    Sibel Tamer

    2016-01-01

    Conclusions: MG has an increased frequency of coexisting ADs. Autoantibodies that are characteristic for ADs can be found in the patients without the presence of any of the clinical findings of ADs. Clinical attention towards the management of ADs is especially needed during the follow-up of patients with MG.

  8. Ephedrine as add-on therapy for patients with myasthenia gravis: protocol for a series of randomised, placebo-controlled n-of-1 trials

    Science.gov (United States)

    Vrinten, Charlotte; Lipka, Alexander F; van Zwet, Erik W; Schimmel, Kirsten J M; Cornel, Martina C; Kuijpers, Marja R; Hekster, Yechiel A; Weinreich, Stephanie S; Verschuuren, Jan J G M

    2015-01-01

    Introduction Myasthenia gravis (MG), a rare neuromuscular disease, is often initially treated using acetylcholinesterase inhibitors. Patients who do not respond adequately depend on the use of corticosteroids or other immunosuppressive medication, but these may have serious side effects. Clinical observations suggest that ephedrine can diminish, postpone or even prevent the need for immunosuppressive therapy when added to acetylcholinesterase inhibitors or low-dose prednisone. In the Netherlands, ephedrine is not licensed for MG nor is reimbursement guaranteed. MG is a rare condition, and ephedrine might be indicated only in a subset of patients. Thus, randomised controlled trials comparing large groups are difficult to conduct. We, therefore, aim to aggregate data from a small series of n-of-1 trials (also known as single patient trials) to assess the effect of ephedrine as add-on treatment for MG. Methods and analysis Single-centre, placebo-controlled, double-blind, randomised, multiple crossover n-of-1 studies in 4 adult patients with generalised MG who show inadequate improvement on pyridostigmine and/or immunosuppressive drugs. Each n-of-1 trial has 3 cycles of two 5-day intervention periods. Treatment: 25 mg ephedrine or placebo, twice daily. Main outcome measure: Quantitative Myasthenia Gravis (QMG) test. Statistical analysis: fixed effects linear model for QMG for all patients combined. Secondary outcome measures: Clinical: effects on MG-Composite and MG-Activities of Daily Living (MG-ADL) scales; QMG at individual level; adverse events. Acceptability of trial design: number of patients eligible and enrolled; number of treatment cycles completed; patients’ and caregivers’ experiences. Ethics and dissemination This study was approved by the Medical Ethics Committee of Leiden University Medical Center, No. P14.108. Results of the trial will be reported in a peer-reviewed publication. Regulatory stakeholders will comment on the suitability of the trial

  9. Neuroelectrophysiological studies on neurological autoimmune diseases

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    Yin-hong LIU

    2014-09-01

    Full Text Available The neuroelectrophysiological manifestations of four clinical typical neurological autoimmune diseases including multiple sclerosis (MS, Guillain-Barré syndrome (GBS, myasthenia gravis (MG, and polymyositis and dermatomyositis were reviewed in this paper. The diagnostic value of evoked potentials for multiple sclerosis, nerve conduction studies (NCS for Guillain-Barré syndrome, repetitive nerve stimulation (RNS and single-fiber electromyography (SFEMG for myasthenia gravis, and needle electromyography for polymyositis and dermatomyositis were respectively discussed. This review will help to have comprehensive understanding on electrophysiological examinations and their clinical significance in the diagnosis of neurological autoimmune diseases. doi: 10.3969/j.issn.1672-6731.2014.09.004

  10. Analysis of Clinical Characteristics and Prognosis of 40 Cases of Myasthenia Gravis With Thymoma%40例伴胸腺瘤的重症肌无力的临床特点及预后分析

    Institute of Scientific and Technical Information of China (English)

    郭素彦

    2015-01-01

    Objective To investigate and analyze the myasthenia gravis with thymoma, the clinical features and prognosis.Methods 40 cases of thymoma patients with myasthenia gravis were chosen as research subjects in our hospital, analyze the Osserman clinical classification. Thymoma histologic features, the incidence of recurrence myasthenia gravis after surgery, and the prognosis of patients.Results The patients with myasthenia gravis with thymoma Osserman clinical classification withⅡB were most, and It was 45.00% (18/40), the most common type of the thymoma histologic pathology was B2, and It was 47.50% (19/40), there were 7.50% of the patients in the postoperative having the recurrence of symptoms of muscle weakness in 6 months. There were 12.50% of the patients in the postoperative rehabilitation process gravis symptoms worsening. There were 5.00% of patients died of thymoma myasthenia gravis crisis.Conclusion The condition of the myasthenia gravis with thymoma is very complicated, cancer symptoms is easy to relapse and transfer. The risk factor of the metastasis is high. There are certain critically ill mortality.%目的:研究分析伴胸腺瘤的重症肌无力的临床特点及预后分析。方法抽取我院收治的40例胸腺瘤的重症肌无力患者作为研究对象,分析其Osserman临床分型、胸腺瘤组织学特征、手术后复发重症肌无力的发生率以及患者预后的情况。结果伴胸腺瘤的重症肌无力患者的Osserman临床分型以ⅡB型最多,为45.00%(18/40),胸腺瘤病理的组织学分型以B2型最多见,为47.50%(19/40)。其中有7.50%的患者在术后6个月内发生肌无力症状复发,有12.50%的患者在术后康复过程中肌无力症状加重,有5.00%的患者死于伴胸腺瘤的重症肌无力危象。结论伴胸腺瘤的重症肌无力疾病病情十分复杂,肿瘤症状容易复发且易转移,危险系数很高,有一定的病危死亡率。

  11. Analysis of scores of Hamilton depression rating scale in patients with myasthenia gravis%重症肌无力患者汉密尔顿抑郁量表评分分析

    Institute of Scientific and Technical Information of China (English)

    马姗; 赵代弟; 李柱一; 范玲玲; 杨永祥; 闫忠军; 张慜; 王钧刚; 王圣元; 李川; 郭鹏

    2016-01-01

    Objective To investigate the scores of Hamilton depression rating scale (HDRS) in patients with myasthenia gravis (MG ) and the related influencing factors. Methods A cross‐sectional study of the clinical data and HDRS score was carried out in 188 MG patients who were admitted to Tangdu Hospital of the Fourth Military Medical University from July 2013 to March 2015. All the recruited patients were classified into the with depression group and the without depression group according to the HDRS score. The judge criterion of depression was as follow :HDRS scores of patients without depression were 0‐9 and patients with depression were above 9. The clinical features of the two groups and their relation with HDRS scores were analyzed. Results Among those MG patients , the male to female ratio was 1.02 ∶ 1. The ratio of ocular myasthenia gravis (OMG) to generalized myasthenia gravis (GMG) was 1.2 ∶ 1.The ratio of patients with onset symptom of OMG to patients with onset symptom of GMG was 6.2 ∶ 1. The median disease course was 2 years and the interquartile range was 1.8 years. The average quantitative myasthenia gravis (QMG) score was 6.7 ± 2.3 and the average HDRS score was 8.7 ± 3.4.One hundred and twenty‐three MG patients were not complicated with depression ,while 65 cases were complicated with depression.Factors correlated with HDRS score and depression state included gender (P<0.01) ,clinical type of MG (P<0.01) ,QMG score (P<0.01) ,myasthenia gravis foundation of America (MGFA ) classification ( P< 0.01 ) , with or without hyperthyreosis ( P< 0.05 ). Conclusions Factors correlated with HDRS score and depression state include gender , clinical type of MG , QMG score and MGFA classification , sufficient understanding of the depression state in MG patients might be helpful for better treatment.%目的:探讨重症肌无力(myasthenia gravis ,MG)患者汉密尔顿抑郁量表(Hamilton depression rating scale ,HDRS)评分情况及其影响

  12. PROTECTIVE EFFECT OF ScFv-DAF FUSION PROTEIN ON THE COMPLEMENT ATTACK TO ACETYLCHOLINE RECEPTOR: A POSSIBLE OPTION FOR TREATMENT OF MYASTHENIA GRAVIS

    Science.gov (United States)

    XU, JIANG; WU, XINGAN; ZHANG, FANGLIN; LIN, HONG; LI, ZHUYI; KAMINSKI, HENRY J.

    2015-01-01

    Introduction Autoantibody-induced complement activation, which causes disruption of the postsynaptic membrane, is recognized as a key pathogenic factor in myasthenia gravis (MG). Therefore, specific targeting of complement inhibitors to the site of complement activation is a potential therapeutic strategy for treatment of MG. Methods We assessed expression of single-chain antibody fragment–decay accelerating factor (scFv-DAF), comprising a single-chain fragment scFv1956 based on the rat complement inhibitor DAF in prokaryotic systems, and studied its inhibitory effect on complement deposition in vitro. Results The recombinant conjugate scFv-DAF completely retained the wild-type binding activity of scFv1956 to AChR and inhibited complement activation of DAF in vitro. Conclusions We found that scFv-DAF could bind specifically to TE671 cells, and it is significantly more potent at inhibiting complement deposition than the untargeted parent molecule DAF. scFv-DAF may be a candidate for in vivo protection of the AChR in MG. PMID:22499093

  13. Plasmapheresis as preparatory method for thymectomy in myasthenia gravis Plasmaferese como método preparatório de timectomia em miastenia grave

    Directory of Open Access Journals (Sweden)

    José Carlos Brant Seggia

    1995-09-01

    Full Text Available To study the effects of plasmapheresis in preparation for thymectomy, two groups of 40 patients were selected from a sample of 286 patients with myasthenia gravis examined by the first author Group 1 included patients (15 male and 25 female; age range 8-64 yrs who underwent thymectomy without previous plasmapheresis, whereas patients in group 2 (17 male and 23 female; age range 11-61 yrs were thymectomized after plasmapheresis. We required patients to have a minimum follow-up period of 12 months to be included in the study. A clinical evaluation protocol composed of 76 items was developed for the study. We found significant improvement in respiratory function and muscular strength in patients thymetomized after plasmapheresis. Furthermore, the combined treatment reduced cost and length of hospital stay. Therefore, we conclude that plasmapheresis should be considered as a coadjuvant to thymectomy in the treatment of myasthenia gravis.No intuito de estudar as repercussões de um curso de plasmaferese como método preparatório para a timectomia, tomamos duas amostras de 40 pacientes selecionados de um universo de 286 casos de miastenia gravis, em que a investigação clínica, eletrofisiológica, laboratorial, o seguimento e o protocolo foram completamente realizados. Consideramos como grupo 1 aquele em que a timectomia foi realizada sem preparação pre-operatória e grupo 2 aquele em que a plasmaferese foi executada como coadjuvante a timectomia. O grupo 1 era composto de 15 pacientes do sexo masculino e 25 do feminino; a idade variou de 8 a 64 anos com mediana de 30 anos. No segundo grupo, 17 eram masculinos e 23 femininos; a idade variou de 11 a 61 anos com mediana de 33 anos. Foi preenchida ficha de avaliação clínica sequencial de 76 itens e traçamos um curso de plasmaferese de 5 sessões seguido da timectomia, mantendo a mesma técnica operatória, realizada pela mesma equipe cirúrgica, tentando assim minimizar as distorções na an

  14. 重症肌无力合并甲状腺机能亢进患者的临床治疗观察%Myasthenia Gravis with Hyperthyroidism Patients Clinical Observation

    Institute of Scientific and Technical Information of China (English)

    郑燕学

    2015-01-01

    Objective To analyze myasthenia gravis merger hyperthyroidism patients clinical characteristics and treatment paths.Methods Selection in August 2013 to September 2014 department of neurology were analyzed during the period of 52 patients with myasthenia gravis hyperthyroidism merger as a clinical research object,combining with the characteristics of patients synchronous gives two development schemes,patients with clinical symptoms was observed.Results 52 patients with clinical orientation after treatment,patients with thyroid function,clinical symptoms of myasthenia gravis were benign improved,the overal treatment ef ect is remarkable.Conclusion For myasthenia gravis patients with hyperthyroidism clinical treatment,by giving two development scheme,which can ef ectively improve the patients,improve the treatment of patients with life.%目的分析重症肌无力合并甲状腺机能亢进患者临床特点及治疗路径。方法选取2013年8月~2014年9月我院神经内科收治的52例重症肌无力合并甲状腺机能亢进患者作为临床研究对象,结合患者病情特点同步给予两病同治方案,观察患者临床症状改善情况。结果52例患者经临床定向治疗后,患者甲状腺功能、重症肌无力临床症状均得到了良性改善,整体治疗效果显著。结论针对重症肌无力合并甲状腺机能亢进患者临床治疗,通过给予两病同治方案,可有效改善患者病情,提升患者生活质量。

  15. Effects of Hormones on Proliferation and Apoptosis of Thymocytes in Patients with Myasthenia Gravis%激素对重症肌无力胸腺细胞增殖和凋亡的影响

    Institute of Scientific and Technical Information of China (English)

    杜英; 阮丽荣; 张清勇; 梁长春; 李倩如

    2002-01-01

    Objective To study the role of glucocorticoid, estrogen and thyroxine on Fas molecularexpression and apoptosis of thymocytes in patients with myasthenia gravis. Method Expression of thethymocytes Fas, CD4 and CD8 molecular was assayed with flow cytometry. The thymocytes apoptosis wasobserved with agarose gel electrophoresis and cell morphology. The cells proliferation was studied withMTT analysis methode. Result Both estrogen and dexasone could inhibit the proliferation of thymocytesof MG patients. There were significant difference(P<0.01) between experiment groups and controlgroups. The cultivated cells with dexasone showed characteration of apoptosis and the DNA of themshowed typically ladder appearance in electrophoresis. The proportion of Fas + thymocytes only in dexas-one groups was significantly higher(P<0.05) than controls. Thyroxine could accelerate the proliferationof thymocytes of MG and normal human. There were significant difference(P<0.05)between experimentgroups and control groups. Conclusion It was significant that the hormones on proliferation and apoptosisof thymocytes in patients with myasthenia gravis.

  16. 王宝亮教授治疗重症肌无力验案三则%Treating 3 cases of myasthenia gravis by Professor WANG Bao-liang

    Institute of Scientific and Technical Information of China (English)

    文凯华; 牛跃辉; 王宝亮

    2012-01-01

      Professor WANG Bao-liang is a famous doctor of Chinese medicine, clinical experience of 30 years, good at using the integrative medicine to treat cerebrovascular disease, motor neuron disease and multiple sclerosis. In recent years, he focused on the clinical study of myasthenia gravis, notable curative effect. The teacher Wang in the clinical treatment of three cases of myasthenia gravis was summarized.%  王宝亮教授,一位名中医,临证30余载,擅长中西医结合治疗脑血管病、运动神经元病、多发性硬化。近年来专注于重症肌无力的临床研究,疗效显著。兹将王师在临床中治疗重症肌无力之验案三则进行了总结。

  17. Thyroid autoimmunity and polyglandular endocrine syndromes.

    Science.gov (United States)

    Wémeau, Jean-Louis; Proust-Lemoine, Emmanuelle; Ryndak, Amélie; Vanhove, Laura

    2013-01-01

    Even though autoimmune thyroiditis is considered as the most emblematic type of organ-specific autoimmune disorder of autoimmunity, autoimmune thyroid diseases can be associated with other autoimmune endocrine failures or non-endocrine diseases (namely vitiligo, pernicious anemia, myasthenia gravis, autoimmune gastritis, celiac disease, hepatitis). Thyroid disorders, which are the most frequent expression of adult polyendocrine syndrome type 2, occur concomitantly with or secondarily to insulinodependent diabetes, premature ovarian failure, Addison's disease (Schmidt syndrome, or Carpenter syndrome if associated with diabetes). Testicular failure and hypoparathyroidism are unusual. The disease is polygenic and multifactorial. Disorders of thyroid autoimmunity are, surprisingly, very rare in polyendocrine syndrome type 1 (or APECED) beginning during childhood. They are related to mutations of the AIRE gene that encodes for a transcriptional factor implicated in central and peripheral immune tolerance. Hypothyroidism can also be observed in the very rare IPEX and POEMS syndromes.

  18. Slow-binding inhibition of acetylcholinesterase by an alkylammonium derivative of 6-methyluracil: mechanism and possible advantages for myasthenia gravis treatment.

    Science.gov (United States)

    Kharlamova, Alexandra D; Lushchekina, Sofya V; Petrov, Konstantin A; Kots, Ekaterina D; Nachon, Florian; Villard-Wandhammer, Marielle; Zueva, Irina V; Krejci, Eric; Reznik, Vladimir S; Zobov, Vladimir V; Nikolsky, Evgeny E; Masson, Patrick

    2016-05-01

    Inhibition of human AChE (acetylcholinesterase) and BChE (butyrylcholinesterase) by an alkylammonium derivative of 6-methyluracil, C-547, a potential drug for the treatment of MG (myasthenia gravis) was studied. Kinetic analysis of AChE inhibition showed that C-547 is a slow-binding inhibitor of type B, i.e. after formation of the initial enzyme·inhibitor complex (Ki=140 pM), an induced-fit step allows establishment of the final complex (Ki*=22 pM). The estimated koff is low, 0.05 min(-1) On the other hand, reversible inhibition of human BChE is a fast-binding process of mixed-type (Ki=1.77 μM; Ki'=3.17 μM). The crystal structure of mouse AChE complexed with C-547 was solved at 3.13 Å resolution. The complex is stabilized by cation-π, stacking and hydrogen-bonding interactions. Molecular dynamics simulations of the binding/dissociation processes of C-547 and C-35 (a non-charged analogue) to mouse and human AChEs were performed. Molecular modelling on mouse and human AChE showed that the slow step results from an enzyme conformational change that allows C-547 to cross the bottleneck in the active-site gorge, followed by formation of tight complex, as observed in the crystal structure. In contrast, the related non-charged compound C-35 is not a slow-binding inhibitor. It does not cross the bottleneck because it is not sensitive to the electrostatic driving force to reach the bottom of the gorge. Thus C-547 is one of the most potent and selective reversible inhibitors of AChE with a long residence time, τ=20 min, longer than for other reversible inhibitors used in the treatment of MG. This makes C-547 a promising drug for the treatment of this disease.

  19. The therapeutic effect of salbutamol on myasthenia gravis%沙丁胺醇治疗重症肌无力的临床观察

    Institute of Scientific and Technical Information of China (English)

    侯世芳; 许贤豪; 刘银红

    2011-01-01

    目的 评价硫酸沙丁胺醇(salbutamol sulfate)治疗重症肌无力(myasthenia gravis,MG)的临床疗效.方法 选取胸腺切除术后的MG住院患者51例,按手术先后顺序分为沙丁胺醇组(SS组,n=27)和非沙丁胺醇组(非SS组,n=24),SS组患者给予免疫抑制剂和沙丁胺醇治疗,非SS组仅给予免疫抑制剂治疗.采用临床绝对评分法和相对评分法对患者的临床疗效进行评定,并对部分患者治疗2年后的疗效进行随访.结果 SS组和非SS组患者治疗前与治疗2个月后的临床绝对评分差值及降幅间比较无统计学差异(均P>0.05),两组间治疗总有效率比较差异无统计学意义(P>0.05),SS组的平均住院天数、呼吸机使用比例显著低于非SS组(均P 0. 05). The rate of respiratory support, as well as in-hospital duration, in the SS group was significantly less than that in the non-SS group (both P<0. 05). 36 patients finished the two years' following-up study, and both of score decrement and withdrawal rates in the SS group were significantly higher than those in non-SS group (both P<0. 05). The relapse rate of the SS group was significantly lower than that of the non-SS group, the same happened regarding the infection rate (both P<0. 05). The difference of side effects between the two groups was statistically insignificant. Conclusions Combining immunosuppressant treatment with salbutamol sulfate might improve the clinical symptoms of MG patients quickly, and might be helpful in reducing infection rate and relapse rate when immunosuppressant was decreased or withdrawn. No side effect developed during the follow-up.

  20. Shock: A possible presenting manifestation of autoimmune polyendocrine syndrome type II

    Directory of Open Access Journals (Sweden)

    Subodh Banzal

    2014-01-01

    Full Text Available Autoimmune polyendocrine syndrome Type II (APS II, also known as polyglandular autoimmune syndrome Type II or Schmidt syndrome, is constellations of multiple endocrine gland insufficiencies. It is a rare, but most common of the immunoendocrinopathy syndrome. It is characterized by the obligatory occurrence of autoimmune Addison′s disease in combination with thyroid autoimmune diseases and/or Type I diabetes, hypogonadism, hypophysitis, myasthenia gravis, vitiligo, alopecia, pernicious anemia, and celiac disease. Here, we report a case of 38-year-old female patient presented with shock, further diagnosed to have APS II.

  1. Miastenia gravis diagnostic in dogs

    Directory of Open Access Journals (Sweden)

    Adriana Patricia Suraniti

    2010-12-01

    Full Text Available Miastenia Gravis is a neuromuscular disease caused by auto antibodies. Early Clinical and biochemical diagnosis and treatment is demanded in the assurementof quality and time of life in all dogs. In this study we describe the conventional diagnosis methods and therapy in 32 dogs with suspected myasthenia gravis and propose the administration of bromide of piridostigmin as another use full diagnosis method in dogs.

  2. Expression of a highly antigenic and native-like folded extracellular domain of the human α1 subunit of muscle nicotinic acetylcholine receptor, suitable for use in antigen specific therapies for Myasthenia Gravis.

    Directory of Open Access Journals (Sweden)

    Athanasios Niarchos

    Full Text Available We describe the expression of the extracellular domain of the human α1 nicotinic acetylcholine receptor (nAChR in lepidopteran insect cells (i-α1-ECD and its suitability for use in antigen-specific therapies for Myasthenia Gravis (MG. Compared to the previously expressed protein in P. pastoris (y-α1-ECD, i-α1-ECD had a 2-fold increased expression yield, bound anti-nAChR monoclonal antibodies and autoantibodies from MG patients two to several-fold more efficiently and resulted in a secondary structure closer to that of the crystal structure of mouse α1-ECD. Our results indicate that i-α1-ECD is an improved protein for use in antigen-specific MG therapeutic strategies.

  3. More stress on the value of thymus pathologic changes in the treatment of myasthenia gravis%重视不同类型胸腺病理变化在重症肌无力治疗中的价值

    Institute of Scientific and Technical Information of China (English)

    涂来慧

    2005-01-01

    针对胸腺病理检查对重症肌无力(myasthenia gravis,MG)的发病、诊断和治疗有重要意义,然而目前仍有不少患者得不到及时诊断和治疗。即使发现MG,大多忽略胸腺病变性质,停留于一般诊断和药物治疗。即使手术,不少医院病理科也只凭HE染色切片作一般形态学描述,未按标准作胸腺病理分类诊断,以致诊断深度不够,

  4. Plasmapheresis in the treatment of myasthenia gravis: retrospective study of 26 patients Plasmaférese no tratamento da miastenia grave: estudo retrospectivo de 26 pacientes

    Directory of Open Access Journals (Sweden)

    Rosana Carandina-Maffeis

    2004-06-01

    Full Text Available We analyzed the experience of Unicamp Clinical Hospital with plasma exchange (PE therapy in myasthenia gravis (MG. About 17.8 % of a totality of MG patients had PE performed: 26 cases, 19 women and seven men. The mean age-onset of MG was 28 years, extremes 11 and 69. Minimum deficit observed in the group was graded IIb (O & G or IIIa (MGFA scale. One patient had prethymectomy PE. In seven the procedures were performed due to myasthenic crisis and in 18 patients due to severe myasthenic symptoms or exacerbation of previous motor deficit. Two patients were also submitted to chronic PE considering refractoriness to other treatments. Twenty-six patients had 44 cycles of PE and 171 sessions. The mean number of sessions was 3.9 (SD ± 1.4 each cycle; median 5, extremes 2 and 6. The mean time by session was 106,5 minutes (SD ± 35.2; median 100.5 (extremes of 55 and 215. The mean volume of plasma exchanged in each session was 2396 ml (SD ± 561; median 2225 (extremes 1512 and 4500. Side effects occurred: reversible hypotension (seven cases, mild tremor or paresthesias (seven cases. Infection and mortality rates due to PE were zero. All patients had immediate benefit of each PE cycle and usually they also received prednisone or other immunosuppressors. Good acceptance of the procedure was observed in 80.7% of patients.Analisamos a experiência do Hospital das Clínicas da Unicamp com plasmaferese: (PF na miastania grave (MG. 17,8 % do total dos casos de MG submeteu-se a PF, 26 casos, 19 mulheres e sete homens. A idade média de início da MG foi 28 anos (extremos 11 e 69. O menor déficit clínico foi IIb (O & G e IIIa (MGFA. A PF foi indicada no pré-operatório de timectomia em um caso e em sete devido a crise miastênica. Em 18 casos, com MG generalizada e sintomas bulbares ou com exacerbação de déficit prévio, a PF foi indicada como intervenção aguda. Em dois pacientes desse grupo ela foi indicada também em regime crônico de ciclos mensais

  5. 重症肌无力患者嗅觉功能与临床特征分析%Evaluations of olfactory function for myasthenia gravis patients

    Institute of Scientific and Technical Information of China (English)

    李欣; 杨丽; 张璘洁; 付莹; 李立敏

    2015-01-01

    Objective To explore the olfactory function of patients with myasthenia gravis (MG) and analyze the correlation between olfactory dysfunction and clinical characteristics.Methods A total of 61 MG patients from March to July 2014 and 32 healthy controls (HC) received T&T olfactometer test.Their clinical data were collected.And the relationship of T&T score and clinical characteristics were analyzed.Results The identification threshold of T&T olfactometer test was (2.07 ± 1.56) versus (0.31 ±1.25) for healthy controls.MG patients had a worse performance on olfaction test than healthy controls (t =5.50,P < 0.01).By Spearman' s analysis,disease course (r =1.348,P =0.006),QMG scores (r =0.393,P =0.025),ADL scores (r =0.421,P =0.01) and dosing time of pyridostigmine (r =0.295,P=0.025) were positively correlated with T&T scores.However,the dosing times of prednisone (r =0.084,P =0.053) or immunodepressants (r =0.153,P =0.251) were not correlated with T&T scores.Conclusion MG patients have poor olfactory sensation.And a potential involvement of non-motor system shall attract the attention of neurologists.%目的 探讨重症肌无力(MG)患者嗅觉功能及其与临床特征的相关性.方法 利用T&T嗅觉检测法对自2014年3-7月在天津医科大学总医院神经内科确诊的61例重症肌无力患者和32名健康对照进行嗅觉检测,收集MG患者临床信息,利用Spearman相关性分析嗅觉功能与临床特征的相关性.结果 重症肌无力患者嗅觉识别阈为(2.07±1.56),健康对照嗅觉识别阈为(0.31±1.25),二者差异具有统计学意义(t=5.50,P<0.01),Spearman相关分析显示重症肌无力患者嗅觉水平与病程(r =0.348,P=1.006)、疾病严重程度定量(QMG)评分(r=0.393,P=0.025)、每日生存活动等级(ADL)评分(r=0.421,P=0.01)和累计服用溴吡斯的明的时间(r=0.295,P=0.025)呈正相关;与累计服用泼尼松时间(r=0.084,P=0.053)、服用免疫抑制剂时间(r=0.153,P=0.251)无相关性.结论

  6. Review on thymoma and thymoma-associated autoimmune diseases

    Directory of Open Access Journals (Sweden)

    Yue-min LI

    2014-10-01

    Full Text Available Thymic carcinomas are tumors of the anterior mediastinum derived from the epithelial cells of the thymus gland. Malignancies linked to thymoma lead to the loss of self-tolerance leading to autoimmune diseases, including myasthenia gravis, pure red cell aplastic anemia, systemic lupus erythematosus and pemphigus etc. In recent years, three main mechanisms have been proposed to elucidate these interactions, such as immature T cell theory, tumor-gene theory and the combination mechanism of cellular and humoral immunity. In fact, the resection of the thymoma is beneficial to many patients of thymoma related autoimmune diseases. DOI: 10.11855/j.issn.0577-7402.2014.08.16

  7. Muscle strength in myasthenia gravis

    DEFF Research Database (Denmark)

    Cejvanovic, S; Vissing, J

    2014-01-01

    is related to disease duration or gender. The aim of this study was to quantify the strength of patients with MG and investigate whether it is related to disease duration. METHODS: Eight muscle groups were tested by manual muscle testing and with a hand-held dynamometer in 38 patients with generalized MG...... and 37 healthy age- and gender-matched controls. The disease duration was recorded and compared with strength measures. RESULTS: On average, muscle strength was decreased by 28% compared with controls (Pstrength measures in individual patients did not differ, suggesting that the muscle...... force reported was not subject to fatigue, but reflected fixed weakness. The male patients showed a greater reduction in muscle force in all eight muscle groups than women with MG (60% vs 77% of normal, Pstrength in shoulder abductors was most affected (51% vs 62...

  8. Myasthenia Gravis (MG): Medical Management

    Science.gov (United States)

    ... most commonly used is pyridostigmine (Mestinon) . Cholinesterase inhibitors boost ACh levels not only at the neuromuscular junction but also in the autonomic nervous system (which controls involuntary bodily functions). Sometimes the ...

  9. 合并胸腺瘤对胸腺切除治疗重症肌无力效果的影响%Influence of concomitant thymoma on effect of thymectomy for myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    李剑锋; 崔健; 杨帆; 刘彦国; 卜梁; 隋锡朝; 王俊

    2012-01-01

    目的 总结胸腔镜胸腺扩大切除治疗重症肌无力的远期疗效,并分析合并胸腺瘤对手术疗效的潜在影响.方法 2001年4月至2009年10月47例重症肌无力接受胸腔镜手术的患者分为有胸腺瘤组和无胸腺瘤组两组,评价肿瘤因素对重症肌无力胸腔镜手术效果的影响.结果 47例中男20例,女27例;平均年龄36.6岁.根据美国重症肌无力基金会(MGFA)分型:Ⅰ型18例、Ⅱa型14例、Ⅱb型14例、Ⅲa型1例.胸腺瘤组22例,无瘤组25例.随访至2011年6月,无瘤组随访20 ~ 122个月,平均57个月;失访2例.无瘤组完全稳定缓解(CSR) 78.3%,药物缓解(PR) 13.0%,微小症状表现(MM)4.3%,无恶化(W)0,无复发(E)0,死亡(D)(4.3%).胸腺瘤组分别为:50.0%、22.7%、13.6%、4.5%、9.1%和0.结论 胸腔镜胸腺扩大切除治疗重症肌无力远期疗效满意,无瘤组在完全缓解率上优于有瘤组,但在总有效率上两组差异无统计学意义.%Objective To evaluate the long term effect of video-assisted thoracoscopic thymectomy for myasth,enia gravis and the influence of concomitant thymoma.Methods 47 cases of myasthenia gravis were retrospectively reviewed who had received video-assisted thoracoscopic thymectomy from Apr.2001 through Oct.2009.The patients were separated to two groups with or without thymoma.Influence of oncologic factors was carefully studied.Results There were 20 males and 27 females with a mean age of 36.6 yrs.According to the typing system of the Myasthenia Gravis Foundation of America ( MGFA),the patients belonged to type Ⅰ 18 cases,type Ⅱ a 14 cases,type Ⅱ b 14 cases,and Ⅲa 1 case.22 patients were in the group with thymoma,and the other 25 in the group without thymoma.Until the deadline of follow-up time of Jun.2011,only two cases in non-thymomatous group were lost.Follow-up time was 20 to 122 months,mean 57 months.The complete stable remission rate(CSR),pharmacologic remission(PR),minimal manifestations

  10. Megaesophagus secondary to myasthenia gravis in a female German shepherd dog/ Megaesôfago secundário a miastenia grave em uma cadela da raça Pastor Alemão

    Directory of Open Access Journals (Sweden)

    Osimar Sanches

    2007-08-01

    Full Text Available Megaesophagus is a common cause of regurgitation being one of the possible etiologies the myasthenia gravis characterized by a neuromuscular disorder that result in weakness of the skeletal muscles, of the esophagus, of the larynx and of the pharynx. The present report describes a case of a female German shepherd dog, with report of muscles weakness and frequent vomits, which was diagnosed as a megaesophagus secondary to myasthenia gravis. The radiograph of the animal presented an accentuated cervical and thoracic megaesophagus. After the institution of the recommended therapeutic protocol the animal presented visible improvement when walking, however the regurgitations worsened and it began to present cough. New radiograph was accomplished with presence, besides megaesophagus, of aspiration pneumonia. The owner opted for the euthanasia of the animal and the necropsy revealed severe pneumonia and an accentuated dilation of the posterior portion of the esophagus, collaborating with subsidies for an unfavorable prognostic of the case.Megaesôfago é uma causa comum de regurgitação sendo uma das possíveis etiologias a miastenia grave caracterizada por uma desordem neuromuscular que resulta em fraqueza dos músculos esqueléticos, do esôfago, da laringe e da faringe. O presente relato descreve um caso de uma cadela da raça Pastor Alemão, com histórico de fraqueza muscular e vômitos freqüentes, que foi diagnosticada como megaesôfago secundário à miastenia grave. A radiografia do animal apresentou um acentuado megaesôfago cervical e torácico. Após a instituição do protocolo terapêutico recomendado o animal apresentou melhora visível ao caminhar, porém as regurgitações pioraram e começou a apresentar tosse. Foi realizado novo exame radiográfico com presença, além de megaesôfago, de pneumonia aspirativa. A proprietária optou pela eutanásia do animal e a necropsia revelou severa pneumonia e dilatação acentuada da por

  11. 重症肌无力合并抑郁78例相关因素分析%Study of incidence and correlation factors of depression in patients with myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    刘慧华

    2014-01-01

    Objective:To investigate the incidence rate and correlation factors of depression in patients with myasthenia gravis( MG) . Methods:Seventy-eight MG patients were assessed and graded with Hamilton Depression Rating scale,activities of daily living and self-designed register form. The correlation factors were analyzed by logistic regression. Results:The overall incidence rate was 60. 3%. The minor,moderate and major incidence was 44. 7%,34. 0% and 21. 3% respectively. The main manifestations of depression were anxiety/body,retardation,hopelessness and insomnia. The regression analysis showed that factors accounted for the occurrence of MG with depression were gender, score of activities of daily living and pyridostigmine bromide dose. Conclusions:The incidence of depression was high in MG patients. Depression with MG had a multifactorial etiology.%目的:探讨重症肌无力( myasthenia gravis,MG)合并抑郁的特点及其相关因素。方法:对78例MG患者采用汉密顿抑郁量表、MG日常生活能力量表及自制一般情况调查表进行测查并对各因素进行相关统计分析。结果:MG患者合并抑郁的发生率为60.3%,其中轻度抑郁44.7%,中度抑郁34.0%,重度抑郁21.3%,其抑郁情绪主要表现为焦虑/躯体化、阻滞、绝望及睡眠障碍等。 MG合并抑郁相关因素为性别(OR=1.198)、日常生活能力量表评分(OR=2.213)和溴吡斯的明使用量(OR=1.997)。结论:MG患者抑郁发生率较高,MG合并抑郁状态是多种因素共同作用所致。

  12. The clinical features of myasthenia gravis associated with thyroid abnormalities%重症肌无力伴发甲状腺异常的临床分析

    Institute of Scientific and Technical Information of China (English)

    陈玉萍; 魏东宁; 陈兵

    2010-01-01

    目的 分析重症肌无力(MG)合并甲状腺异常患者的临床特点.方法 回顾性分析2008年7月-2009年9月同期住院的300例MG患者临床资料,比较260例甲状腺正常及40例伴甲状腺异常的MG患者临床特点,血清甲状腺抗体与乙酰胆碱受体抗体水平的相关性及外周血T淋巴细胞亚群百分比情况.结果 (1)合并甲状腺异常的MG占13.3%,MG伴甲状腺异常以出现甲状腺抗体阳性为多见(30例,占10.0%);(2)甲状腺正常与异常的MG患者在发病性别构成、年龄分布、病程、临床分型及胸腺病理上差异均无统计学意义;(3)伴有甲状腺抗体阳性MG患者血突触后膜乙酰胆碱受体水平(1.15±0.11)显著高于甲状腺抗体阴性者(1.01±0.11);(4)甲状腺异常组CD8+T淋巴细胞百分比(21.63±5.17)%明显低于甲状腺正常组(24.28±5.79)%,P<0.05,CD4+/CD8+比值甲状腺异常组明显高于正常组(2.10±0.67比1.81±0.61,P<0.05).结论 MG合并甲状腺异常以甲状腺抗体阳性多见,伴有甲状腺抗体阳性MG患者血突触后膜乙酰胆碱受体水平显著升高;MG合并甲状腺异常者其外周血T淋巴细胞亚群紊乱更为明显.%Objective To analyze the clinical characteristics of myasthenia gravis(MG) associated with thyroid abnormalities.Methods A total of 300 MG patients admitted to the department of neurology from July 2008 to September 2009 were reviewed retrospectively.Based on the thyroid function and abnormality of thyroid related antibodies (thyroglobulin antibody and peroxidase antibody), the patients with MG were divided into two groups (260 cases without thyroid disease and 40 cases with thyroid abnormalities).The different clinical features, the relationship between the antithyroid antibodies and antiacetylcholine receptor antibody (AChRAb) and the distribution of T cell subsets between the two groups were compared.Results ( 1 ) Among the 300 MG patients, 13.3% of them was accompanied with thyroid disease and the

  13. Late-onset myasthenia not on the increase

    DEFF Research Database (Denmark)

    Pedersen, E G; Hallas, Jesper; Hansen, K;

    2013-01-01

    BACKGROUND: An increase in late-onset myasthenia gravis (MG) has been reported. There are few large population-based studies over longer periods of time reflecting recent developments in MG incidence. METHODS: We identified a nationwide cohort of patients with incident myasthenia in Denmark in 1996...

  14. 重症肌无力伴甲状腺病变106例的临床分析%Clinical features of myasthenia gravis with thyroid disease with 106 patients

    Institute of Scientific and Technical Information of China (English)

    孟超; 景筠; 李然; 张晓君; 王佳伟

    2016-01-01

    目的 探讨重症肌无力(MG)伴甲状腺病变的临床特点、病程及预后.方法 应用回顾性病例分析方法,收集首都医科大学附属北京同仁医院2004年1月1日至2013年12月31日门诊及住院MG患者106例,对其临床特点、甲状腺功能及抗体与病程和预后的关系等进行分析.结果 (1)106例患者甲状腺功能异常者20例(18.87%),甲状腺球蛋白抗体(TGAb)和(或)甲状腺微粒体抗体(TMAb)升高者37例(34.91%),甲状腺异常者46例(43.40%).甲状腺抗体升高阳性率高于甲状腺功能阳性率,差异有统计学意义(P =0.036).(2)眼肌型MG的促甲状腺激素(TSH)水平[(2.9 ±4.0) mIU/L)]明显高于全身型[(1.5±1.1)mIU/L],P=0.01.(3)52例眼肌型患者向全身型转化的时间,甲状腺抗体升高组比抗体正常组长,P=0.04,差异有统计学意义.而TSH是否升高、甲状腺功能是否异常、甲状腺是否异常之间的转化时间相比较差异无统计学意义.(4)在病程的第1年、2年、5年,缓解组与未缓解组的TSH水平、甲状腺抗体水平、甲状腺功能、甲状腺是否异常相比较,差异均无统计学意义(均P>0.05).结论 MG易合并甲状腺异常,MG更易患自身免疫性甲状腺疾病.眼肌型比全身型患者更易罹患自身免疫性甲状腺疾病.合并自身免疫性甲状腺疾病的眼肌型MG患者对激素等治疗手段反应比较敏感,短期预后相对较好.MG病情缓解率与TSH水平、甲状腺抗体水平、甲状腺功能、甲状腺是否异常等均无显著相关.%Objective To report the presentation,clinical course and prognosis of myasthenia gravis (MG) with thyroid disease.Methods Retrospective data analysis was conducted.Between 2004 and 2013,we reviewed a total of 106 patients with MG.We analyzed the clinical features,the relationship between the thyroid function,antibodies and the clinical course,prognosis.Results (1) In our study,20/106 (18.87%) patients were thyroid function-abnormal,37

  15. 发生胆碱能危象的重症肌无力危象患者抢救成功1例%Cholinergic Crisis of Myasthenia Gravis Crisis Patients Rescue Success in 1 Case

    Institute of Scientific and Technical Information of China (English)

    张妍; 于馥伟; 孙悦; 绪红; 李英

    2016-01-01

    Myasthenia gravis (MG) is common in clinic, when its in cholinergic crisis after the disease is dangerous, should be timely diagnosis and offer effective emergency treatment. Once confirmed, the patients should immediately stop using cholinesterase inhibitor, the so-called dry therapy, 0.5 ~ 1 mg intravenous injection atropine, at the same time when the patients to the ICU should be closely monitoring, to closely observe patient vital signs, prompting patients turned the corner.%重症肌无力(MG)在临床中十分常见,当其出现胆碱能危象后病情十分凶险,需及时明确诊断并给予有效抢救。患者一旦确诊,即应立即停用胆碱酯酶抑制剂,即所谓的干涸疗法,同时静脉注射阿托品0.5~1 mg,当患者送至ICU后应严密监护,对患者生命体征密切观察,促使患者转危为安。

  16. Management of pregnancy with myasthenia gravis: 7 cases report%妊娠合并重症肌无力七例临床分析

    Institute of Scientific and Technical Information of China (English)

    戚庆炜; 王丹; 刘俊涛; 边旭明

    2012-01-01

    诊至儿科严密监护.%Objective To discuss the interaction of pregnancy and myasthenia gravis(MG) and the management of pregnancy with MG.Methods Seven cases of pregnancy with MG in Peking Union Medical College Hospital were analyzed retrospectively,with respect to the therapy of MG,pregnancy complications and outcomes.Results Totally 38 683 pregnant women were admitted to Peking Union Medical College Hospital between Oct.1983 and Oct.2010.Among them there were 9 patients suffered from MG,with the incidence of 0.023%.Two pregnancies were terminated because of personal reasons,and seven continued.( 1 ) Onset of MG:in the 7 cases,6 were diagnosed before conception,with the mean course of 5.9 years.The other one occurred in the third trimester.(2) Management:all the cases were under close surveillance during pregnancy.Four women took thymectomy before conception,and one of them kept taking medication after surgery. In those who received thymectomy,3 cases remained stable and 1 case worsened during prenancy.The latter one took medication at 33 weeks,and continued to full term.MG exacerbated in the other three women who had not undergone thymectomy before conception.Among them, one woman complicated with systemic lupus erythematosus and lupus nephritis delivered the baby at 31 weeks.(3) Delivery and neonatal outcomes:cesarean deliveries were performed in 5 cases and the other two underwent vaginal deliveries.All the newborns were admitted to neonatal intensive care unit for surveillance.There were three smaller than gestational week (SGA) infants.No MG was observed in newborns.Conclusions Patients with MG should have an overall evaluation before conception.The course of MG during pregnancy is unpredictable.They may get a promising outcome under the control of a multidisciplinary team including obstetricians and neurologists.Newborns should be carefully monitored for sings of transitory MG in the department of pediatrics.

  17. AUTOIMMUNE DISEASE

    Institute of Scientific and Technical Information of China (English)

    2004-01-01

    2004456 The clinical features of myasthenia gravis affecting nonskeleton muscles. LIU Weibin (刘卫彬), et al. Dept Neurol, 1st Affili Hosp Sun Yat-sen Univ, Guangzhou 510080. Chin J Intern Med 2004; 43(8):588-590.

  18. Evaluation of the respiratory function in myasthenia gravis: an important tool for clinical feature and diagnosis of the disease Avaliação da função respiratória na miastenia gravis: importância na caracterização clínica e no diagnóstico da doença

    Directory of Open Access Journals (Sweden)

    Paulo A. P. Saraiva

    1996-12-01

    Full Text Available Myasthenic gravis may affect both inspiratory and expiratory muscles. Respiratory involvement occurred in almost all patients with myasthenia gravis in all clinical forms of the disease: 332 lung function tests done in 324 myasthenic patients without respiratory symptoms (age 34.6 ± 18.3 years were examined. Lung volumes analysis showed that all the patients of both sexes with generalized or ocular myasthenia gravis showed "myasthenic pattern". Male patients with "ocular" form only presented the "myasthenic pattern" with lung impairment and had, from the lung function point of view, a more benign behaviour. Female patients with the "ocular" form exhibited a behaviour of respiratory variables similar to that of the generalized form. It was not observed modification of the variables that suggested obstruction of the higher airways. The "myasthenic pattern" was rarely observed in other neuromuscular diseases, except in patients with laryngeal stenosis.O comprometimento respiratório é fator limitante na evolução clinica da miastenia gravis (MG e as formas clínicas mais graves apresentavam acometimento bulbar e respiratório. Para avaliar a reserva respiratória foram examinados em 324 pacientes com MG (forma ocular 62, generalizada 246 e timomatosa 16 as seguintes variáveis da prova de função pulmonar (PFP: capacidade vital forçada (FVC; volume onde o fluxo expiratório é igual a 1 litro por segundo (VF=1; volume expiratório forçado no primeiro segundo (FEV1; fluxo expiratório forçado medido entre 0,2 e 1,2 litros (FEF; fluxo médio expiratório forçado, medido entre 25 e 75% da FVC (FMF; intervalo de tempo entre 25 e 75% da FVC (FMFT; tempo médio de trânsito na expiração forçada (MTT; capacidade pulmonar total (TLC; volume residual (RV; curva fluxo-volume para pesquisa do "padrão miastênico". A análise estatística realizada foi: "t pareado" entre paciente e seu padrão e "t não pareado" entre grupos. Conclusões: Todos os

  19. The progress in immunology and genetics of myasthenia gravis%重症肌无力的免疫学和遗传学进展

    Institute of Scientific and Technical Information of China (English)

    胡学强; 江滢

    2005-01-01

    重症肌无力(Myasthenia Gravis,MG)是乙酰胆碱能运动神经元与骨骼肌之间突触功能障碍性疾病,它是主要影响青壮年的一种自身免疫性疾病,近60%患者在20-40岁之间发病,50岁以后发病的情况少见。据估计,MG的年发病率约为每10万人口0.25—2.00人。

  20. Present situation of the diagnosis and treatment for myasthenia gravis%重症肌无力的诊断与治疗现状

    Institute of Scientific and Technical Information of China (English)

    周水珍

    2007-01-01

    重症肌无力(myasthenia gravis,MG)是一种发生在神经.肌肉接头处,由乙酰胆碱受体(AChR)抗体介导、细胞免疫依赖的获得性自身免疫性疾病。其发病机制与遗传因素、致病性自身抗体、细胞因子、补体参与及胸腺肌细胞等复杂因素有关。

  1. Tolerance to a self-peptide of acetylcholine receptor induces myasthenia gravis%重症肌无力的AChR自身耐受研究进展

    Institute of Scientific and Technical Information of China (English)

    徐秀娟; 黄志

    2005-01-01

    重症肌无力(myasthenia gravis,MG)是抗乙酰胆碱受体(acetycholine receptor,AChR)抗体介导、细胞免疫依赖性、补体参与的神经-肌肉接头处的自身免疫性疾病,是神经肌肉接头处最常见的疾病,其发生的关键在于机体针对AChR所产生的免疫应答异常。

  2. Progress in pathogenesis of myasthenia gravis%重症肌无力发病机制的研究进展

    Institute of Scientific and Technical Information of China (English)

    肖波

    2005-01-01

    重症肌无力(myasthenia gravis,MG)是一种神经肌肉接头(neuromuscular junction,NMJ)传递功能障碍的自身免疫性疾病,其发病机制尚未完全阐明。传统观点认为MG的发病与乙酰胆碱受体自身抗体(AChR-Ab)介导的体液免疫密切相关,但最近发现NMJ处其他抗体、抗原特异性T细胞、细胞因子、调节性T细胞、遗传因素等在MG的发病中也扮演了重要的角色。

  3. New Recognition of Diagnosis and Treatment of Myasthenia Gravis in Children%小儿重症肌无力诊断与治疗的新认识

    Institute of Scientific and Technical Information of China (English)

    黄志; 徐秀娟

    2006-01-01

    重症肌无力(myasthenia gravis,MG)是主要累及神经肌肉接头(neuromuscular junction,NMJ)处突触后膜上乙酰胆碱受体(acetycholine receptor,AChR)的,由其抗体(AChRAb)介导、细胞免疫依赖性、补体参与的自身免疫性疾病。MG也是小儿神经科的一种常见病,本文就近年来小儿MG的诊断与治疗新进展作一简要综述。

  4. A Study of Magnetic Motor Evoked Potentials in the Central Nervous System Dysfunction Model of Myasthenia Gravis%大鼠重症肌无力中枢损害运动诱发电位研究

    Institute of Scientific and Technical Information of China (English)

    张旭; 夏君慧; 孙成超; 叶好好

    2000-01-01

    Objective To explore the relation of acetylcholine receptor antibodies(AChRAb) and dysfunction of central neuvous system (CNS) motor transmission in myasthenia gravis(MG) . Methods IgG purified from MG patient sera was injected into the lateral cerebroventricular system before and after application of motor evoked potential(MEP) examination .Results Intracerebroventricular administration of AChRAb produced loss of MEP wave or prolongation of the latency of the MEP wave,compared to injection of IgG purified from healthy control . Conclusion It is concluded that intracerebroventricular administration of AChRAb causes dysfunction of motor pulse transmission in rats . Present study has further demonstrated that AChRAb cause CNS dysfunction .%目的 探讨乙酰胆碱受体抗体(AChRAb)与中枢神经系统(CNS)运动传导通路之间的关系。方法 从AChRAb阳性的全身型重症肌无力(MG)患者血清中提取纯化IgG,然后注入大鼠侧脑室,观察其对运动诱发电位(MEP)的影响。结果 注入大鼠侧脑室内的AChRAb可致大鼠MEP异常,表现为MEP波消失或潜伏期延长。结论 本研究进一步证实MG患者的AChRAb不仅可以作用于NMJ处的AChR,而且还可作用于大鼠CNS,致CNS运动传导通路功能异常。

  5. 重症肌无力患者血清中Ryanodine受体抗体对其症状学的评估作用%Serum ryanodine receptor antibody on the assessment of clinical symptoms in patients with myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    张祥; 乔健; 吕传真

    2005-01-01

    BACKGROUND: Myasthenia gravis (MG) patients with thymoma were often neglected in clinical work and delayed the therapy.OBJECTIVE: To investigate the significance of the Ryanodine receptor (RyR) antibody on the assessment of MG.DESIGN: A case analysis.SETTING: Institute of neurology in a hospital of a university.PARTICIPANTS: This experiment was carried out in the Institute of Neurology, Fudan University from June 1999 to March 2002. There were 66 MG patients with thymoma(MGT group), 98 MG patients with non-thymoma (NTMG group), 50 patients with non-myasthenia gravis(NMG) and 123 normal persons (NC group).METHODS: Sarcoplasmic reticulum(SR) abounded in RyR was extracted with differential centrifugation, in order to establish a detecting system of ELISA-RyR-RyR antibody (RyR-ab).MAIN OUTCOME MEASURES: The levels of RyR-ab in serum of researched subjects.RESULTS: Positive rate of RyR-ab in MGT group was higher than that in NTMG and NMG groups(P < 0.01), moreover, the sensitivity and the specificity were 81.8% and 94.5% respectively. The positive rates of MGT groups with different thymus histology were no significant difference(P> 0.05). Ages, clinical scores and levels of acetylcholine receptor antibody (AchR-ab) in patients with positive RyR-ab were higher than those in patients with negative RyR-ab( P < 0.01 ) in MG group. The levels of RyR-ab was positive correlated with the severities of clinical symptoms in MG patients, especially the patients in MGT group( r = 0. 626, P < 0.01) . And among the different histological types of MGT, thymoma of epithelioid cells has the highest correlation coefficient ( r = 0. 592, P < 0. 01).CONCLUSION: The detection of RyR-ab has better sensitivity and specificity for the diagnosis of MGT and the levels of RyR-ab is positive correlatied with the severities of MG patients.%背景:临床工作中对伴胸腺瘤重症肌无力患者仍存在漏诊,从而贻误治疗.目的:探讨Ryanodine受体(ryanodine receptor,RyR)抗体在重症肌无力(myasthenia

  6. Clinical characteristics and follow-up management of 135 children with myasthenia gravis%135例儿童重症肌无力患者的临床特点及其治疗后随访

    Institute of Scientific and Technical Information of China (English)

    杨志晓; 林庆; 吴希如; 熊晖; 张月华; 包新华; 姜玉武; 吴晔; 王爽; 常杏芝; 秦炯

    2011-01-01

    Objective : To investigate and analyze the clinical manifestations , classification , therapeutic approaches and follow-up of myasthenia gravis ( MG) in children in order to improve its management and prognosis. Methods : Clinical information of 135 children with MG, who were diagnosed between January 1993 to January 2008 , were collected and retrospectively analyzed. And prospective following-up of these patients were conducted. Results : Among the 135 cases, 59 were males and 76 females, giving the ratio of M/F around 1:1.3. Totally, 115 cases (85. 2% ) were type Ⅰ MG ( ocular type) , of which only 4.2%developed to generalized type during the subsequent clinical course. Type Ⅱ MG ( generalized type ) was found in 18 cases ( 13.4% ) and tpye Ⅲ MG in two cases(1.5% ). The onset age ranged from 5 month to 15 years, with 50. 3% before three years and 80. 7% before seven years. Upper respiratory tract infection was presented in 26. 7% (36/135) of the sick children before the onset of MG. Among the 106 children being followed up, recurrence of the disease identified in 50.9% and the number of relapse ranged from 1 to 9. Altogether, 40. 19% (43/106) of the cases were positive for anti-acetylcholine receptor antibodies (AchR-Ab) on the initial examination, and the AchR-Ab postitive rate showed no difference among different clinical subtypes and states. However, during the follow-up, 53% (9/17) of the recurrent cases, who were negative at the first onset, turned to be positive, and 37. 97% (30/79) were positive for repetitive nerve stimulation in electromyogram test. There were 71 % (45/63) of all the cases showed reduced levels of CD4 + and/or CD3 + and/or CD8 + . Thymus proliferation was found in 5. 93% ( 8/135 ) through CT scan and thymoma in 1. 48% ( 2/135 ) . Steroids and anti-cholinesterase administration were effective in most cases with good prognosis. Conclusion: Childhood MG, mainly type Ⅰ , is relatively common in China, with

  7. 重症肌无力患者胸腺组织中microRNA-27a-3p的表达水平及其临床意义%Altered expression of microRNA-27a-3p in the thymus tissue of patients with myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    李千; 王丽华; 王健健; 张荟雪; 单雪; 孔晓彤

    2016-01-01

    目的 探讨microRNA-27a-3p在重症肌无力(myasthenia gravis,MG)患者胸腺组织中的表达水平并分析其临床意义.方法 收集2004年12月至2015年2月于哈尔滨医科大学附属第二医院胸外科经手术治疗的19例MG患者的胸腺组织标本作为病例组,17例经手术治疗的非MG患者胸腺组织及心外科先天性心脏病患者手术时切取的胸腺组织标本作为对照组,通过实时荧光定量PCR方法检测,采用Wilcoxon秩和检验分析两组标本中microRNA-27 a-3p的表达情况.采用Spearman秩相关分析方法分析MG组胸腺中microRNA-27a-3p的表达水平与定量重症肌无力评分(Quantitative Myasthenia Gravis Score,QMGS)之间的相关性.结果 (1)MG患者胸腺中microRNA-27a-3p的表达水平[0.195(0.049,0.714)]显著高于对照组[0.045 (0.004,0.088);Z=-2.646,P=0.008];(2)19例MG患者中,眼肌型MG(ocular myasthenia gravis,OMG)7例,全身型MG(generalized myasthenia gravis,GMG) 12例,与OMG[0.035(0.008,0.103)]相比,GMG患者胸腺中microRNA-27a-3p的表达[0.493(0.157,1.123)]显著上调(Z=-2.620,P=0.009);(3)microRNA-27a-3p的表达水平与QMGS之间具有正相关性(r=0.576,P=0.010).结论 microRNA-27 a-3p在MG患者胸腺中异常高表达,可能与肌无力的严重程度呈正相关,并与其临床类型有关,但与发病年龄、性别、胸腺病理无明显关联.%Objective To investigate the expression level of thymus microRNA-27a-3p in patients with myasthenia gravis (MG) and to explore the pathogenesis of MG.Methods Thymus tissue samples from 36 cases were collected from December 2014 to February 2015 in the Second Affiliated Hospital of Harbin Medical University.Nineteen thymus tissue samples of MG group were collected from department of chest surgery,17 thymus tissue samples of control group were collected from department of chest surgery or congenital heart disease patients from department of cardiac surgery.The expression of microRNA-27a-3p in the thymus from 36 patients

  8. Efficacy of Qiangli Yiqi Formula on Myasthenia Gravis and Acoustic Evaluation%强力益气方治疗重症肌无力的疗效及声学评价

    Institute of Scientific and Technical Information of China (English)

    盛昭园; 董云; 俞淼青; 应汝炯; 胡粤杭; 陈钢; 李庚和

    2015-01-01

    Objective To study the efficacy of qiangli yiqi formula on myasthenia gravis( MG)and computer acoustic evaluation, Methods Eighty patients of MG of spleen qi deficiency type were divided into a dysarthria group(40 cases)and a non - dysarthria group(40 cases), Each group was subdivided into a male group and a female group,20 cases in each one, The modified qiangli yiqi formula was adopted and the duration of treatment was 6 weeks, Additionally,the healthy voice control subgroups were set up,for males and females separately,20 cases in each one, The absolute score scale,TCM spleen deficiency scale and computer acoustic detection including jitter,shimmer,F0 SD and NNE were selected for the quantitative comparison of efficacy and efficacy assessment before and after treatment separately, Results The total effective rate was 85, 00% in the dysarthria group, The differences in F0 SD,shimmer and NNE were significant as compared with those before treatment (P <0, 05,P <0, 01), The total effective rate was 95, 00% in the non - dysarthria group, The difference in acoustic detection was not apparent, Conclusion Qiangli yiqi formula achieves the definite efficacy on dysarthria in MG patients, The acoustic evaluation method is significant clinically in the diagnosis and efficacy evaluation on MG dysarthria.%目的:研究强力益气方治疗重症肌无力( Myasthenia gravis,MG)的疗效及计算机声学评价。方法将80例脾气虚型重症肌无力( MG)患者,分为MG构音障碍组40例,MG非构音障碍组40例,每组又分为男女两小组,每小组20例。均采用强力益气方加减治疗,疗程6周。另设健康嗓音对照组男女各20例。治疗前后分别选用绝对评分量表、脾气虚型中医症候量表、计算机声学检测包括基频微扰( jitter)、振幅微扰( shimmer)、F0标准差和标准声门噪声能量( NNE),对疗效进行量化比较及疗效评定。结果构音障碍组总有效率85,00%

  9. Toll样受体在重症肌无力患者胸腺组织中的表达及其临床意义%Expression of Toll-like receptors in thymus of myasthenia gravis patients

    Institute of Scientific and Technical Information of China (English)

    高珂; 王允; 王英成; 马小红; 李涛; 伍贮; 刘伦旭; 车国卫; 寇瑛莉; 黄颐

    2008-01-01

    Objective To investigate the expression ofToll-1ike receptors(TLRs)in thymus of myasthenia gravis(MG)patients and the relationship with clinical features.Methods Thymie specimens of 36 patients received extended thymectomy for MG were divided into three groups by pathological type:13 thymoma tissues(thymoma group) and 13 thymie tissues adjacent to thymomas(parathymoma group)from 13 eases of MG patients with thymomas,and 23 thymie tissues from MG patients without thymomas(MG nonthymoma group).Twenty-one normal thymic specimens from cardiac surgery were used as controls.The levels of TLR2-4 mRNA were examined by RT-PCR,then the levels of TLR4 mRNA were assayed by real time RT-PCR and their relationship with chnieal features were analyzed.Results The levels Of TLR4 mRNA among the different groups had significant differences,while there was no difference in TLR2 and nm levels.The real time RT-PCR showed that the level of TLR4 mRNA in nonthymoma group waft significantly higher than that in control group(0.8544t±0.1200 vs0.6851±0.1524.P=0.018).And SO is parathymoma group compared With thc thymoma group(0.8214±0.1019 Vs 0.7101±0.0916,P=0.005).No significant difference of TLR4A level was found between the parathymoma and nonthymoma groups.Nevertheless,the expression of TLR4 in both groups WaS increased compared with control group.The levels of TLR4 mRNA had positive correlation with Osserman type(R=0.609;P=0.004).Conclusion TLR4 may play a key role in the pathogenesis ofMG.It Was the thymie tissues sdjacent to thymomas but not thymomas themselves participated in the onset of MG.%目的 研究Toll样受体(Toll-like receptor,TLRs)在重症肌无力(myasthenia gravis,MG)患者胸腺组织中的表达,分析其与MG发生、发展的关系.方法 收集我科2007年7月至2008年2月手术治疗的36例MG患者胸腺标本,分为胸腺瘤组、瘤旁胸腺组、非瘤MG胸腺组,以无MG的正常胸腺21例为对照(正常胸腺组),采用逆转录-PCR技术,检测TLR2

  10. 机器人胸腺扩大切除术在老年重症肌无力患者中的应用%Robotic-assisted Extended Thymectomy for Myasthenia Gravis in Elderly Patients

    Institute of Scientific and Technical Information of China (English)

    褚剑; 韩冰; 尹东涛; 陈秀; 崔琦; 马孟琦; 刘娜; 隋波

    2013-01-01

    Objective To evaluate the efficacy of robotic-assisted extended thymectomy for myasthenia gravis in elderly patients. Methods From May 2009 to December 2011 , 9 elderly patients with myasthenia gravis, including 4 cases of thymoma, received extended thymectomy by using Da Vinci S surgical system with a double-lumen endobronchial intubation under general anesthesia. All the patients were set at a supine position with one side of the chest rising up by 30°. An 1. 5-cm incision was made on the anterior axillary line, at the fifth intercostal space so that to insert a trocar as a observation hole. And then on the anterior axillary line, at the third intercostal space, and on the midclavicular line, at the sixth intercostal space, two incisions were made ( both were 10 cm away from the observation hole) to introduce the left and fight mechanical arm. Finally, a fourth trocar was placed through an incision on the midaxillary line at the seventh intercostal space, as an assistant operation hole. Artificial pneumothorax was established with a pressure set at 6 - 12 mm Hg. The thymus and surrounding fat tissues were all removed via the assistant operation hole. Results The procedure was completed successfully without conversion to open surgery. The mean anesthesia time was 180 min (ranged from 60 to 210 min) , and the mean time for robotic-assisted procedure was 60 min (ranged from 30 to 110 min) . The mean intraoperative blood loss was 100 ml (ranged from 30 to 200 ml) ,and no patient received blood transfusion. The mean ICU stay of the cases ranged from 1 to 3 days ( mean, 1 day). Nine patients were followed up for 5 to 32 months with a mean of 12 months. According to DeFilippi standards, two patients got grade one, two grade two, three grade three, and the effective rate was 100%. Conclusion Robotic-assited extended thymectomy is effective and safe for selected elderly patients.%目的 评价达芬奇S(da Vinci S)机器人胸腺扩大切除术在老年重症肌

  11. 益气祛湿方治疗重症肌无力的临床疗效及对患者生活质量的影响评价%Evaluate the clinical efficacy of the Yiqi Qushi Decoction in the treatment of myasthenia gravis and its impact on patients' quality of life

    Institute of Scientific and Technical Information of China (English)

    徐向青

    2012-01-01

    目的 观察益气祛湿方治疗重症肌无力的临床疗效及其对患者生活质量的影响.方法 将符合纳入标准的40例重症肌无力(Ⅰ型、Ⅱa 型、Ⅱb型)患者随机分为治疗组和对照组,治疗组给予益气祛湿方,对照组给予溴吡斯的明,疗程均为8周.治疗前后分别对患者进行定量重症肌无力评分(QMG)表、Busch生活质量评价表的问卷调查,观察治疗前后的QMG评分、生活质量的改善情况.结果 治疗后两组QMG量表评分比较差异有统计学意义(P﹤0.05),说明治疗组临床症状的改善优于对照组;治疗后两组Busch生活质量量表总分比较差异有统计学意义(P﹤0.05),说明治疗组对生活质量的改善优于对照组,尤其是在体力功能、情感功能、总生活质量方面的改善均明显优于对照组(P﹤0.01),且未发现不良反应.结论 益气祛湿方不仅可以改善患者的QMG评分,还可以提高患者的生活质量,且无明显不良反应,说明益气祛湿方治疗重症肌无力是一种安全有效的方法.%Objective To observe the clinical efficacy of the Yiqi Qushi Decoction in the treatment of myasthenia gravis and the influence on patients' quality of life. Methods Meeting the inclusion criteria, 40 cases of myasthenia gravis patients with type I, II a and II b were randomly divided into treatment group and control group. The former were treated by Yiqi Qushi Decoction while the control group were given Pyridostigmine Bromide and the therapy lasted 8 weeks. Patients were given "Quantitative myasthenia gravis score (QMG) Table" and "Busch quality of life evaluation form" before and after treatment. The QMG score and improvement on quality of life were Observed. Results After treating, the QMG and Busch quality of life scale score in both groups had significant differences (P < 0.05). It suggested that the improvement on clinical symptoms and life quality received a better efficacy in treatment group, especially in

  12. Ophthalmological affectation: A way to mask Miastenia Gravis. A case purpose.

    Directory of Open Access Journals (Sweden)

    Néstor R. Sánchez Dacal

    2012-06-01

    Full Text Available Myasthenia Gravis MG is an autoimmune and chronic neuromuscular disease characterized by variable of weakness in the skeletal muscles that control the eye movements and it is confused with an ophthalmological disorder. With this presentation we pretend to systematize the Theoretical references about MG which allow making a correct diagnosis of the disease from the experience of a clinical case. The theory about MG regarding the presentation of the disease is discussed, emphasising on the significance of its differential diagnosis with an ophthalmopathy, which will contribute to apply a proper treatment and a satisfactory evolution of the patient, arriving to the conclusion that affectation of the III cranial pair is a way of frequent presentation of MG, being valuable the differential diagnosis of the ophthalmopaties in these entities.

  13. Detection significance of AchR antibody,Titin antibody and MuSK antibody in myasthenia gravis combined with thymic lesion%重症肌无力合并胸腺病变患者血清乙酰胆碱受体抗体、肌联蛋白抗体和肌肉特异性酪氨酸激酶抗体的检测意义

    Institute of Scientific and Technical Information of China (English)

    刘岚剑; 杨文娟

    2016-01-01

    目的:探究对重症肌无力合并胸腺病变的患者血清中乙酰胆碱受体(acetylcholine receptors,AchR)抗体、肌联蛋白(Titin)抗体和肌肉特异性酪氨酸激酶(muscle specific tyrosine kinase,MuSK)抗体的检测意义。方法选取我院收治的80例重症肌无力患者,根据是否存在胸腺病变分为胸腺病变组48例和非胸腺病变组32例,另取80例健康个体作为对照组,通过免疫酶联吸附试验对3组成员血清中 AchR 抗体、Titin 抗体和 MuSK 抗体进行检测并比较。结果重症肌无力患者血清 AchR 抗体和 Titin 抗体阳性率(76.3%,52.5%)均明显高于对照组(11.3%,5.0%)(P <0.05);重症肌无力组及对照组患者血清 MuSK 抗体阳性率均为0,但前者血清中 MuSK 抗体水平明显高于后者(P <0.05);AchR,Titin 两种抗体联合检测可明显提高检测重症肌无力的灵敏度;AchR 和 Titin 抗体在胸腺病变组中的阳性率明显高于非胸腺病变组(P <0.05);全身型重症肌无力患者的血清 AchR 抗体和 Titin 抗体阳性率明显高于眼肌型(P <0.05)。结论 AchR 抗体和 Titin 抗体的阳性率与重症肌无力患者的病情相关,病情严重或合并胸腺病变可明显提高两者的阳性率,此外两者联合检测可提高诊断的灵敏度。而 MuSK 抗体在我国重症肌无力患者中的阳性率较低。%ABSTRACT:Objective To explore the significance of AchR antibody,Titin antibody and MuSK antibody in myasthenia gravis patients combined with thymic lesion.Methods A total 80 cases of myasthenia gravis patients treated in our hospital were selected.They were divided into thymic lesion group(n =48)and no-thymic lesion group (n = 32 ).Another 80 cases of healthy individuals were selected as control group.The positive rates of AchR antibody,Titin antibody and MuSK antibody were compared among three groups and different Osserman types. Results The positive rates of AchR antibody and Titin antibody of myasthenia gravis

  14. Dental Treatment Considerations

    Science.gov (United States)

    ... ask about Dental Treatment Considerations www.myasthenia.org Myasthenia gravis (MG) is an autoimmune neuromuscular disorder that presents ... diagnosis and optimal care of individuals affected by myasthenia gravis and closely related disorders and to improve their ...

  15. 正常及重症肌无力肌肉蛋白成分的比较%Comparison of Protein Components in Normal and Myasthenia Gravis Skeletal Muscles

    Institute of Scientific and Technical Information of China (English)

    任惠民; 周志刚; 陈向军; 黄俊; 吕传真

    2000-01-01

    Aim:To explore whether the protein components have difference between normal and myasthenia gravis(MG)skeletal muscles. Methods: Muscular proteins were extracted from 8 cases of normal and 15 cases of MG biopsy specimenswith PBS solution ,and the components of proteins were analyzed by SDS-PAGE in common and micro methods. Levels of thedifferential protein band in normal and MG muscles were calculated by scanning and densitometry. Two-dimensional elec-trophoresis was used to further determine composition of the differential protein band. Results:Concentration of protein withmass of about 25×103 protein band in the normal muscles was markedly higher than that in the MG muscles,from commonand micro electrophoretic patterns. The density of the protein band ,in the common method ,was 4.20±2.31 and 1.40±0.47in the normal and MG muscles,respectively(P<0.01). Moreover,in the micro method,it was 4.62±1.94 and 1. 66±0.56 inthe normal and MG muscles,respectively (P<0.001). Two-dimensional electrophoresis showed that the 25×103 band con-sisted of two major protein components at least,but the differential protein was a component on the alkaline side in the gel.Conclusion:Concentration of 25×103 protein of the MG muscles were markedly lower than that of the normal. The protein inthe MG muscle could be associated with the pathogeny or developing of MG.%目的:了解正常及重症肌无力(MG)肌肉蛋白成分是否存在差异。方法:用PBS提取正常和MG肌肉的蛋白,以常量及超微量SDS-PAGE分析蛋白成分;扫描计算有差异的谱带密度,并以双向电泳鉴定其组分。结果:正常肌肉有一相对分子质量约25×103的蛋白明显高于MG肌肉,常量分析该谱带密度:正常肌肉为4.20±2.31,MG肌肉为1.40±0.47,差异十分显著(P<0.01)。微量分析该谱带密度:正常肌肉为4.62±1.94MG肌肉为1.66±0.56,差异非常显著(P<0.001)。双向电泳显示25×103谱带至少

  16. 自整合障碍因子1在重症肌无力患者胸腺组织中的表达%Expression of barrier to autointegration factor-1 in thymus of patients with myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    李龙腾; 金湘东; 郭玲霞; 董忠生; 方华; 陈志勇; 张清勇; 高峰

    2014-01-01

    Objective To observe the expression of barrier to autointegration factor-1 (BANF-1) in thymus tissue of myasthenia gravis (MG) patients,and discuss the relationship between BANF-1 and abnormal thymus of MG.Methods The total RNA was extracted by using Trizol from the thymus tissues of MG patients (30 cases of hyperplasia,22 cases of atrophy,and 20 cases of thymoma) and normal thymus (24 cases).The BANF-1 mRNA expression was detected by real-time fluorescent quantitative polymerase chain reaction (FQ-PCR).The expression of BANF-1 protein was detected by immunohistochemical staining.Results There was significant difference in the BANF-1 expression among four kinds of thymic tissues (F =3.627,P <0.05).As compared with normal controls (17.77 ± 13.01),hyperplasia patients (29.58 ±22.81) and thymoma patients (15.99 ± 16.33),the expression of BANF-1 gene in thymus atrophy patients (54.79 ± 35.13) was increased (P < 0.01).Immunohistochemistry revealed the expression of BANF-1 in thymic tissues of hyperplasia,atrophy,and thymoma.Conclusion Differences in the expression level of BANF-1 may be related to the pathogenic mechanisms for abnormal thymus of MG patients,especially associated with MG thymus atrophy.%目的 观察重症肌无力(MG)患者胸腺组织中自整合障碍因子1(BANF-1)的表达,探讨BANF-1与MG胸腺异常的关系.方法 Trizol法提取增生型(30例)、萎缩型(22例)、胸腺瘤型(20例)及正常胸腺(24例)总RNA,实时荧光定量聚合酶链反应(FQ-PCR)检测BANF-1 mRNA含量;免疫组织化学法分析BANF-1蛋白表达.结果 4种胸腺组织类型之间BANF-1 mRNA含量差异有统计学意义(F=3.627,P<0.05).与正常(17.77 ±13.01)、增生型(29.58 ±22.81)和胸腺瘤型(15.99± 16.33)胸腺组织比较,萎缩型胸腺BANF-1基因(54.79 ±35.13)表达增高(P<0.01);免疫组织化学结果显示,MG患者3种类型胸腺组织BANF-1均有表达.结论 BANF-1可能与MG患者异常胸腺的发生有关,尤其与MG胸腺萎缩有关.

  17. Application of anesthesia without muscle relaxant in pediatric myasthenia gravis patients undergoing thymectomy%无肌松剂麻醉在儿童重症肌无力患者胸腺切除术中的应用

    Institute of Scientific and Technical Information of China (English)

    王显望; 王卓强; 王恒林; 王庆东; 刘峰

    2012-01-01

    目的 观察无肌松药麻醉技术对儿童重症肌无力(MG)患者经胸骨胸腺切除围术期及拔管时间的影响,并与肌松药麻醉技术比较.方法 将26例儿童MG患者随机分为肌松药组(M组)和无肌松药组(N组)各13例.所有患者以咪唑安定、丙泊酚和芬太尼麻醉诱导,气道表面麻醉后行气管插管.异丙酚复合七氟醚维持麻醉.M组中麻醉诱导和维持使用少量卡肌宁,N组不使用肌松药.记录两组患者插管条件、血流动力学变化、清醒及拔管时间、术后并发症等.结果 所有患者均顺利插管;两组血流动力学变化比较差异无统计学意义(P>0.05),N组插管后收缩压(SBP)和舒张压(DBP)显著低于麻醉前(P0.05);N组拔管时间显著少于M组(P0.05).结论 无肌松药麻醉技术对儿童MG胸腺切除术患者是一种安全、可靠的麻醉方法,可以缩短患者术后拔管时间,有利于患者迅速恢复.%Objective To compare non-muscle relaxant anesthetic techniques with muscle relaxant anesthetic techniques in pedi-atric myasthenia gravis(MG) patients undergoing trans-sternal thymectomy and evaluate the intra and postoperative conditions including extubation time in the operating room. Methods Twenty-six pediatric patiens with MG were randomly divided into two groups:non-muscle relaxant(N group,n= 13) and muscle relaxant(M group,n= 13). In both groups anesthesia was induced with midazolam,fentanyl and propofol and intubation was performed after topical anesthesia of the airway with lidocaine. Anesthesia was maintained with propofol-sevoflurane. In M group small dose of atracurium was used in induction and maintenance of anesthesia and no neuromuscular blocker in N group. Intubating conditions, hemodynamic changes, wakeup time, extubation time and postoperative complications were evaluated. Results Intubating conditions were good in all patients. There were no significant haemodynamic changes between two groups(P>0. 05). The SBP and

  18. Myeshenia Gravis Presented with Respiratory Failure

    Directory of Open Access Journals (Sweden)

    Vandana Dhangar, Snehal B Patel

    2014-01-01

    Full Text Available A case of 41 year old female known case of depression since 10 years, developed dry cough, low grade fever, breathlessness and drowsiness since 4 days was admitted in ICU and initially diagnosed as type 2 respiratory failure due to pneumonia but on further investigating for altered sensorium patient was found to be NCV positive and was diagnosed as seronegative myasthenia gravis.

  19. Regulatory T-Cells in Chronic Lymphocytic Leukemia and Autoimmune Diseases

    Science.gov (United States)

    D’Arena, Giovanni; Rossi, Giovanni; Vannata, Barbara; Deaglio, Silvia; Mansueto, Giovanna; D’Auria, Fiorella; Statuto, Teodora; Simeon, Vittorio; De Martino, Laura; Marandino, Aurelio; Del Poeta8, Giovanni; De Feo, Vincenzo; Musto, Pellegrino

    2012-01-01

    Regulatory T-cells (Tregs) constitute a small subset of cells that are actively involved in maintaining self-tolerance, in immune homeostasis and in antitumor immunity. They are thought to play a significant role in the progression of cancer and are generally increased in patient with chronic lymphocytic leukemia (CLL). Their number correlates with more aggressive disease status and is predictive of the time to treatment, as well. Moreover, it is now clear that dysregulation in Tregs cell frequency and/or function may result in a plethora of autoimmune diseases, including multiple sclerosis, type 1 diabetes mellitus, myasthenia gravis, systemic lupus erythematosus, autoimmune lymphoproliferative disorders, rheumatoid arthritis, and psoriasis. Efforts are made aiming to develop approaches to deplete Tregs or inhibit their function in cancer and autoimmune disorders, as well. PMID:22973497

  20. Immunoadsorption in patients with autoimmune ion channel disorders of the peripheral nervous system.

    Science.gov (United States)

    Antozzi, Carlo

    2013-01-01

    Autoimmune ion channel disorders of the peripheral nervous system include myasthenia gravis, the Lambert-Eaton myasthenic syndrome, acquired neuromyotonia and autoimmune autonomic ganglionopathies. These disorders are characterized by the common feature of being mediated by IgG autoantibodies against identified target antigens, i.e. the acetylcholine receptor, the voltage-gated calcium and potassium channels, and the neuronal acetylcholine receptor. Moreover, experimental animal models have been identified for these diseases that respond to immunotherapy and are improved by plasmapheresis. On this basis, autoimmune ion channel disorders represent the ideal candidate for therapeutic apheresis. Immunoadsorption can be the treatment of choice when intensive apheretic protocols or long-term treatments must be performed, in patients needing frequent apheresis to keep a stable clinical condition, in case of unresponsiveness to corticosteroids and immunosuppressive treatments, or failure with TPE or intravenous immunoglobulins, and in patients with severe contraindications to long-term corticosteroids.

  1. Autoimmune diseases and HIV infection

    Science.gov (United States)

    Virot, Emilie; Duclos, Antoine; Adelaide, Leopold; Miailhes, Patrick; Hot, Arnaud; Ferry, Tristan; Seve, Pascal

    2017-01-01

    Abstract To describe the clinical manifestations, treatments, prognosis, and prevalence of autoimmune diseases (ADs) in human immunodeficiency virus (HIV)-infected patients. All HIV-infected patients managed in the Infectious Diseases Department of the Lyon University Hospitals, France, between January 2003 and December 2013 and presenting an AD were retrospectively included. Thirty-six ADs were found among 5186 HIV-infected patients which represents a prevalence of 0.69% including immune thrombocytopenic purpura (n = 15), inflammatory myositis (IM) (n = 4), sarcoidosis (n = 4), Guillain–Barré syndrome (GBS) (n = 4), myasthenia gravis (n = 2), Graves’ disease (n = 2), and 1 case of each following conditions: systemic lupus erythematosus, rheumatoid arthritis, autoimmune hepatitis, Hashimoto thyroiditis and autoimmune hemolytic anemia. One patient presented 2 ADs. Thirty patients were known to be HIV-infected when they developed an AD. The AD preceded HIV infection in 2 patients. GBS and HIV infection were diagnosed simultaneously in 3 cases. At AD diagnosis, CD4 T lymphocytes count were higher than 350/mm3 in 63% of patients, between 200 and 350/mm3 in 19% and less than 200/mm3 in 19%. Twenty patients benefited from immunosuppressant treatments, with a good tolerance. ADs during HIV infection are uncommon in this large French cohort. Immune thrombocytopenic purpura, sarcoidosis, IM, and GBS appear to be more frequent than in the general population. Immunosuppressant treatments seem to be effective and well tolerated. PMID:28121924

  2. Myasthenia Gravis: Drugs to be Avoided

    Science.gov (United States)

    ... explanation) on this drug in MG patients. • The fluoroquinolones , including Ciprofloxacin and Levofloxacin – commonly prescribed antibiotics that ... drugs that carry black box warnings include the fluoroquinolones (e.g. Ciprofloxacin , Levafloxacin ; see above) and Telethromycin ( ...

  3. A Difficult and Rare Diagnosis of Autoimmune Enteropathy in a Patient Affected by Down Syndrome.

    Science.gov (United States)

    Depince-Berger, Anne; Cremilieux, Clara; Rinaudo-Gaujous, Melanie; Genin, Christian; de Freminville, Benedicte; Lambert, Claude; Bruneau, J; Paul, Stephane

    2016-07-01

    Patients with Down syndrome are more susceptible to autoimmune pathologies, in particular endocrine or digestive diseases such as celiac disease. Autoimmune enteropathy is another form of digestive autoimmune disease, non-gluten-dependant, more often diagnosed in male neonates with immunodysregulation and polyendocrinopathy such as the Immunodysregulation, Polyendocrinopathy, Enteropathy, X-linked syndrome. It also exists in the adult, but this pathology is less known and therefore frequently under-diagnosed. Clinical manifestations are similar to celiac disease, but not improved after a gluten-free diet. Autoimmune enteropathy is frequently associated with other autoimmune diseases, such as thyroiditis, myasthenia gravis, lupus or immune deficiencies, as Common Variable Immunodeficiency. Pathological analysis of intestinal biopsies can frequently distinguish autoimmune enteropathy and celiac disease. Autoimmune enteropathy usually has an important lymphoplasmacytic infiltration of the mucosa and a lack of intraepithelial lymphocytes in the gastrointestinal mucosal surface, while celiac disease usually has a polymorph infiltration of the mucosa and an important intraepithelial lymphocytes infiltration. Nevertheless, the two pathological patterns may overlap. Here we report the first case of a patient with Down syndrome associated to autoimmune enteropathy (initially diagnosed as celiac disease), chronic pancreatitis and cutaneous lupus erythematosus. Even if autoimmune pathologies are much more common in patients with Down syndrome, we would like to report on this rare and original association found in our patient.

  4. Determinação imunohistoquímica da presença de células mióides em pacientes submetidos à timectomia Determination of the presence of myoid cells in the thymus by immunohistochemistry after thymectomy for myasthenia gravis

    Directory of Open Access Journals (Sweden)

    Olanrewaju Muisi Adedamola Ladipo

    2004-08-01

    Full Text Available OBJETIVO: Detectar e quantificar células mióides em timos de pacientes com miastenia grave, estabelecendo possível correlação entre a quantidade de células mióides com variáveis demográficas e clínico-patológicas. MÉTODO: Foram analisados por meio de método imuno-histoquímico com anticorpo antidesmina (clone D33; marca Dako, timos de 22 pacientes (16 mulheres e seis homens, entre 12 e 61 anos submetidos à timectomia, entre 1981 e 1995, no Serviço de Cirurgia Torácica do Hospital Heliópolis como parte do tratamento de miastenia grave. RESULTADOS: As maiores médias de células mióides foram encontrados em timos dos pacientes da raça negra (29,4:17,8, do sexo feminino (23,2:13,0 e com faixa etária entre 60 e 80 anos (média de 33,0. Pela classificação clínica da Fundação de Miastenia Grave da América (MGFA, a maior média de células mióides (26,7 encontra-se na classe IIIa, sendo do tipo histológico de hiperplasia verdadeira (média 42,0. As células mióides foram identificadas em 11 timos com hiperplasia linfóide, três hiperplasias verdadeiras e em quatro timos normais. Os timomas malignos (três e um timo normal não apresentaram células mióides. CONCLUSÕES: As células mióides podem ser identificadas e quantificadas pelo método imuno-histoquímico com anticorpo antidesmina, porém não existe correlação entre a quantidade de células mióides e as variáveis demográficas, clínico-patológicas. Elas não foram identificadas no timoma fusocelular.BACKGROUND: To detect and quantify myoid cells in thymus of patients with myasthenia gravis, establishing possible correlation between the amount of myoid cells and demographics, clinics, and pathological variables. METHODS: thymic specimens of 22 patients (16 women and six men, age ranging from 12 to 61 years, were analyzed through immunohistochemistry with monoclonal antibody anti- desmin (Dako - D33. They were submitted to tymectomy, between 1981 and 1995, in

  5. Susceptible mechanisms of extraocular muscles in the passive transferred experimental myasthenia gravis rats%重症肌无力被动转移大鼠模型眼外肌的易感机制研究

    Institute of Scientific and Technical Information of China (English)

    刘睿; 王桂平; 杜婴; 周琼; 苗建亭; 李柱一

    2012-01-01

    目的 探讨眼外肌在重症肌无力发病过程中的易感机制.方法 给予SD大鼠腹腔注射mAb35建立重症肌无力被动转移(PTMG)大鼠模型,对照组大鼠注射等量生理盐水.选取PTMG组和对照组大鼠眼外肌、膈肌、胫前肌3种骨骼肌组织.采用乙酰胆碱酯酶(AChE)染色法观察神经肌肉接头(NMJ)并检测NMJ面积和灰度;采用银环蛇毒免疫组化法检测乙酰胆碱受体( AChR)数量;采用电镜观察NMJ超微结构和其AChR情况,并分析比较神经末端面积和突触后膜面积的比值以及突触前后膜长度的比值.结果 AChE染色结果显示,对照组眼外肌NMJ面积相对其他两种骨骼肌更小(P<0.01),PTMG组眼外肌与其他两种骨骼肌NMJ面积比较无统计学差异(P>0.05).银环蛇毒免疫组化结果显示,PTMG组和对照组眼外肌与其它两种骨骼肌间AChR灰度值比较均有统计学差异(P<0.01).电镜观察结果显示,PTMG组3种骨骼肌突触前后膜长度比值均较对照组下降(P<0.01),神经末端面积与突触后膜面积比值较对照组增加(P<0.01),其中眼外肌的变化较其他骨骼肌更为显著.结论 PTMG大鼠模型眼外肌易感机制可能与眼外肌和其他骨骼肌间NMJ面积、AChR数量差异造成眼外肌NMJ安全系数较低有关.%Objective To investigate the susceptible mechanisms of extraocular muscles in passive transferred experimental myasthenia gravis ( PTMG) rats. Methods PTMG model was induced by intraperitoneally injection of purified monoclonal antibody 35. The control group were intraperitoneally injected with normal saline. The extraocular muscles, diaphragms and tibial front muscles in the PTMG group and control group were dissected. Acetylcholinesterase staining, which showed neuromuscular junctions, and alpha bungarotoxin immunohistochemical reaction, which showed acetylcholine receptors (AChR) were used. Neuromuscular junction (NMJ) ultrastructure was observed by transmission

  6. B cells in the pathophysiology of autoimmune neurological disorders: a credible therapeutic target.

    Science.gov (United States)

    Dalakas, Marinos C

    2006-10-01

    There is evidence that B cells are involved in the pathophysiology of many neurological diseases, either in a causative or contributory role, via production of autoantibodies, cytokine secretion, or by acting as antigen-presenting cells leading to T cell activation. Clonal expansion of B cells either in situ or intrathecally and circulating autoantibodies are critical elements in multiple sclerosis (MS), Devic's disease, paraneoplastic central nervous system disorders, stiff-person syndrome, myasthenia gravis, autoimmune demyelinating neuropathies and dermatomyositis. The pathogenic role of B cells and autoantibodies in central and peripheral nervous system disorders, as reviewed here, provides a rationale for investigating whether depletion of B cells with new agents can improve clinical symptomatology and, potentially, restore immune function. Preliminary results from several clinical studies and case reports suggest that B cell depletion may become a viable alternative approach to the treatment of autoimmune neurological disorders.

  7. B cells as therapeutic targets in autoimmune neurological disorders.

    Science.gov (United States)

    Dalakas, Marinos C

    2008-10-01

    B cells have a fundamental role in the pathogenesis of various autoimmune neurological disorders, not only as precursors of antibody-producing cells, but also as important regulators of the T-cell activation process through their participation in antigen presentation, cytokine production, and formation of ectopic germinal centers in the intermeningeal spaces. Two B-cell trophic factors-BAFF (B-cell-activating factor) and APRIL (a proliferation-inducing ligand)-and their receptors are strongly upregulated in many immunological disorders of the CNS and PNS, and these molecules contribute to clonal expansion of B cells in situ. The availability of monoclonal antibodies or fusion proteins against B-cell surface molecules and trophic factors provides a rational approach to the treatment of autoimmune neurological diseases. This article reviews the role of B cells in autoimmune neurological disorders and summarizes the experience to date with rituximab, a B-cell-depleting monoclonal antibody against CD20, for the treatment of relapsing-remitting multiple sclerosis, autoimmune neuropathies, neuromyelitis optica, paraneoplastic neurological disorders, myasthenia gravis, and inflammatory myopathies. It is expected that ongoing controlled trials will establish the efficacy and long-term safety profile of anti-B-cell agents in several autoimmune neurological disorders, as well as exploring the possibility of a safe and synergistic effect with other immunosuppressants or immunomodulators.

  8. 年龄对重症肌无力患者顺式阿曲库铵肌松效应的影响%Influence of age on neuromuscular block effect of cisatracurium in myasthenia gravis patients

    Institute of Scientific and Technical Information of China (English)

    贾瑞芳; 周淑珍; 张宏业; 左明章

    2015-01-01

    目的 观察重症肌无力(MG)患者术中应用顺式阿曲库铵的肌松效应,比较老年MG患者与中青年MG患者顺式阿曲库铵肌松效应的差别. 方法 择期胸腔镜下行胸腺切除术的MG患者,根据年龄分为老年组(≥65岁)和中青年组(20~50岁).其中老年组Ⅱb型MG有15例,从中青年组中根据性别、病情、病程、术前治疗情况、麻醉用药、手术时间配对选择Ⅱb型MG患者15例.麻醉诱导期用TOF Watch SX肌松监测仪行拇内收肌肌松监测,静注顺式阿曲库铵,首剂量从0.05mg/kg[1倍95%有效剂量(ED95)]开始,根据TOF值追加顺式阿曲库铵0.015 mg/kg,直到T1/T0<95%时实施双腔气管支气管导管插管.术中当T4/T1达到25%时追加0.015 mg/kg顺式阿曲库铵.观察顺式阿曲库铵的插管剂量和作用时间、临床作用持续时间、术毕TOFr(T4/T1)恢复到25%、70%和90%的时间. 结果 老年MG组和中青年MG组顺式阿曲库铵的插管剂量和首剂量的作用持续时间差异无统计学意义(均P>0.05);术中老年MG组顺式阿曲库铵的临床作用时间(21.6±6.7)min,较中青年MG组顺式阿曲库铵的临床作用时间(33.7±13.4)min短(t=-2.139,P=0.045);老年MG组术中追加顺式阿曲库铵(3.9±1.3)次,多于中青年MG组术中追加次数(2.3±2.2)次(t=2.601,P=0.025);术毕中老年组TOFr恢复到25%的时间(22.1±6.9)min短于中青年MG组TOFr恢复到25%的时间(34.0±18.3)min(t=-2.139,P=0.037);两组患者术毕TOFr恢复到70%和90%的时间差异无统计学意义(均P>0.05). 结论 老年MG患者的顺式阿曲库铵阻滞时间较中青年MG患者有不同程度的缩短;有必要进行更多的相关研究来进一步明确年龄对MG患者顺式阿曲库铵以及其他肌松药肌松效应的影响.%Objective To investigate the neuromuscular block effect of intravenous injection of cisatracurium in myasthenia gravis patients with different ages.Methods Fifteen geriatric patients

  9. Animal models of antimuscle specific kinase myasthenia

    Science.gov (United States)

    Richman, David P.; Nishi, Kayoko; Ferns, Michael J.; Schnier, Joachim; Pytel, Peter; Maselli, Ricardo A.; Agius, Mark A.

    2014-01-01

    Antimuscle specific kinase (anti-MuSK) myasthenia (AMM) differs from antiacetylcholine receptor myasthenia gravis in exhibiting more focal muscle involvement (neck, shoulder, facial, and bulbar muscles) with wasting of the involved, primarily axial, muscles. AMM is not associated with thymic hyperplasia and responds poorly to anticholinesterase treatment. Animal models of AMM have been induced in rabbits, mice, and rats by immunization with purified xenogeneic MuSK ectodomain, and by passive transfer of large quantities of purified serum IgG from AMM patients into mice. The models have confirmed the pathogenic role of the MuSK antibodies in AMM and have demonstrated the involvement of both the presynaptic and postsynaptic components of the neuromuscular junction. The observations in this human disease and its animal models demonstrate the role of MuSK not only in the formation of this synapse but also in its maintenance. PMID:23252909

  10. 不同种类抗生素影响重症肌无力小鼠神经肌肉接头处的传递功能%Effect of different kinds of antibiotics on transmission function at neuromuscular junction in mice with myasthenia gravis

    Institute of Scientific and Technical Information of China (English)

    邓敏; 王云甫; 胡芳; 吕永宁; 刘昌勤; 曹学兵; 杨汉菊; 徐楚鸿

    2005-01-01

    BACKGROUND: It is recently found that some kinds of antibiotics can aggravate the obstruction of neuromuscular junction(NM J) transmission,exacerbate myasthenia gravis (MG). Hitherto, there are few reports about the effect of antibiotics on transitive function on animal models. Along with the appearance of new antibiotics, the effects of the antibiotics on NMJ transitive function need to be further observed.OBJECTIVE: To investigate the effect of aminoglycoside antibiotics, fluoroquinolone antibiotics and cephalosporin antibiotics on the transitive function of NMJ in MG, and to provide an experimental basis for using those antibiotics securely in clinic and for selecting those antibiotics to treat MG properly.DESIGN: Randomized controlled study based on experimental animals.SETTING: Department of nosocomial infection, neurology and pharmacy in a university hospital.MATERIALS: The experiment was conducted at the Neurological Institute of Tongji Medical College, Huazhong University of Science and Technology from March 2002 to January 2003. Totally 150 healthy female C57BL/6mice, 6 - 8 weeks old, weighting 18 - 20 g, were divided randomly into 4groups: normal group( n = 10), MG group( n = 10), saline group( n = 10)and antibiotics group( n = 120) . Mice in antibiotics group were divided randomly again into gentamicin group, etimicin group, ciprofloxacin group,fleroxacin group, cefuroxime group and cephradine group, with 20 mice in each group.INTERVENTIONS: C57BL/6 mice were immunized with the acetylcholine receptor(AChR) protein in complete Fruend' s adjuvant(CFA) to establish experimental autoimmune myasthenia gravis(EAMG) . Mice in saline group were injected normal saline and mice in antibiotics group were injected antibiotics(10 mg/kg), lasted 14 days. Mice in MG group were without any treatments. On the 7th day after the last immunization and the 14th day after the antibiotics treatments, MG scores was evaluated, repetitive nerve stimulation(RNS) and the levels of

  11. Allergy and Immunology

    Institute of Scientific and Technical Information of China (English)

    2009-01-01

    2009037 Passive transfer/of experimental autoimmune myasthenia gravis in rabbits with IgG of myasthenia gravis patients. LI Xiuhua(李秀华). et al. Neurol Dept, Tianjin Med Univ General Hosp, Tianjin 300052. Chin J Ment Nerv Dis 2009;35(1):7-10. Objective To create experimental autoimmune myasthenia gravis (EAMG) rabbits by passive transfer the IgG.

  12. Efficacy of Video-assisted Thoracoscopic Thymectomy for Myasthenia Gravis and the Factors Influencing the Outcome%胸腔镜胸腺扩大切除术治疗重症肌无力的疗效观察及影响因素分析

    Institute of Scientific and Technical Information of China (English)

    崔健; 李剑锋; 周足力; 黄宇清; 陈应泰; 孟宪璞; 刘军; 王俊

    2012-01-01

    目的 对胸腔镜胸腺扩大切除术治疗重症肌无力( myasthenia gravis,MG)的疗效进行总结,分析影响MG手术疗效的因素. 方法 回顾性研究我院2000年6月~2009年10月47例MG接受胸腔镜手术的临床资料,采用单因素分析和logistic回归模型对患者性别、年龄、病程、MGFA分型、胸腺病理类型等可能影响因素进行分析. 结果 45例完成随访,随访16~111个月,平均51个月.根据MGFA提出的治疗后状况分类:完全稳定缓解占64.4% (29/45),药物缓解占17.8%( 8/45),微小症状表现占8.9%(4/45),恶化占2.2%( 1/45),复发占4.4% (2/45),死亡占2.2% (1/45).年龄(β=0.059,P =0.027,OR =1.060,95%CI为1.007 ~1.117)和胸腺病理类型(β=1.558,P=0.025,OR=4.750,95% CI为1.214~18.584)是影响手术疗效的因素. 结论 MG行电视胸腔镜下胸腺扩大切除术疗效满意,年龄和胸腺病理类型是手术疗效的影响因素.%Objective To assess the efficacy of and video-assisted thoracoscopic thymectomy in the treatment of myasthenia gravis (MG) and the factors influencing the results. Methods A total of 47 patients with MG, who underwent video-assisted thoracoscopic thymectomy in our hospital from June 2000 to, October 2009, were enrolled in this retrospective study. The sex, age, course of disease, MGFA classification, and pathological type of thymus of the patients were analyzed with univariate and logistic regression analyses. Results Follow-up for a mean of 51 months (ranged from 16 to 111 months) was achieved in 45 of the 47 patients. Therapeutic regimen was determined based on the MGFA for the patients. After the treatment, 29 of the patients achieved complete stable remission (64.4% ) , 8 had pharmacologic remission (17.8% ) , 4 still had minimal manifestations (8.9% ) , 1 was deteriorated (2.2% ) , 2 showed recurrent MG (4.4% ) , and 1 patient died (2. 2% ). Age and pathological type of thymus were main factors influencing the outcomes of the

  13. Therapeutic gymnastics in comprehensive treatment of patients with generalized myasthenia

    Science.gov (United States)

    Kapelovich, R. L.

    1980-01-01

    The technique of therapeutic gymnastics was used for patients with mayasthenia gravis to control the consequences of hypodynamia induced by the myasthenic process. It is concluded that during myasthenia, the severity of the disease is due to the affection of the cross striated musculature. The most life threatening are the disorders in respiration and swallowing, that can be intensified by forced stay in bed and immobility. It is also concluded that the use of therapeutic gymnastics in patients which myasthenia promotes efficient presurgical preparation, and in the post surgical period; prevention of pulmonary complications and normalization of respiration. Therapeutic gymnastics with regard to the severity and localization of the myasthenic disorders must be a component part of the presurgical preparation and postsurgical management of patients with generalized myasthenia.

  14. Attach importance to clinical diagnosis and strandard treatment for myasthenia gravis%应重视重症肌无力的临床诊断和规范化治疗

    Institute of Scientific and Technical Information of China (English)

    崔丽英

    2007-01-01

    重症肌无力(myasthenia gravis,MG)是累及骨骼肌神经肌肉接头处的自身免疫性疾病,临床诊断主要依据活动后加重,休息后减轻或晨轻暮重的骨骼肌无力,以及神经电生理检查和血清乙酰胆碱受体抗体(AChR-ab)异常等特点。正确的诊断是合理治疗的前提。目前国内MG的诊断和治疗现状尚不能令人完全满意。

  15. 重肌灵冲剂治疗眼肌型重症肌无力的临床研究%Clinical Study on Treating Ocular Myasthenia Gravis with Zhongjiling Granule

    Institute of Scientific and Technical Information of China (English)

    许凤全; 吴以岭

    2004-01-01

    重症肌无力(myasthenia gravis,MG)是一种主要累及神经肌肉接头处突触后膜上乙酰胆碱受体(AchR)的,由特异性抗体nAchRab介导的细胞免疫起重要作用且补体参与的自身免疫性疾病。其特点包括:①临床上表现为活动后加重,休息后减轻、朝轻暮重的骨骼肌无力;②电生理上表现为低

  16. Miastenia Gravis

    OpenAIRE

    Martins, Cecília; Marques, Eduarda; Santos, Fátima; Gomes,Roseli; A. Pereira, Susana

    2014-01-01

    Introdução: A miastenia gravis é uma doença neuro-muscular com três formas de apresentação: neonatal transitória, congénita ou juvenil. O diagnóstico é sugerido pela história, exame físico, teste anti-colinesterase, electromiografia e anticorpos específicos. A imagiologia torácica pode ser importante pela associação frequente desta patologia com anomalias tímicas. O tratamento deve ser individualizado.Caso clínico: Apresenta-se o caso de uma criança do sexo masculino de sete anos de idade com...

  17. Risk of non-melanoma skin cancer in myasthenia patients treated with azathioprine

    DEFF Research Database (Denmark)

    Pedersen, E G; Pottegård, A; Hallas, J

    2014-01-01

    The association between use of azathioprine and risk of non-melanoma skin cancer (NMSC) in patients with myasthenia was evaluated in a nationwide setting. Treatment of autoimmune myasthenia frequently involves long-term exposure to immunosuppressants, including azathioprine. Use of azathioprine...

  18. Practical considerations on the use of rituximab in autoimmune neurological disorders

    Science.gov (United States)

    Kosmidis, Mixalis L.; Dalakas, Marinos C.

    2010-01-01

    Rituximab (Mabthera, Rituxan) is a chimeric human/murine monoclonal antibody against CD-20 surface antigen expressed on B-cells. Rituximab, by causing B-cell depletion, appears to be effective in several autoimmune disorders; it has been approved for rheumatoid arthritis and is a promising new agent in the treatment of several autoimmune neurological disorders. A controlled study in patients with relapsing remitting multiple sclerosis has shown that rituximab significantly reduces the number of new MRI lesions and improves clinical outcome; it also showed some promise in a subset of patients with primary progressive MS. The drug is also effective in a number of patients with Devic’s disease, myasthenia gravis, autoimmune neuropathies, and inflammatory myopathies. The apparent effectiveness of rituximab has moved B-cells into the center stage of clinical and laboratory investigation of autoimmune neurological disorders. We review the evidence-based effectiveness of rituximab in neurological disorders based on controlled trials and anecdotal reports, including our own experience, and address the immunobiology of B-cells in autoimmune central nervous system (CNS) and peripheral nervous system (PNS) disorders. In addition, we provide practical guidelines on how best to use this drug in clinical practice and highlight its potential toxicity. PMID:21179602

  19. IVIg in other autoimmune neurological disorders: current status and future prospects.

    Science.gov (United States)

    Dalakas, Marinos

    2008-07-01

    A number of autoimmune disorders have been identified in which IVIg treatment may be beneficial. Evidence for the use of IVIg in inflammatory myopathies comes from controlled trials in dermatomyositis (DM) and sporadic-inclusion body myositis (s-IBM). In DM, muscle strength was increased and neuromuscular scores and skin rashes improved. Results for s-IBM have not been as encouraging as those observed for DM. Subsequently, IVIg should be recommended as a second-line therapy in DM and used for life-threatening dysphagia in s-IBM. Using an animal model of experimental autoimmune myasthenia gravis (MG), studies also indicate that IVIg can significantly improve clinical symptoms and affect pathogenic idiotypic antibodies. In human MG, studies indicate that IVIg exhibited equal efficacy compared to plasmapheresis. IVIg can therefore be recommended for use in an MG crisis or in lieu of plasmapheresis. The role of IVIg in the chronic management of MG has not been studied. IVIg has also been investigated in autoimmune CNS disorders. In a controlled study in patients with stiff person syndrome IVIg was effective, with improvements in the distribution of stiffness index and heightened sensitivity scores. For neurodegenerative diseases such as Alzheimer's disease, post-polio syndrome, pain, fibrosis, and autoimmune sleep disorders, some early promising results for the use of IVIg are emerging, but remain to be fully investigated. In conclusion, IVIg appears to be an effective treatment for a number of autoimmune disorders, however, optimal dosing and pharmacogenetic studies are necessary.

  20. Practical considerations on the use of rituximab in autoimmune neurological disorders.

    Science.gov (United States)

    Kosmidis, Mixalis L; Dalakas, Marinos C

    2010-03-01

    Rituximab (Mabthera, Rituxan) is a chimeric human/murine monoclonal antibody against CD-20 surface antigen expressed on B-cells. Rituximab, by causing B-cell depletion, appears to be effective in several autoimmune disorders; it has been approved for rheumatoid arthritis and is a promising new agent in the treatment of several autoimmune neurological disorders. A controlled study in patients with relapsing remitting multiple sclerosis has shown that rituximab significantly reduces the number of new MRI lesions and improves clinical outcome; it also showed some promise in a subset of patients with primary progressive MS. The drug is also effective in a number of patients with Devic's disease, myasthenia gravis, autoimmune neuropathies, and inflammatory myopathies. The apparent effectiveness of rituximab has moved B-cells into the center stage of clinical and laboratory investigation of autoimmune neurological disorders. We review the evidence-based effectiveness of rituximab in neurological disorders based on controlled trials and anecdotal reports, including our own experience, and address the immunobiology of B-cells in autoimmune central nervous system (CNS) and peripheral nervous system (PNS) disorders. In addition, we provide practical guidelines on how best to use this drug in clinical practice and highlight its potential toxicity.

  1. 危重型重症肌无力病人术后危象的预防及护理%Prevention and nursing care of post-operative articulo in severe myasthenia gravis patients

    Institute of Scientific and Technical Information of China (English)

    潘佩珍; 唐白云; 谭妙娜; 刘雅玲; 郑莹

    2006-01-01

    [目的]探讨危重型重症肌无力(myadthenia gravis,MG)围手术期病人护理的方法,以降低危象的发生率.[方法]将53例Ⅲ型、Ⅳ型危重型病人术后按不同方法处理,分为治疗组33例和对照组20例,治疗组病人用甲基强的松龙(MP)20 mg/(kg·d)和环磷酰胺(CTX)0.25 g/(m2·d)而对照组术毕静脉仅用地塞米松0.40mg/(kg·d),两组均连用3 d.两组同时采用"干涸"治疗.[结果]治疗组危象发生率为9.09%,对照组50.00%.[结论]做好充分的术前准备,加强心理护理,做好用药指导,和"干涸"治疗及免疫冲击治疗过程中的护理观察对疾病的恢复有重要作用.

  2. Comparison of medium-term efficacy and quality of life after extended thymectomy for nonthymomatous myasthenia gravis between video-assisted thoracoscopy and median sternotomy%电视胸腔镜与胸骨正中切口行胸腺扩大切除治疗非胸腺瘤重症肌无力中期疗效和生活质量比较

    Institute of Scientific and Technical Information of China (English)

    张凯; 崔键; 赵桂彬

    2011-01-01

    [Objective] To investigate the value of video-assisted thoracoscopic surgery (VATS) in the treatment of nonthymomatous myasthenia gravis (NTMG) by comparing the medium-term efficacy and quality of life after extensive thymectomy of VATS and median sternotomy. [Methods] A retrospective analysis on 52 cases of NTMG, who were preoperatively confirmed to be NTMG from May 2003 to June 2006 were divided into VATS group (26 cases) and conventional thoracotomy group (26 cases); Clinical efficacy was measured by DeFlippi class for changes in myasthenia gravis-associated symptoms and MGFA score, quality of life was studied by modified the European Organization for Research and Treatment of Cancer (EORTC) quality-of-life questionnaire (QLQ) at 3-years of the follow-up. [ Results ] 1 cases occurred muscle weakness crises in open-chest group, but none in VATS group. At 3-years of the follow-up, symptoms improved of patients with VATS better than open-chest group (P 0.05), six item score of EORTC-QLQ (Physical, Role, Cognitive, Emotional, Social, Global ability) of VATS group is superior over conventional group (P >0.05). [Conclusions] VATS thymectomy can be regarded as the treatment of choice for patients undergoing surgery for nonthymomatous myasthenia gravis, it will has a wonderful applied future of clinic.%目的 比较电视胸腔镜手术(VATS)与胸骨正中切口胸腺扩大切除治疗非胸腺瘤重症肌无力(NT-MG)的中期疗效和生活质量,以合理选择手术方式.方法 回顾性分析该科2003年5月~2006年6月行胸腺扩大切除治疗NTMG 52例,根据手术方式的不同分为VATS组(n=26)和开胸组(n=26),术后3年随访时采用术后MG相关症状变化DeFilippi分级和美国重症肌无力基金会MG评分(MGFA评分)分析术后疗效.采用欧洲癌症研究和治疗组织(EORTC)生活质量问卷(QLQ)评估术后生活质量.结果 开胸组发生肌无力危象1例,VATS组无发生.术后3年随访时MG相关症状变化DeFilippi分

  3. Characterization of the in vitro expressed autoimmune rippling muscle disease immunogenic domain of human titin encoded by TTN exons 248-249

    Energy Technology Data Exchange (ETDEWEB)

    Zelinka, L. [Biomedical Sciences Program, Kent State University, Kent, OH (United States); McCann, S.; Budde, J.; Sethi, S.; Guidos, M.; Giles, R. [Center for Applied Chemical Biology, Department of Biological Sciences, Youngstown State University, One University Plaza, Youngstown, OH 44555 (United States); Walker, G.R., E-mail: grwalker@ysu.edu [Center for Applied Chemical Biology, Department of Biological Sciences, Youngstown State University, One University Plaza, Youngstown, OH 44555 (United States); Biomedical Sciences Program, Kent State University, Kent, OH (United States)

    2011-08-05

    Highlights: {yields} Affinity purification of the autoimmune rippling muscle disease immunogenic domain of titin. {yields} Partial sequence analysis confirms that the peptides is in the I band region of titin. {yields} This region of the human titin shows high degree of homology to mouse titin N2-A. -- Abstract: Autoimmune rippling muscle disease (ARMD) is an autoimmune neuromuscular disease associated with myasthenia gravis (MG). Past studies in our laboratory recognized a very high molecular weight skeletal muscle protein antigen identified by ARMD patient antisera as the titin isoform. These past studies used antisera from ARMD and MG patients as probes to screen a human skeletal muscle cDNA library and several pBluescript clones revealed supporting expression of immunoreactive peptides. This study characterizes the products of subcloning the titin immunoreactive domain into pGEX-3X and the subsequent fusion protein. Sequence analysis of the fusion gene indicates the cloned titin domain (GenBank ID: (EU428784)) is in frame and is derived from a sequence of N2-A spanning the exons 248-250 an area that encodes the fibronectin III domain. PCR and EcoR1 restriction mapping studies have demonstrated that the inserted cDNA is of a size that is predicted by bioinformatics analysis of the subclone. Expression of the fusion protein result in the isolation of a polypeptide of 52 kDa consistent with the predicted inferred amino acid sequence. Immunoblot experiments of the fusion protein, using rippling muscle/myasthenia gravis antisera, demonstrate that only the titin domain is immunoreactive.

  4. Pharmacodynamics of remifentanil blunting responses to tracheal intubation performed under peopofol anesthesia administered by TCI In patients with myasthenia gravis%复合靶控输注异丙酚时瑞芬太尼抑制重症肌无力患者气管插管反应的药效学

    Institute of Scientific and Technical Information of China (English)

    石好; 左明章; 孟小燕; 闫春伶; 杨宁

    2009-01-01

    目的 探讨复合靶控输注异丙酚时瑞芬太尼抑制重症肌无力患者气管插管反应的药效学.方法 拟行经胸骨正中劈开胸腺切除术的重症肌无力患者45例,ASA Ⅰ或Ⅱ级,TCI异丙酚和瑞芬太尼进行麻醉诱导,异丙酚血浆靶浓度为4 μg/ml,瑞芬太尼初始效应室靶浓度(Ce)为1.8 ng/ml,依次按2.7、4.0、6.0 ng/ml梯度递增,至患者可耐受喉麻管置入声门下进行气管内表麻后,进行气管插管.采用概率单位回归分析法分别计算瑞芬太尼抑制气管插管反应的Ce50和Ce95.结果 瑞芬太尼抑制气管插管反应的Ce50为2.2 ng/ml,其95%可信区间为2.0~2.3 ng/ml;抑制气管插管反应的Ce95为3.0 ng/ml,其95%可信区间为2.8~3.5 ng/ml.结论 复合靶控输注异丙酚(血浆靶浓度为4 μg/ml)时,瑞芬太尼抑制重症肌无力患者气管插管反应的Ce50和Ce95分别为2.2、3.0 ng/ml.%Objective To investigate the pharmacodynamies of remifentanil blunting the responses to tracheal intubation performed under propofol anesthesia in patients with myasthenia gravis. Methods Forty-five ASA Ⅰ or Ⅱ patients with myasthenia gravis aged 18-64 yr weighing 45-95 kg scheduled for trans-stemal thymectomy were enrolled in this study. ECG, BP, HR, SpO2 and BIS were monitored. Anesthesia was induced with propofol and remifentanil both administered by TCI. The target plasma concentration (Cp) of propofol was set at 4 μg/ml during induction. The initial target effect-site concentration (Ce) of remifentanil was set at 1.8 ng/ml and was increased step by step to 2.7, 4.0 and 6.0 ng/ml until the patients could tolerate laryngoscopy and insertion of larynx unesthetic tube into subglottic space without body movement, coughing or bucking. Topical anesthesia of trachea was then performed by spraying 2% lidocaine 2 ml through the larynx anesthetic tube. Laryngoscope and larynx anesthetic tube were removed and the patients were ventilated with O2 through anesthetic mask

  5. Response to thymectomy and analysis of influencing factors in the treatment of children with myasthenia gravis%胸腺手术治疗儿童重症肌无力的疗效随访及影响因素分析

    Institute of Scientific and Technical Information of China (English)

    李言; 刘卫彬; 罗传铭; 冯慧宇; 黄鑫; 王海燕; 邱力; 黄如训

    2012-01-01

    目的 通过长期的随访评价胸腺扩大切除术治疗儿童重症肌无力的疗效与相关影响因素.方法 回顾性分析2003年1月至2009年8月在中山大学附属第一医院行胸腺扩大切除术的59例儿童重症肌无力患儿临床资料及长期随访结果,疗效评价分为完全稳定缓解、药物缓解、好转、无变化、恶化(包括死亡).对性别、手术年龄、临床分型、术前病程、异位胸腺等因素进行统计分析.结果 (1)术后总随访人数53例,中位随访时间35个月,无恶化和死亡病例.总缓解率(完全缓解+药物缓解)69.8%,有效率(完全缓解+药物缓解+好转)90.6%.16例完全缓解患者无复发.眼肌型患者术后无1例转化为全身型,而16例全身型手术患者是由眼肌型转化而来.(2)单因素分析和多因素分析显示术前病程影响手术疗效(P<0.05).生存分析眼肌型和全身型患者术后2年、4年的总缓解率分别为56%、88%和42%、75%,Log-rank法比较两者之间总缓解率无差别.结论 严格挑选的重症肌无力儿童手术治疗安全、有效,特别是术前病程较短者.总缓解率随术后时间延长而升高.%Objective To evaluate the efficacy of thymectomy and relevant influencing factors in the treatment of children with myasthenia gravis through a long-term follow-up.Methods The clinical records of 59 patients undergoing expanded thymectomy for the treatment of myasthenia gravis ( MG ) between January 2003 and August 2009 were reviewed retrospectively.Their postoperative outcomes were categorized into complete stable remission (CSR),pharmacological remission (PR),improvement,no change and deterioration ( including mortality).Results During a median follow-up period of 35 months,none of them died or deteriorated clinically among 53 patients with a postoperative follow-up.The overall remission rate was 69.8% and the effective rate 90.6%.No symptomatic relapse occurred among 16 patients in CSR

  6. Animal models of antimuscle-specific kinase myasthenia.

    Science.gov (United States)

    Richman, David P; Nishi, Kayoko; Ferns, Michael J; Schnier, Joachim; Pytel, Peter; Maselli, Ricardo A; Agius, Mark A

    2012-12-01

    Antimuscle-specific kinase (anti-MuSK) myasthenia (AMM) differs from antiacetylcholine receptor myasthenia gravis in exhibiting more focal muscle involvement (neck, shoulder, facial, and bulbar muscles) with wasting of the involved, primarily axial, muscles. AMM is not associated with thymic hyperplasia and responds poorly to anticholinesterase treatment. Animal models of AMM have been induced in rabbits, mice, and rats by immunization with purified xenogeneic MuSK ectodomain, and by passive transfer of large quantities of purified serum IgG from AMM patients into mice. The models have confirmed the pathogenic role of the MuSK antibodies in AMM and have demonstrated the involvement of both the presynaptic and postsynaptic components of the neuromuscular junction. The observations in this human disease and its animal models demonstrate the role of MuSK not only in the formation of this synapse but also in its maintenance.

  7. Myasthenia Revealed Following Laparotomy - A Case Report

    Directory of Open Access Journals (Sweden)

    Abdelilah GHANNAM

    2015-06-01

    Full Text Available Myasthenia (muscle weakness is a rare neuromuscular disease of which respiratory failure is the main complication. The accidental discovery of such disease in the perioperative period is rare and potentially serious.We report a case of a woman who underwent emergency operation for appendiceal peritonitis, and failed repeatedly at weaning from postoperative mechanical ventilation. The usual etiologies such as postoperative respiratory complications, ventilator-associated pneumonia, acute respiratory distress syndrome complicating the septic shock or having no impact on it, and neuromyopathy’s resuscitation were considered, researched, examined or eliminated.Faced with the diagnostic impasse and the obvious weaning failure, another interview revealed signs of muscle fatigue which led to the diagnosis of myasthenia gravis decompensated perioperatively. Once the diagnosis was confirmed by means of a neostigmine test, the specific treatment began, particularly through plasma exchange sessions, and the process of weaning resumed. The result was complete weaning. A three-month follow-up showed a stable patient with no significant muscular disability.

  8. Dermatomyositis and myastenia gravis: An uncommon association with therapeutic implications.

    Science.gov (United States)

    Sangüesa Gómez, Clara; Flores Robles, Bryan Josué; Méndez Perles, Clara; Barbadillo, Carmen; Godoy, Hildegarda; Andréu, José Luis

    2015-01-01

    The association of dermatomyositis with myasthenia gravis (MG) is uncommon, having been reported so far in only 26 cases. We report the case of a 69 year-old man diagnosed with MG two years ago and currently treated with piridostigmyne. The patient developed acute proximal weakness, shoulder pain and elevated creatine-kinase (CK). He also developed generalized facial erythema and Gottron's papules. Laboratory tests showed positive antinuclear and anti-Mi2 antibodies. Further analysis confirmed CK levels above 1000 U/l. The clinical management of the patient and the therapeutic implications derived from the coexistence of both entities are discusssed.

  9. Meta-analysis reveals an association of PTPN22 C1858T with autoimmune diseases, which depends on the localization of the affected tissue.

    Science.gov (United States)

    Zheng, J; Ibrahim, S; Petersen, F; Yu, X

    2012-12-01

    Protein tyrosine phosphatase non-receptor type 22 (PTPN22) is a strong susceptibility gene shared by many autoimmune diseases. The aim of this study was to explore the mechanisms underlying this relationship. We performed a comprehensive analysis of the association between PTPN22 polymorphism C1858T and autoimmune diseases. The results showed a remarkable pattern; PTPN22 C1858T was strongly associated with type I diabetes, rheumatoid arthritis, immune thrombocytopenia, generalized vitiligo with concomitant autoimmune diseases, idiopathic inflammatory myopathies, Graves' disease, juvenile idiopathic arthritis, myasthenia gravis, systemic lupus erythematosus, anti-neutrophil cytoplasmic antibody-associated vasculitis and Addison's disease. By contrast, PTPN22 C1858T showed a negligible association with systemic sclerosis, celiac disease, multiple sclerosis, psoriasis, ankylosing spondylitis, pemphigus vulgaris, ulcerative colitis, primary sclerosing cholangitis, primary biliary cirrhosis, Crohn's disease and acute anterior uveitis. Further analysis revealed a clear distinction between the two groups of diseases with regard to their targeted tissues: most autoimmune diseases showing an insignificant association with PTPN22 C1858T manifest in skin, the gastrointestinal tract or in immune privileged sites. These results showed that the association of PTPN22 polymorphism with autoimmune diseases depends on the localization of the affected tissue, suggesting a role of targeted organ variation in the disease manifestations.

  10. 重症肌无力患者胸腺组织抑制性消减cDNA文库的构建及分析%Construction and analysis of suppression subtractive cDNA library of thymus from myasthenia gravis patients

    Institute of Scientific and Technical Information of China (English)

    杨俊红; 崔新征; 方华; 关兵峰; 赵国强; 张清勇; 高峰

    2012-01-01

    Aim:To build suppression subtractive cDNA library of thymus from myasthenia gravis (MG) patients.Methods:The total mRNA was extracted from thymus of normal people and MG patients and synthethized to cDNA by reverse transcription.The differentially expressed genes obtained by using suppression subtractive hybridization were proceeded by T-A cloning and sequence analysis.The expressions of GSTM3 and KPNA5 in thymus of 20 MG patients and 10 normal people were detected by real-time quantitative PCR.Results:A total of 125 positive clones were obtained;27 differentially expressed genes were obtained by Blast homology search.The expression levels of GSTM3, KPNA5 in thymus tissue of MG patients(0.671±0.097,0.712±0.080)were significantly higher than those of the control group (0.582±0.047,0.571±0.018)(tGSTM3=5.458,P<0.001;tKPNA5=2.755,P=0.010),consistent with the result of subtractive library.Conclusion:The establishment of suppression subtractive cDNA library of MG thymus is successful.%目的:构建重症肌无力(MG)患者胸腺组织抑制性消减cDNA文库,分析MG胸腺差异表达基因.方法:分别从6例正常和6例MG患者胸腺组织中分离出mRNA,逆转录合成cDNA,行抑制性消减杂交,将得到的差异表达基因进行T-A克隆,构建cDNA文库,并进行序列分析.应用实时荧光定量PCR检测20例MG患者、10例正常对照胸腺组织中GSTM3和KPNA5 mRNA的表达.结果:共获得125个阳性克隆;Blast同源性检索共得到27个差异表达基因;MG患者GSTM3、KPNA5基因表达量(0.671±0.097和0.712±0.080)高于正常对照胸腺(0.582±0.047和0.571±0.018),差异有统计学意义(tGSTM3=5.458,P<0.001;tKPNA5=2.755,P=0.010),与消减文库结果一致.结论:成功建立了MG胸腺组织抑制性消减cDNA文库.

  11. 分阶段心理护理对重症肌无力机械通气患者撤机时间的影响%The impact of programmatic psychological nursing on the time length of ventilator weaning in myasthenia gravis patients with mechanical ventilation

    Institute of Scientific and Technical Information of China (English)

    冯洁贞; 吴婉玲; 苏永静

    2010-01-01

    目的 探讨分阶段心理护理对重症肌无力机械通气期患者撤机时间的影响.方法 选择重症肌无力机械通气期的ICU患者作为实验组(n=44),根据其生理基础、血气分析结果等确定其撤机时间,根据不同的过渡阶段应用护理程序对其实施有计划性、有针对性、个体化的心理护理,对照组(n=42)仅采用从CMV+→SIMV+→SPONT→间歇脱机→完全撤机的方法.记录并比较两组患者撤机的时间及住院天数.结果 对照组平均撤机时间为18 d,实验组为14 d,实验组的撤机时间低于对照组,但差异无统计学意义(P=0.752);对照组患者的住院天数比实验组长,差异有统计学意义(P=0.039).结论 在重症肌无力患者机械通气的不同阶段,及时有效地实施个体化的分阶段心理护理,可以缩短患者的撤机时间及住院天数,从而提高治疗护理效果,减轻了患者的痛苦及经济负担.%Objective To investigate the impact of psychological nursing on time length of ventilator weaning in patients with myasthenia gravis (MG). Methods Forty-four MG patients with mechanical ventilation were enrolled to the testing group. In this group, the time of ventilator weaning was determined according to the results of basic status and blood gas analysis. Furthermore, the designed, targeted and individuated psychological care was conducted in this group at different phases. In the control group, only the programmatic method of ventilator weaning was used with the steps of CMV, SIMV, SPONT, intermittence and completely weaning. The time length of ventilator weaning and the length of stay were compared between two groups.Results The time length of weaning in testing group was 14 days comparing with 18 days in control group.Although there was no statistical significance ( P = 0.752), the time length of ventilator weaning in testing group was shorter than that in control group. The length of stay was longer in control group than that in

  12. Selenomethionine Se 75 thymus scans in myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Toole, J.F.; Cowan, R.; Maynard, D.; Witcofski, R.L.; Janeway, R.

    1975-01-01

    In 1966, Toole and Witcofski introduced selenomethionine Se 75 mediastinal scanning as a diagnostic test for thymomas. Since then we have performed such scans on patients with myasthenic syndrome. Because the technique is atraumatic, safe, and accurate, it can be performed on critically ill myasthenics. Two hundred and fifty microcuries of selenomethionine Se 75 is injected intravenously. Within 1 hour the mediastinum from the suprasternal notch to the ziphoid process is scanned, using a 2 x 3 inch scanner. Delayed scans have been made in a few instances but they have not increased the number of positive cases. Selenomethionine Se 75 is incorporated into tissues undergoing rapid protein synthesis such as thyroid, pancreas, liver, and lymphomas. Of the 34 mediastinal scans performed on myasthenics between 1966 and December 31, 1974, 4 were positive for thymoma. In addition, there was a positive scan with uptake in an area of atelectasis of the lung adjacent to the mediastinum. Of interest is the fact that 1 patient with carcinoma of the lung had a positive scan over the lesion. In 13 patients with chronic lymphatic leukemia the mediastinal scans were negative. In another patient with a mediastinal mass noted on chest x-ray, a variety of differential diagnostic possibilities were considered, such as pericardial cyst, dermoid, and aneurysm. A selenomethionine scan was strongly positive, suggesting a thymoma which subsequent surgery confirmed.

  13. Absence of autoantibodies connected to autoimmune polyendocrine syndrome type I and II and Addison's disease in girls and women with Turner syndrome

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    Kämpe Olle

    2007-12-01

    Full Text Available Abstract Background A disturbance in the immune system has been described in Turner syndrome (45,X, with an association to low levels of IgG and IgM and decreased levels of T- and B-lymphocytes. Also different autoimmune diseases have been connected to Turner syndrome (45,X, thyroiditis being the most common. Other autoimmune diseases seen are inflammatory bowel disease, insulin dependent diabetes mellitus, Addison's disease, rheumatoid arthritis, myasthenia gravis, vitiligo, alopecia, pernicious anaemia and hypoparathyroidism, but the association to Turner syndrome is not definite. Besides the typical features of Turner syndrome (short stature, failure to enter puberty spontaneously and infertility due to ovarian insufficiency ear problems are common. Otitis media and a progressive sensorineural hearing disorder are commonly seen. In the normal population there are known inner ear disorders related to autoimmune diseases. The aim of this study was to investigate patients with Turner syndrome regarding autoantibodies connected to the autoimmune disorders; autoimmune polyendocrine syndrome type I and II and Addison's disease, to screen for overlapping profile of autoantibodies. Blood samples from 110 Turner patients (7–65 years were investigated using in vitro transcription, translation and immunoprecipitation techniques regarding autoantibodies connected to autoimmune polyendocrine syndrome type I and II and Addison's disease (21-hydroxylase, 17α-hydroxylase, side-chain cleavage enzyme, aromatic L-amino acid decarboxylase, tyrosine hydroxylase and tryptophan hydroxylase. Results The autoantibodies investigated were not overrepresented among the Turner patients. Conclusion The autoimmune disorders associated with Turner syndrome do not seem to be of the same origin as Addison's disease, the type I or II autoimmune polyendocrine syndrome.

  14. Outcome of Extended Thymectomy in Myasthenia Crisis Patient.

    Science.gov (United States)

    Aftabuddin, M; Bhandari, S

    2016-07-01

    Myasthenic crisis is a life-threatening condition. We studied the demographic, frequency, causes and clinical presentation of isolated Myasthenic crisis, steps of treatment and to review our experience of extended thymectomy on patients with at least one episode myasthenic crisis. A prospective and retrospective study was conducted on patients with at least one episode of myasthenic crisis, from March 2010 to September 2014, at the Department of Cardiac Surgery, BSMMU, Dhaka, Bangladesh who were referred for thymectomy. Eighteen patients (13.6% of the total 132 patients with myasthenia gravis were admitted with single to multiple episodes of myasthenic crisis, median crisis was 2.5 episodes. Mean age of the patient was 35.5 (18-72) years with male predominance. All eighteen patients had undergone extended thymectomy after completion of 5 cycle plasmapheresis, of which 2 had experienced postoperative respiratory crisis, required invasive ventilator support for median 14 days. One patient required invasive ventilator support after third post operative day. Six patients had thymoma and 12 had thymic hyperplasia. Three patients needed Intravenous immunoglobin. Nine patients needed post operative anti acetylcholinesterase inhibitor after median 2.5 post days. Post thymectomy remission and decreases the frequency of myasthenic crisis was seen in follow up and post operative medication requirement reduced significantly as compared to the preoperative requirement. This report highlights that the patients who had extended thymectomy after episodes of myasthenia crisis are benefitted even in the histhopathology report does not confirmed thymoma. After thymectomy, there was remission of myasthenic crisis. Patients with myasthenic crisis should have judicious drug adjustments under supervision and should be treated aggressively during impending myasthenic crisis. With modern management of myasthenia gravis, early surgery with myasthenic crisis is safe with good long

  15. Sjögren syndrome and neuromyelitis optica spectrum disorder co-exist in a common autoimmune milieu

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    Diogo C. Carvalho

    2014-06-01

    Full Text Available The relationship between Sjögren’s syndrome (SS and neuromyelitis optica spectrum disorder (NMOSD is not completely understood. We report two patients with both conditions and review 47 other previously reported cases meeting currently accepted diagnostic criteria, from 17 articles extracted from PubMed. Out of 44 patients whose gender was informed, 42 were females. Mean age at onset of neurological manifestation was 36.2 years (10-74. Serum anti-AQP4-IgG was positive in 32 patients, borderline in 1, and negative in 4. Our Case 1 was seronegative for AQP4-IgG and had no non-organ-specific autoantibodies other than anti-SSB antibodies. Our Case 2 had serum anti-AQP4, anti-SSA/SSB, anti-thyreoglobulin and anti-acethylcholine-receptor antibodies, as well as clinical hypothyreoidism, but no evidence of myasthenia gravis. Our Cases and others, as previously reported in literature, with similar heterogeneous autoimmune response to aquaporin-4, suggest that SS and NMO co-exist in a common autoimmune milieu which is not dependent on aquaporin-4 autoimmunity.

  16. Different familial association patterns of autoimmune diseases between juvenile-onset systemic lupus erythematosus and juvenile rheumatoid arthritis.

    Science.gov (United States)

    Huang, Chun-Mei; Yang, Yao-Hsu; Chiang, Bor-Luen

    2004-04-01

    The aim of this study was to determine if the prevalence of autoimmune disorders in the relatives of patients with systemic lupus erythematosus (SLE) is greater than that of relatives of patients with juvenile rheumatoid arthritis (JRA). Interviews were used to obtain histories of the following autoimmune disorders among living or deceased first-, second-, and third-degree relatives of 91 SLE and 110 JRA families: ankylosing spondylitis, SLE, rheumatoid arthritis (RA), JRA, multiple sclerosis, juvenile dermatomyositis, Sjögren's syndrome, myasthenia gravis, psoriasis, and thyroid diseases. There were statistically significant differences between the SLE and JRA probands in mean age and gender ratio (19.1 +/- 4.8 vs 14.0 +/- 5.5 years; M (male)/F (female): 17/74 vs 62/48, pJRA families (11.8%), but not statistically significantly so. The mean age (18.0 +/- 5.3 vs 14.0 +/- 4.3 years), mean age at diagnosis (13.4 +/- 4.3 vs 7.9 +/- 3.9 years) and gender ratio (F/M, 16/3 vs 5/8) of the patients with affected relatives between these 2 groups all had statistically significant differences. A higher prevalence of SLE in relatives was found in SLE families than in JRA cases. Furthermore, this study revealed a higher incidence of autoimmune disorders among second- and third-degree relatives of SLE or JRA probands versus first-degree ones, especially sisters (including 1 pair of twins) and the maternal aunt in SLE families. These data demonstrate that the prevalence of autoimmune disorders in the relatives of patients with SLE is greater than those of relatives of patients with JRA. This suggests that clinically different autoimmune phenotypes may share common susceptibility genes, which may act as risk factors for autoimmunity.

  17. Sequence and timing of immunosuppressants-thymectomy combination therapy in patients with myasthenia gravis%免疫抑制-胸腺切除联合治疗顺序和手术时机对重症肌无力结局的影响

    Institute of Scientific and Technical Information of China (English)

    刘欣欣; 李然; 于磊; 景筠

    2015-01-01

    Objective Following the prognosis of patients with myasthenia gravis(MG) who received both immunosuppressants and thymectomy, to determine whether the sequence and timing of treatment have influences on outcomes of MG and also to select significant predictors of prognosis.Methods It was a retrospective study which reviewed 105 patients with MG who underwent thymectomy and immunosuppressants between Januay, 2000 and December, 2013.All patients were divided into two subgroups based on their order of treatment.Thirty two patients were called as immunosuppressants-first group, and 73 patients were called as thymectomy-first group.The ratios of ideal status of two groups were compared with use of the ChiSquare test.Predictors of ideal status were analyzed by logistic regression.Results (1) After 1 year, 2 years and 5 years of intervention, the proportion of ideal status was significantly higher in the thymectomy-first group than that in the immunosuppressants-first group[(33%-50%) vs.(10%-20%), P <0.05].For oMG patients, after 1 year, 2 years and 5 years of treatment, thymectomy-first group had a higher ideal status rates than immunosuppressants-first group[(25%-50%) vs.(11%-18%)].But no significant difference was noted in the rates of ideal status at each follow-up time point.(2)Multivariate logistic regression analysis showed that sequence of treatment (OR =0.154, 95 % CI: 0.046-0.518, P =0.002) and symptom-thymectomy interval(OR =0.903,95 % CI: 0.835-0.976, P =0.010) were the significant predictors of ideal status.Conclusion Thymectomy prior to immunosuppressants is associated with relatively good prognosis.It is suggested that patients with gMG, or adult patients with oMG who are resistant to drug, will be better to undergo thymecotomy as early as possible.%目的 研究免疫抑制-胸腺切除联合治疗策略对重症肌无力(MG)结局的影响.方法 回顾性研究2000年1月至2013年12月免疫抑制-胸腺切除联合治疗105例,根

  18. The Relationship between AChR-Ab and the Post-thymectomy Clinical Outcome of Generalized Myasthenia Gravis%乙酰胆碱受体抗体与全身型重症肌无力手术预后的关系

    Institute of Scientific and Technical Information of China (English)

    刘天芳; 任明山; 吴元波; 杨毅

    2015-01-01

    Aim To compare the clinical outcome of the post-thymectomy between generalized myasthenia gravis (gMG) with acetylcholine receptor antibody (AChR-Ab) positive and AChR-Ab negative. Methods124 cases of gMG who were retrospectively reviewed had received video-assisted thoracoscopic thymectomy in the thoracic surgery. According to the type of the serum antibodies, the patients were divided in to AChR-Ab-positive group and AChR-Ab-negative group. The primary endpoint was to assess differences in the rate of complete stable remission (CSR) in patients in the two groups.ResultsThere were 81 patients in the AChR-Ab positive group, and 43 patients in AChR-Ab-negative group, there was no signiifcant difference among the three groups regarding sex, age at onset and disease duration, and thymus pathology. One case in AChR-Ab-positive group was lost, two cases in AChR-Ab-negative group were lost. Until the deadline of follow-up time of Sep. 2014, a total of 121 patients were followed, including 80 cases in AChR-Ab-positive group, 41 cases in AChR-Ab-negative group. Follow-up time was 15 to 84 months, mean (44.9±19.9) months in AchR-Ab positive group, mean (48.2±20.3) months in AChR-Ab-negative group. The complete stable remission rate (CSR) in AChR-Ab-positive group was 48.8%, in the AChR-Ab-negative group theP vaule was 26.8%. The CSR in the AChR-Ab-positive group was higer than that in the AChR-Ab-negative group [(χ2=5.372,P=0.020)]. The overall remission rate in the AChR-Ab-positive group was 85.1%, and 73.2% in the AChR-Ab-negative. There was no signiifcant difference in the overall remission rate between the two group. By Kaplan–Meier analysis, there was signiifcant difference in CSR rates between AChR-Ab-positive group and AChR-Ab-negative group (P=0.03).Conclusion Long-term post-thymectomy clinical outcome was better in AChR-Ab-positive group than in AChR-Ab-negative group, but our results showed no differences in the overall remission rate.%目的:比较乙酰

  19. Difference in time-course of relaxant effect of rocuronium between patients with ocular and generalized myasthenia gravis%眼肌型和全身型重症肌无力患者罗库溴铵肌松时效的比较

    Institute of Scientific and Technical Information of China (English)

    曹迎亚; 鲁卫华; 姜小敢; 金孝岠; 鲁美静

    2013-01-01

    Objective To compare the time-course of relaxant effect of rocuronium between patients with ocular and generalized myasthenia gravis (MG).Methods Twenty-seven ASA physical status I or Ⅱ patients with MG of both sexes,aged 12-64 yr,with body mass index of 17-26 kg/m2,scheduled for elective extended thymectomy,were divided into 2 groups according to Osserman stage:ocular group (group O,n =10) and generalized group (group G,n =17).Anesthesia was induced with iv injection of fentanyl 2 μg/kg,midazolam 0.05 mg/kg,and propofol 1.5 mg/kg.All patients were tracheal intubated and mechanically ventilated.Anesthesia was maintained with iv infusion of propofol 4-8 mg· kg-1 · h-1 and remifentanil 0.2 μg· kg-1 · min-1.Twitch tension was monitored in the adductor pollicis muscle by train-of-four stimulation of the ulnar nerve (intensity 60 mA,interval 12 s,frequency 2 Hz,wave length 0.2 ms).Rocuronium 0.6 mg/kg was injected intravenously after calibration.Mean arterial pressure (MAP),HR,heart rate variability (HRV) and low frequency and high frequency (LF/HF) ratio was recorded.The onset time of muscle relaxation,time for T1 to recover to 25%,time for T1 to recover to 50% and recovery index were recorded.Results Compared with the baseline value,there were no significant changes in MAP,HR,HRV,LF/HF ratio at all time points in the two groups (P > 0.05).Compared with group O,there was no significant change in the onset time of muscle relaxation,and the time for T1 to recover to 25%,time for T1 to recover to 50% and recovery index were significantly prolonged in group G (P < 0.05).Conclusion The duration of rocuronium-induced neuromuscular block is significantly longer in patients with generalized MG than those with ocular MG,while the onset time is comparable between the two groups.%目的 比较眼肌型和全身型重症肌无力患者罗库溴铵的肌松时效.方法 择期行胸骨正中切口胸腺切除术的27例患者,性别不限,年龄12 ~64

  20. Surgery Effective Against Immune Disorder That Weakens Muscles

    Science.gov (United States)

    ... html Surgery Effective Against Immune Disorder That Weakens Muscles Myasthenia gravis affects 60,000 Americans, but removal ... gravis, an autoimmune disorder that causes life-threatening muscle weakness, researchers report. Since the 1940s, doctors have ...

  1. 9 CFR 319.313 - Beef with gravy and gravy with beef.

    Science.gov (United States)

    2010-01-01

    ... 9 Animals and Animal Products 2 2010-01-01 2010-01-01 false Beef with gravy and gravy with beef... Dehydrated Meat Food Products § 319.313 Beef with gravy and gravy with beef. “Beef with Gravy” and “Gravy with Beef” shall not be made with beef which, in the aggregate for each lot contains more than...

  2. CD8+ T-Cell Deficiency, Epstein-Barr Virus Infection, Vitamin D Deficiency, and Steps to Autoimmunity: A Unifying Hypothesis

    Directory of Open Access Journals (Sweden)

    Michael P. Pender

    2012-01-01

    Full Text Available CD8+ T-cell deficiency is a feature of many chronic autoimmune diseases, including multiple sclerosis, rheumatoid arthritis, systemic lupus erythematosus, Sjögren's syndrome, systemic sclerosis, dermatomyositis, primary biliary cirrhosis, primary sclerosing cholangitis, ulcerative colitis, Crohn's disease, psoriasis, vitiligo, bullous pemphigoid, alopecia areata, idiopathic dilated cardiomyopathy, type 1 diabetes mellitus, Graves' disease, Hashimoto's thyroiditis, myasthenia gravis, IgA nephropathy, membranous nephropathy, and pernicious anaemia. It also occurs in healthy blood relatives of patients with autoimmune diseases, suggesting it is genetically determined. Here it is proposed that this CD8+ T-cell deficiency underlies the development of chronic autoimmune diseases by impairing CD8+ T-cell control of Epstein-Barr virus (EBV infection, with the result that EBV-infected autoreactive B cells accumulate in the target organ where they produce pathogenic autoantibodies and provide costimulatory survival signals to autoreactive T cells which would otherwise die in the target organ by activation-induced apoptosis. Autoimmunity is postulated to evolve in the following steps: (1 CD8+ T-cell deficiency, (2 primary EBV infection, (3 decreased CD8+ T-cell control of EBV, (4 increased EBV load and increased anti-EBV antibodies, (5 EBV infection in the target organ, (6 clonal expansion of EBV-infected autoreactive B cells in the target organ, (7 infiltration of autoreactive T cells into the target organ, and (8 development of ectopic lymphoid follicles in the target organ. It is also proposed that deprivation of sunlight and vitamin D at higher latitudes facilitates the development of autoimmune diseases by aggravating the CD8+ T-cell deficiency and thereby further impairing control of EBV. The hypothesis makes predictions which can be tested, including the prevention and successful treatment of chronic autoimmune diseases by controlling EBV infection.

  3. Same-sex marriage, autoimmune thyroid gland dysfunction and other autoimmune diseases in Denmark 1989-2008.

    Science.gov (United States)

    Frisch, Morten; Nielsen, Nete Munk; Pedersen, Bo Vestergaard

    2014-01-01

    Autoimmune diseases have been little studied in gay men and lesbians. We followed 4.4 million Danes, including 9,615 same-sex married (SSM) persons, for 47 autoimmune diseases in the National Patient Registry between 1989 and 2008. Poisson regression analyses provided first hospitalization rate ratios (RRs) comparing rates between SSM individuals and persons in other marital status categories. SSM individuals experienced no unusual overall risk of autoimmune diseases. However, the risk of autoimmune thyroid dysfunction was increased, notably Hashimoto's thyroiditis (women(SSM), RR = 2.92; 95% confidence interval (CI) 1.74-4.55) and Graves' disease (men(SSM), RR = 1.88; 95% CI 1.08-3.01). There was also an excess of primary biliary cirrhosis (women(SSM), RR = 4.09; 95% CI 1.01-10.7), and of psoriasis (men(SSM), RR = 2.48; 95% CI 1.77-3.36), rheumatic fever (men(SSM), RR = 7.55; 95% CI 1.87-19.8), myasthenia gravis (men(SSM), RR = 5.51; 95% CI 1.36-14.4), localized scleroderma (men(SSM), RR = 7.16; 95% CI 1.18-22.6) and pemphigoid (men(SSM), RR = 6.56; 95% CI 1.08-20.6), while Dupuytren's contracture was reduced (men(SSM), RR = 0.64; 95% CI 0.39-0.99). The excess of psoriasis was restricted to same-sex married men with HIV/AIDS (men(SSM), RR = 10.5; 95% CI 6.44-15.9), whereas Graves' disease occurred in excess only among same-sex married men without HIV/AIDS (men(SSM), RR = 1.99; 95% CI 1.12-3.22). Lesbians and immunologically competent gay men in same-sex marriage face no unusual overall risk of autoimmune diseases. However, the observed increased risk of thyroid dysfunction in these lesbians and gay men deserves further study.

  4. An Adult Patient with Ocular Myasthenia and Unusually Long Spontaneous Remission

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    Jasem Al-Hashel

    2014-01-01

    Full Text Available A male patient developed ocular myasthenia gravis (MG at the age of 33. He was anti-acetylcholine receptor antibody (anti-AChR Ab negative. He received cholinesterase blocker for 5 months and went into a complete clinical remission that lasted untreated for 17 years. He relapsed recently with ocular symptoms only. He is now anti-AChR Ab positive and SFEMG is abnormal in a facial muscle. The patient is controlled with steroids. He had one of the longest spontaneous remissions reported in the natural history of MG, particularly unusual for an adult with the disease.

  5. Autoimmune thyroid disease and other non-endocrine autoimmune diseases

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    Todorović-Đilas Ljiljana

    2011-01-01

    Full Text Available Introduction, Autoimmune diseases are chronic conditions initiated by the loss of immunological tolerance to self-antigens. They constitute heterogeneous group of disorders, in which multiple alterations in the immune system result in a spectrum of syndromes that either target specific organs or affect the body systematically. Recent epidemiological studies have shown a possible shift of one autoimmune disease to another or the fact that more than one autoimmune disease may coexist in a single patient or in the same family. Numerous autoimmune diseases have been shown to coexist frequently with thyroid autoimmune diseases. Autoimmune thyroid disease and other organ specific non-endocrine autoimmune diseases. This part of the study reviews the prevalence of autoimmune thyroid disease coexisting with: pernicious anaemia, vitiligo, celiac disease, autoimmune liver disease, miastenia gravis, alopecia areata and sclerosis multiplex, and several recommendations for screening have been given. Autoimmune thyroid disease and other organ non-specific non-endocrine autoimmune diseases. Special attention is given to the correlation between autoimmune thyroid disease and rheumatoid arthritis, systemic lupus erythematosus, syndrome Sjögren, systemic sclerosis and mixed connective tissue disease. Conclusions. Screening for autoimmune thyroid diseases should be recommended in everyday clinical practice, in patients with primary organ-specific or organ non-specific autoimmune disease. Other­wise, in patients with primary thyroid autoimmune disease, there is no good reason of seeking for all other autoimmune diseases, although these patients have a greater risk of developing other autoimmune disease. Economic aspects of medicine require further analyzing of these data, from cost/benefit point of view to justified either mandatory screening or medical practitioner judgment.

  6. Intravenous immunoglobulin in the treatment of autoimmune neuromuscular diseases: present status and practical therapeutic guidelines.

    Science.gov (United States)

    Dalakas, M C

    1999-11-01

    This review summarizes the current status of intravenous immunoglobulin (IVIg) in the treatment of autoimmune neuromuscular disorders and the possible mechanisms of action of the drug based on work in vivo, in vitro, and in animal models. Supply of idiotypic antibodies, suppression of antibody production, or acceleration of catabolism of immunoglobulin G (IgG) are relevant in explaining the efficacy of IVIg in myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS), and antibody-mediated neuropathies. Suppression of pathogenic cytokines has putative relevance in inflammatory myopathies and demyelinating neuropathies. Inhibition of complement binding and prevention of membranolytic attack complex (MAC) formation are relevant in dermatomyositis (DM), Guillain-Barré syndrome (GBS), and MG. Modulation of Fc receptors or T-cell function is relevant in chronic inflammatory demyelinating polyneuropathy (CIDP), GBS, and inflammatory myopathies. The clinical efficacy of IVIg, based on controlled clinical trials conducted in patients with GBS, CIDP, multifocal motor neuropathy (MMN), DM, MG, LEMS, paraproteinemic IgM anti-myelin-associated glycoprotein (anti-MAG) demyelinating polyneuropathies, and inclusion body myositis is summarized and practical issues related to each disorder are addressed. The present role of IVIg therapy in other disorders based on small controlled or uncontrolled trials is also summarized. Finally, safety issues, risk factors, adverse reactions, spurious results or serological tests, and practical guidelines associated with the administration of IVIg in the treatment of neuromuscular disorders are presented.

  7. INFLUENCE OF ALLOFERIN ON NEUROMUSCULAR FUNCTION IN MYASTHENIA PATIENTS UNDERGOING THYMECTOMY

    Institute of Scientific and Technical Information of China (English)

    罗霞; 叶铁虎; 罗爱伦; 任洪智; 金永芳

    1994-01-01

    Myasthenia gravis patients are hypersensitive to nondepolarizing relaxants,such as alcuronium,an intermedi-ate-long nondepolarizing agent. This study observed the effects of alcuronium treatment in myasthenia gravis pa-tients as compared with non-MG patients during operation.Ten MG patients (Ossermann class Ⅰ-Ⅳ,scheduled for thymectomy)and 10non-MG patients(ASA class Ⅰ-Ⅱ,scheduled for operation)were selected.An induc-tion dose of alcuronium 0.2 mg/kg and thiopental 4-6 mg/kg was given,followed by intubation and ventilation with 50% nitrous oxide in oxygen and 0.5-1.5% ethrane.Neuromuscular transmission was monitored using an accelerogragh and degrees of neuromuscular function at different depths were recorded.There were statistically significant differences between the tow groups.The effect of alloferin in the MG group was quicker and deeper.This study also found a relation between MG class and the recovery of respiration: Respiratory recovery was quicker in classes Ⅰ-Ⅱ than in classes Ⅲ-Ⅳ.

  8. Millora en el tractament contra la miastènia gravis

    OpenAIRE

    2006-01-01

    La miastènia gravis és una malaltia autoimmune i crónica, caracteritzada per una gran debilitat muscular. Un assaig clínic realitzat per la Unitat de Miastènia de l'Hospital General Universitari Vall d'Hebron durant cinc anys acaba de demostrar els beneficis d'incloure un fàrmac, el tacrolimus, per guarir aquesta malaltia.

  9. Use of azathioprine for non-thymoma myasthenia and risk of cancer

    DEFF Research Database (Denmark)

    Pedersen, E G; Pottegård, Anton; Hallas, J

    2013-01-01

    BACKGROUND AND PURPOSE: To evaluate the association between the use of azathioprine and risk of cancer in patients with non-thymoma myasthenia gravis (MG) in a nationwide setting. METHODS: Case-control study based on population-based registries. Cases were patients with MG with a first time...... diagnosis of cancer (except non-melanoma skin cancer) registered during 2000-2009, and controls were patients with MG with no history of cancer. Prior use of azathioprine in cases and controls was assessed through prescription records (1995-2009). We used unconditional logistic regression to calculate odds...... ratios (ORs) with 95% confidence intervals (CIs) for cancer associated with a high cumulative dose [≥ 1000 defined daily doses (DDD)] or long-term use (≥ 5 years) of azathioprine, compared with never use of the drug and adjusted for potential confounders. RESULTS: We identified 89 cases and 873 controls...

  10. Computed tomography of the anterior mediastinum in myasthemia gravis: a radiologic-pathologic correlative study

    Energy Technology Data Exchange (ETDEWEB)

    Fon, G.T.; Bein, M.E.; Mancuso, A.A.; Keesey, J.C.; Lupetin, A.R.; Wong, W.S.

    1982-01-01

    Chest radiographs and computed tomographic (CT) scans of the mediastinum were correlated with pathologic findings of the thymus following thymectomy in 57 patients with myasthenia gravis. Based on the patient's age and the overall morphology of the anterior mediastinum, CT scans were assigned one of four grades in an attempt to predict thymus pathologic findings. Using this grading, 14 of 16 cases of thymoma were suspected or definitely diagnosed. One of the two cases not diagnosed on CT was a microscopic tumor. There were no false-positive diagnoses in 11 cases graded as definitely thymoma. We conclude that thymoma can be sensitively diagnosed in patients older than 40 years of age. However, thymoma cannot be predicted with a high level of confidence in patients younger than 40 because of the difficulty in differentiating normal thymus or hyperplasia from thymoma. Recommendations for the use of CT in the preoperative evaluation of myasthenic patients are presented.

  11. The Protective Role of HLA-DRB113 in Autoimmune Diseases

    Directory of Open Access Journals (Sweden)

    Andreia Bettencourt

    2015-01-01

    Full Text Available Autoimmune diseases (AIDs are characterized by a multifactorial aetiology and a complex genetic background, with the MHC region playing a major role. We genotyped for HLA-DRB1 locus 1228 patients with AIDs-213 with Systemic Lupus Erythematosus (SLE, 166 with Psoriasis or Psoriatic Arthritis (Ps + PsA, 153 with Rheumatoid Arthritis (RA, 67 with Systemic Sclerosis (SSc, 536 with Multiple Sclerosis (MS, and 93 with Myasthenia Gravis (MG and 282 unrelated controls. We confirmed previously established associations of HLA-DRB115 (OR = 2.17 and HLA-DRB103 (OR = 1.81 alleles with MS, HLA-DRB103 with SLE (OR = 2.49, HLA-DRB101 (OR = 1.79 and HLA-DRB104 (OR = 2.81 with RA, HLA-DRB107 with Ps + PsA (OR = 1.79, HLA-DRB101 (OR = 2.28 and HLA-DRB108 (OR = 3.01 with SSc, and HLA-DRB103 with MG (OR = 2.98. We further observed a consistent negative association of HLA-DRB113 allele with SLE, Ps + PsA, RA, and SSc (18.3%, 19.3%, 16.3%, and 11.9%, resp., versus 29.8% in controls. HLA-DRB113 frequency in the AIDs group was 20.0% (OR = 0.58. Although different alleles were associated with particular AIDs, the same allele, HLA-DRB113, was underrepresented in all of the six diseases analysed. This observation suggests that this allele may confer protection for AIDs, particularly for systemic and rheumatic disease. The protective effect of HLA-DRB113 could be explained by a more proficient antigen presentation by these molecules, favouring efficient clonal deletion during thymic selection.

  12. Addison disease

    Science.gov (United States)

    ... amounts of some or all of its hormones ( hypopituitarism ) Autoimmune disorder that affects the nerves and the ... disease) Dermatitis herpetiformis Diabetes Graves disease Hyperthyroidism Hypoparathyroidism Hypopituitarism Immune response Myasthenia gravis Ovarian hypofunction Pernicious anemia ...

  13. A case of chronic inflammatory demyelinating polyneuropathy presented with unilateral ptosis.

    Science.gov (United States)

    Izadi, Sadegh; Karamimagham, Sina; Poursadeghfard, Maryam

    2014-01-01

    Chronic Inflammatory Demyelinating Polyneuropathy is an autoimmune disease with progressive and relapsing courses. The main clinical presentations are diffuse deep tendon hyporeflexia or areflexia and symmetric proximal-distal muscles weakness. Myasthenia gravis is also an immune mediated disease with fluctuating ocular and bulbar symptoms and sometimes weakness. Although both myasthenia gravis and chronic inflammatory demyelinating polyneuropathy are immune mediated disorders, clinical presentations are obviously different in the two diseases. Herein, we will report a case of chronic inflammatory demyelinating polyneuropathy who presented with isolated unilateral ptosis. Initially, the patient was managed as ocular type of myasthenia gravis, but after progression to general limb weakness and areflexia, the diagnosis of chronic inflammatory demyelinating polyneuropathy was made. Although unilateral ptosis is a typical feature of myasthenia gravis, it may be seen as the first presentation of chronic inflammatory demyelinating polyneuropathy as well which mimics myasthenia gravis disease.

  14. Higgs Decays and Brane Gravi-vectors

    OpenAIRE

    Clark, T. E.; Liu, B. Y.; Love, S. T.; Xiong, C.; ter Veldhuis, T.

    2008-01-01

    Higgs boson decays in flexible brane world models with stable, massive gravi-vectors are considered. Such vectors couple bilinearly to the Standard Model fields through either the Standard Model energy-momentum tensor, the weak hypercharge field strength or the Higgs scalar. The role of the coupling involving the extrinsic curvature is highlighted. It is found that within the presently allowed parameter space, the decay rate of the Higgs into two gravi-vectors (which would appear as an invisi...

  15. Cognitive impairment as a central cholinergic deficit in patients with Myasthenia Gravis

    Directory of Open Access Journals (Sweden)

    Antonia Kaltsatou

    2015-06-01

    Conclusions: VCmax and ACmax are governed mainly by the action of the Parasympathetic Nervous System, through acetylcholine. The results of this study demonstrate that the CNS may be affected in MG and support the hypothesis that MG has central cholinergic effects manifested by cognitive dysfunction.

  16. Effect of ultra-low dose whole-body-irradiation on patients with severe myasthenia gravis

    Energy Technology Data Exchange (ETDEWEB)

    Arimori, Shigeru; Koriyama, Kenji (Tokai Univ., Isehara, Kanagawa (Japan). School of Medicine)

    1982-12-01

    An ultra-low dose whole body irradiation therapy was given to 5 patients with intractable bulbar syndrome, in a dose of 10 rad/fraction, 2 times a week for 5 weeks, with a total of 100 rad; and effects of this therapy on their clinical symptoms and immunological ability were discussed. In 3 of them, bulbar syndrome was improved, and the other one, the first irradiation was effective. The peripheral leukocyte count and lymphocyte count became lowest immediately after completion of the irradiation, and returned to the normal level within 1 to 2 months. The function of T-cells, especially suppressive T-cells, was recovered; and decrease in B-cells, resulted in a decrease in the AChR antibody titer.

  17. Muscle strength and fatigue in newly diagnosed patients with myasthenia gravis

    DEFF Research Database (Denmark)

    Vinge, Lotte; Andersen, Henning

    2016-01-01

    significantly with manual muscle testing (MG Composite). Dynamometry revealed improved muscle strength of up to 50% (median 17%, range -1.8 - 49.8) despite no change in the MG Composite score. DISCUSSION: Dynamometry appears to be a more sensitive method of identifying changes in limb strength than MG...

  18. [Validation of the ice pack test in ophthalmoparesis due to myasthenia gravis].

    Science.gov (United States)

    Ramirez-Antunez, Ángel G; García-Ramos, Guillermo; Estañol-Vidal, Bruno; Juárez-Flores, Alejandra

    2013-11-01

    Introduccion. La miastenia grave es una enfermedad autoinmune de la union neuromuscular que se presenta clinicamente como debilidad fluctuante de los musculos estriados, como los de la region ocular (miastenia ocular). Objetivo. Demostrar que la sensibilidad y la especificidad de la prueba de hielo son altas en el diagnostico diferencial de la oftalmoparesia y ptosis palpebral por miastenia grave y miastenia ocular. Sujetos y metodos. Estudio observacional, analitico, no aleatorizado, de una muestra de 43 sujetos, 21 con miastenia grave y 22 controles. A todos los pacientes se les aplico un guante con hielo sobre sus parpados superiores afectados durante dos minutos, despues de los cuales se evaluo el grado de mejoria de la ptosis palpebral y la oftalmoparesia. Todos tenian estudio de estimulacion nerviosa repetitiva. Resultados. Se analizaron 36 pacientes, 18 con miastenia grave u ocular y 18 controles. Todos presentaron ptosis palpebral y solo 20 de ellos oftalmoparesia. La prueba de hielo para la oftalmoparesia mostro una sensibilidad del 83%, especificidad del 100%, valor predictivo positivo (VPP) del 100% y valor predictivo negativo (VPN) del 80% en el diagnostico de la miastenia grave. Para la ptosis palpebral, se determino una sensibilidad del 89%, especificidad del 100%, VPP del 100% y VPN del 90%. Para la estimulacion nerviosa repetitiva se calculo una sensibilidad del 61%, especificidad del 83%, VPP del 79% y VPN del 68%. Conclusion. La prueba de hielo es sencilla, segura, economica, rapida y fiable para utilizarse de rutina en pacientes con sospecha de ptosis u oftalmoparesia por miastenia grave, ya que tiene una alta validez, seguridad y reproducibilidad como prueba diagnostica.

  19. Castleman′s disease with paraneoplastic pemphigus

    Directory of Open Access Journals (Sweden)

    Dinesha

    2014-01-01

    Full Text Available Castleman′s disease is a rare autoimmune disorder with varied clinical presentations. Castleman′s commonly involves mediastinum and hence it is thoracic in most of the reported cases. Paraneoplastic pemphigus (PNP and myasthenia gravis can be associated with multicentric Castleman′s disease. Its association with HIV, Kaposi sarcoma, and lymphoma is also well known. We report a rare combination of unicentric, extrathoracic Castleman′s disease with PNP and myasthenia gravis.

  20. Identification and characterization of a new multigene family in the human MHC: A candidate autoimmune disease susceptibility element (3.8-1)

    Energy Technology Data Exchange (ETDEWEB)

    Harris, J.M.; Venditti, C.P.; Chorney, M.J. [Pennsylvania State Univ. College of Medicine, Hershey, PA (United States)

    1994-09-01

    An association between idiopathic hemochromatosis (HFE) and the HLA-A3 locus has been previously well-established. In an attempt to identify potential HFE candidate genes, a genomic DNA fragment distal to the HLA-A9 breakpoint was used to screen a B cell cDNA library; a member (3.8-1) of a new multigene family, composed of five distinct genomic cross-reactive fragments, was identified. Clone 3.8-1 represents the 3{prime} end of 9.6 kb transcript which is expressed in multiple tissues including the spleen, thymus, lung and kidney. Sequencing and genome database analysis indicate that 3.8-1 is unique, with no homology to any known entries. The genomic residence of 3-8.1, defined by polymorphism analysis and physical mapping using YAC clones, appears to be absent from the genomes of higher primates, although four other cross-reactivities are maintained. The absence of this gene as well as other probes which map in the TNF to HLA-B interval, suggest that this portion of the human HMC, located between the Class I and Class III regions, arose in humans as the result of a post-speciation insertional event. The large size of the 3.8-1 gene and the possible categorization of 3.8-1 as a human-specific gene are significant given the genetic data that place an autoimmune susceptibility element for IDDM and myasthenia gravis in the precise region where this gene resides. In an attempt to isolate the 5{prime} end of this large transcript, we have constructed a cosmid contig which encompasses the genomic locus of this gene and are progressively isolating coding sequences by exon trapping.

  1. Autoimmune epilepsy.

    Science.gov (United States)

    Greco, Antonio; Rizzo, Maria Ida; De Virgilio, Armando; Conte, Michela; Gallo, Andrea; Attanasio, Giuseppe; Ruoppolo, Giovanni; de Vincentiis, Marco

    2016-03-01

    Despite the fact that epilepsy is the third most common chronic brain disorder, relatively little is known about the processes leading to the generation of seizures. Accumulating data support an autoimmune basis in patients with antiepileptic drug-resistant seizures. Besides, recent studies show that epilepsy and autoimmune disease frequently co-occur. Autoimmune epilepsy is increasingly recognized in the spectrum of neurological disorders characterized by detection of neural autoantibodies in serum or spinal fluid and responsiveness to immunotherapy. An autoimmune cause is suspected based on frequent or medically intractable seizures and the presence of at least one neural antibody, inflammatory changes indicated in serum or spinal fluid or on MRI, or a personal or family history of autoimmunity. It is essential that an autoimmune etiology be considered in the initial differential diagnosis of new onset epilepsy, because early immunotherapy assures an optimal outcome for the patient.

  2. [Autoimmune hepatitis].

    Science.gov (United States)

    Ostojić, Rajko

    2003-01-01

    Autoimmune hepatitis is an unresolving, hepatocellular inflammation of unknown cause that is characterized by the presence of periportal hepatitis on histologic examination, tissue autoantibodies in serum, and hypergammaglobulinemia. By international consensus, the designation autoimmune hepatitis has replaced alternative terms for the condition. Three types of autoimmune hepatitis have been proposed based on immunoserologic findings. Type 1 autoimmune hepatitis is characterized by the presence of antinuclear antibodies (ANA) or smooth muscle antibodies (SMA) (or both) in serum. Seventy percent of patients with type 1 of autoimmune hepatitis are women. This type is the most common form and accounts for at least 80% of cases. Type 2 is characterized by the presence of antibodies to liver-kidney microsome type 1 (anti-LKM1) in serum. Patients with this type of autoimmune hepatitis are predominantly children. Type 3 autoimmune hepatitis is characterized by the presence of antibodies to soluble liver antigen (anti-SLA) in serum. There are no individual features that are pathognomonic of autoimmune hepatitis, and its diagnosis requires the confident exclusion of other conditions. The large majority of patients show satisfactory response to corticosteroid (usually prednisone or prednisolone) therapy. For the past 30 years it has been customary to add azathioprine as a "steroid sparing" agent to allow lower doses of steroids to be used and remission, once achieved, can be sustained in many patients with azathioprine alone after steroid withdrawal. Patients with autoimmune hepatitis who have decompensated during or after corticosteroid therapy are candidates for liver transplantation.

  3. Autoimmune hepatitis

    Science.gov (United States)

    ... PA: Elsevier Saunders; 2010:chap 88. Read More Autoimmune disorders Chronic thyroiditis (Hashimoto disease) Cirrhosis Glomerulonephritis Hemolytic anemia Liver cancer - hepatocellular carcinoma Mesenteric venous thrombosis Type ...

  4. Learning about Antiphospholipid Syndrome (APS)

    Science.gov (United States)

    ... other autoimmune disorders, including myasthenia gravis, Graves' disease, autoimmune hemolytic anemia, and Evan's syndrome. Top of page How is APS diagnosed? A diagnosis of APS is made based on both clinical and ... Autoimmune Related Diseases Association, Inc. [aarda.org] An organization ...

  5. Autoimmune myelopathies.

    Science.gov (United States)

    Flanagan, Eoin P

    2016-01-01

    Autoimmune myelopathies are a heterogeneous group of immune-mediated spinal cord disorders with a broad differential diagnosis. They encompass myelopathies with an immune attack on the spinal cord (e.g., aquaporin-4-IgG (AQP4-IgG) seropositive neuromyelitis optica (NMO) and its spectrum disorders (NMOSD)), myelopathies occurring with systemic autoimmune disorders (which may also be due to coexisting NMO/NMOSD), paraneoplastic autoimmune myelopathies, postinfectious autoimmune myelopathies (e.g., acute disseminated encephalomyelitis), and myelopathies thought to be immune-related (e.g., multiple sclerosis and spinal cord sarcoidosis). Spine magnetic resonance imaging is extremely useful in the evaluation of autoimmune myelopathies as the location of signal change, length of the lesion, gadolinium enhancement pattern, and evolution over time narrow the differential diagnosis considerably. The recent discovery of multiple novel neural-specific autoantibodies accompanying autoimmune myelopathies has improved their classification. These autoantibodies may be pathogenic (e.g., AQP4-IgG) or nonpathogenic and more reflective of a cytotoxic T-cell-mediated autoimmune response (collapsin response mediator protein-5(CRMP5)-IgG). The presence of an autoantibody may help guide cancer search, assist treatment decisions, and predict outcome/relapse. With paraneoplastic myelopathies the initial goal is detection and treatment of the underlying cancer. The aim of immunotherapy in all autoimmune myelopathies is to maximize reversibility, maintain benefits (while preventing relapse), and minimize side effects.

  6. Management of disease-modifying treatments in neurological autoimmune diseases of the central nervous system

    Science.gov (United States)

    Salmen, A; Gold, R; Chan, A

    2014-01-01

    sclerosis. Clinical and Experimental Immunology 2014, 175: 373–84. CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Review of an increasingly recognized entity within the spectrum of inflammatory central nervous system disorders. Clinical and Experimental Immunology 2014, 175: 385–96. Requirement for safety monitoring for approved multiple sclerosis therapies: an overview. Clinical and Experimental Immunology 2014, 175: 397–407. Myasthenia gravis: an update for the clinician. Clinical and Experimental Immunology 2014, 175: 408–18. Cerebral vasculitis in adults: what are the steps in order to establish the diagnosis? Red flags and pitfalls. Clinical and Experimental Immunology 2014, 175: 419–24. Multiple sclerosis treatment and infectious issues: update 2013. Clinical and Experimental Immunology 2014, 175: 425–38. Diagnosis, pathogenesis and treatment of myositis: recent advances 2014, 175: 349–58. Neuromyelitis optica: clinical features, immunopathogenesis and treatment 2014, 176: 149–64. PMID:24358961

  7. Autoimmune disorders

    Science.gov (United States)

    ... as azathioprine, cyclophosphamide, mycophenolate, sirolimus, or tacrolimus. Targeted drugs called tumor necrosis factor (TFN) blockers can be used for some diseases. Outlook (Prognosis) The outcome depends on the disease. Most autoimmune diseases are chronic , but many can be controlled ...

  8. Autoimmune hepatitis.

    Science.gov (United States)

    Heneghan, Michael A; Yeoman, Andrew D; Verma, Sumita; Smith, Alastair D; Longhi, Maria Serena

    2013-10-26

    Autoimmune hepatitis is a disease of the hepatic parenchyma that can present in acute or chronic forms. In common with many autoimmune diseases, autoimmune hepatitis is associated with non-organ-specific antibodies in the context of hepatic autoimmunity. This dichotomy has made definition of a unifying hypothesis in the pathophysiology of the disease difficult, although data from the past 8 years have drawn attention to the role of regulatory T cells. Several triggers have been identified, and the disease arises in genetically susceptible individuals. Clinical and biochemical remission is achievable in up to 85% of cases. For the remaining patients, alternative immunosuppression strategies are an option. Liver transplantation provides an excellent outcome for patients with acute liver failure or complications of end-stage liver disease, including hepatocellular carcinoma. Variant or overlapping syndromes are worthy of consideration when unexpected disease features arise.

  9. Autoimmune hypophysitis.

    Science.gov (United States)

    Ezzat, S; Josse, R G

    1997-03-01

    Autoimmune (lymphocytic) hypophysitis has emerged as a distinct and specific clinical and pathological disease entity. Although relatively rare compared with other autoimmune endocrine diseases, nearly a hundred cases have been described. The condition is much more common in females (9:1) and appears to have a particular predilection for the pregnant and postpartum states. The anterior pituitary, and less often the neurohypophysis, appear to be the target for inflammatory autoimmune destruction. During the evolution of the disease process, pituitary hyperfunction (usually hyperprolactinemia) has been noted. This disease should now be included in the differential diagnosis of pituitary disorders, especially in females presenting with pituitary enlargement, particularly if symptoms occur in temporal relationship to pregnancy. The disease may form part of the spectrum of the polyglandular autoimmune endocrine disorders. (Trends Endocrinol Metab 1997;8:74-80). (c) 1997, Elsevier Science Inc.

  10. Detection of Acetyicholing Receptor and Presynaptic Membrane Antibodies in Serum and its Clinical Significance from Patients with Myasthemia Gravis%重症肌无力患者血清中乙酰胆碱受体、突触前膜抗体检测的临床意义

    Institute of Scientific and Technical Information of China (English)

    施国文; 沈崇欣

    2002-01-01

    Objective To observe the relationship between the detectable rate of Acetylcholine re-ceptor and presynaptic membrane antibodies in serum from Myasthenia Gravis(MG)patients with Osser-man clincal typing and concomitant thymoma and efficacy of adreno - corticosteroid therapty. MethodsAntibodies to AchR and Presynaptic membrance protein was detected by ELISA from patient with MG.Results The detectable rate from MG patients in clinical typing Osserman I and from patients not con-comitant thymoma were lower than others. Adreno - corticosteroid treat MG had good effect. ConclusionThe results indicate that detection AchR and Psm antibodies in serum was accessory diagnosetic method.

  11. Autoimmun hypophysitis

    DEFF Research Database (Denmark)

    Krarup, Therese; Hagen, Claus

    2010-01-01

    Autoimmune hypophysitis (AH) - often referred to as lymphocytic hypophysitis - is a rare disease that affects the pituitary gland and causes inflammation. The disease enlarges the pituitary gland and the clinical presentations are lack of pituitary function and headaches. AH is mostly seen in women...... during pregnancy or postpartum, but also occurs in males and children. AH is often associated with other autoimmune diseases, most frequently with Hashimoto's thyroiditis. The symptoms are caused by enlargement of the pituitary gland and disturbances of the hormone function. Treatment is either...

  12. Autoimmune disease

    Institute of Scientific and Technical Information of China (English)

    2005-01-01

    2005164 Optimal cut-point of glutamic acid decar-boxylase antibody (GAD-Ab) for differentiating two subtypes of latent autoimmune diabetes in adults (LADA). LI Xia(李霞), et al. Dept Endocrinol, 2nd Xiangya Hosp, Central South Univ, Changsha, 410011. Chin J Diabetes, 2005;13(1) :34-38. Objective: To investigate the optimal cut-point of glutamate decarboxylase antibody (GAD-Ab) for differentiating two subtypes of latent autoimmune diabetes in adults (I. ADA). Methods: The frequency

  13. Autosomal recessive limb girdle myasthenia in two sisters.

    Directory of Open Access Journals (Sweden)

    Shankar A

    2002-10-01

    Full Text Available Limb girdle myasthenic syndromes are rare genetic disorders described under the broad heterogeneous group known as congenital myasthenic syndromes and present with mixed features of myasthenia and myopathy. The familial limb girdle myasthenia has been described as one with selective weakness of pectoral and pelvic girdles, showing a positive response to edrophonium chloride. A report of two sisters affected by this disorder is presented.

  14. Autoimmune sialadenitis

    NARCIS (Netherlands)

    Guntinas-Lichius, O.; Vissink, A.; Ihrler, S.

    2010-01-01

    Using the European-American classification criteria the diagnosis of autoimmune sialadenitis in Sjogren's syndrome can generally be easily established or excluded. In addition, sonography performed by the ENT physician is helpful in diagnosing and especially in follow-up screening for MALT lymphomas

  15. The use of intravenous immunoglobulin in the treatment of autoimmune neuromuscular diseases: evidence-based indications and safety profile.

    Science.gov (United States)

    Dalakas, Marinos C

    2004-06-01

    Intravenous immunoglobulin (i.v.Ig) has multiple actions on the immunoregulatory network that operate in concert with each other. For each autoimmune neuromuscular disease, however, there is a predominant mechanism of action that relates to the underlying immunopathogenetic cause of the respective disorder. The best understood actions of i.v.Ig include the following: (a) modulation of pathogenic autoantibodies, an effect relevant in myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS), Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and stiff-person syndrome (SPS); (b) inhibition of complement activation and interception of membranolytic attack complex (MAC) formation, an action relevant to the complement-mediated mechanisms involved in GBS, CIDP, MG, and dermatomyositis (DM); (c) modulation of the inhibitory or activation Fc receptors on macrophages invading targeted tissues in nerve and muscle, as seen in CIDP, GBS, and inflammatory myopathies; (d) down-regulation of pathogenic cytokines and adhesion molecules; (e) suppression of T-cell functions; and (f) interference with antigen recognition. Controlled clinical trials have shown that i.v.Ig is effective as first-line therapy in patients with GBS, CIDP, and multifocal motor neuropathy (MMN), and as second-line therapy in DM, MG, LEMS, and SPS. In paraproteinemic IgM anti-MAG (myelin-associated glycoprotein) demyelinating polyneuropathies and inclusion body myositis (IBM), the benefit is variable, marginal, and not statistically significant. i.v.Ig has a remarkably good safety record for long-term administration, however, the following side effects have been observed: mild, infusion-rate-related reactions, such as headaches, myalgia, or fever; moderate but inconsequential events, such as aseptic meningitis and skin rash; and severe, but rare, complications, such as thromboembolic events and renal tubular necrosis. Future studies are needed to (a) find the

  16. Efficacy Evaluation of Thymectomy in Myasthenia Patients

    Directory of Open Access Journals (Sweden)

    A.P. Reshetov

    2009-06-01

    Full Text Available The condition of 71 patients with myasthenia after thymectomy has been analyzed. Supervision has been carried out from 6 months till 9 years. Thymic hyperplasia has been marked at histological research of operational material in 46,5% of patients. Thymic tumors have been revealed in 50,7% of cases, thymomas have been noticed in 16,7% of cases. In 2 cases (2,8% atrophy of the thymus gland has been revealed. The excellent and good effects of surgical treatment have been received at 73,8 % of patients, in case of thymic hyperplasia in 76,7 %, and at thymic tumors — in 69,7%. Efficiency dependence includes surgery term according to the disease onset and the age of patients by the moment of operation. Thus, the excellent and good results have been received in 82 % of patients operated within the first two years after the onset of the disease.

  17. Autoimmun pankreatitis

    DEFF Research Database (Denmark)

    Fjordside, Eva; Novovic, Srdan; Schmidt, Palle Nordblad;

    2015-01-01

    Autoimmune pancreatitis (AIP) is a rare inflammatory disease. AIP has characteristic histology, serology and imaging findings. Two types of AIP exist, type 1, which is a part of the systemic immunoglobulin G4-related disease, and type 2, which is only localized to the pancreas. Patients with type 1...... are predominantly older men, have involvement of other organs and more often experience relapse than patients with type 2. Both types respond well to steroid treatment. The most important differential diagnose is pancreatic cancer....

  18. Sedenion unified theory of gravi-electromagnetism

    Science.gov (United States)

    Chanyal, B. C.

    2014-11-01

    In this paper, we represent 16-component sedenions, the generalization of octonions, which is noncommutative space-time algebra. The sedenions is neither a composition algebra nor a division algebra because it has zero divisors. Here we have formulated the sedenionic unified potential equations, unified fields equations and unified current equations of dyons and gravito-dyons. We have developed the sedenionic unified theory of dyons and gravito-dyons in terms of two eight-potentials leading to the structural symmetry between generalized electromagnetic fields of dyons and generalized gravito-Heavisidian fields of gravito-dyons. Thus we have obtained the sedenionic form of generalized Dirac-Maxwell's equations, unified work-energy theorem (Poynting theorem), generalized unified gravi-electromagnetic force and other quantum equations of dyons and gravito-dyons in simple, compact and consistent way incorporating the non-associativity and non-commutativity of sedenion variables.

  19. Autoimmune liver disease panel

    Science.gov (United States)

    Liver disease test panel - autoimmune ... Autoimmune disorders are a possible cause of liver disease. The most common of these diseases are autoimmune hepatitis and primary biliary cirrhosis. This group of tests helps your health care provider ...

  20. Autoimmune hepatitis

    Directory of Open Access Journals (Sweden)

    F Motamed

    2014-04-01

    Full Text Available Autoimmune hepatitis is (AIH is a chronic hepatitis that occurs in children and adults of all ages. It is characterized by immunologic and autoimmune features, including circulating auto antibodies and high serum globulin concentrations. It was first described in the 1950s by term of chronic active hepatitis. It has 2 types with different auto antibodies. Diagnosis is based upon serologic and histologic findings and exclusion of other forms of chronic liver disease.   A scoring system should be used in assessment based upon: 1 Auto anti bodie titer 2 Serum IgG level  3 Liver histology 4 Absence of viral and other causes of hepatitis. Clear indications for treatment: 1   rise of aminotrasferases 2   clinical symptoms of liver disease 3   histological features in liver biopsy 4   Children with AIH initial treatment involve glucocorticoid with or without azathioprine. For patients with fulminant hepatitis liver transplantation, should be kept in mind.   Remission is defined by: 1   Resolution of symptoms 2   Normalization of serum trasaminases 3   Normalization of serum bilirubin and gamma globuline levels. 4   Improvement in liver histology 5   Treatment is continued for at least 2-5 years, glucocorticoids are with drawn first, by tapering over six weeks. Azathioprine will be with drawn.  

  1. [Autoimmune pancreatitis].

    Science.gov (United States)

    Beyer, G; Menzel, J; Krüger, P-C; Ribback, S; Lerch, M M; Mayerle, J

    2013-11-01

    Autoimmune pancreatitis is a relatively rare form of chronic pancreatitis which is characterized by a lymphoplasmatic infiltrate with a storiform fibrosis and often goes along with painless jaundice and discrete discomfort of the upper abdomen. Clinically we distinguish between two subtypes, which differ in terms of their histology, clinical picture and prognosis. Type 1 autoimmune pancreatitis is the pancreatic manifestation of the IgG4-associated syndrome which also involves other organs. About one third of the patients can only be diagnosed after either histological prove or a successful steroid trail. Type 2 is IgG4-negative with the histological picture of an idiopathic duct centric pancreatitis and is to higher degree associated with inflammatory bowel disease. A definitive diagnosis can only be made using biopsy. Usually both forms show response to steroid treatment, but in type 1 up to 50 % of the patients might develop a relapse. The biggest challenge and most important differential diagnosis remains the discrimination of AIP from pancreatic cancer, because also AIP can cause mass of the pancreatic head, lymphadenopathy and ductal obstruction. This article summarizes recent advances on epidemiology, clinical presentation, diagnostic strategy, therapy and differential diagnosis in this relatively unknown disease.

  2. Oral Tolerance: Therapeutic Implications for Autoimmune Diseases

    Directory of Open Access Journals (Sweden)

    Ana M. C. Faria

    2006-01-01

    Full Text Available Oral tolerance is classically defined as the suppression of immune responses to antigens (Ag that have been administered previously by the oral route. Multiple mechanisms of tolerance are induced by oral Ag. Low doses favor active suppression, whereas higher doses favor clonal anergy/deletion. Oral Ag induces Th2 (IL-4/IL-10 and Th3 (TGF-β regulatory T cells (Tregs plus CD4+CD25+ regulatory cells and LAP+T cells. Induction of oral tolerance is enhanced by IL-4, IL-10, anti-IL-12, TGF-β, cholera toxin B subunit (CTB, Flt-3 ligand, anti-CD40 ligand and continuous feeding of Ag. In addition to oral tolerance, nasal tolerance has also been shown to be effective in suppressing inflammatory conditions with the advantage of a lower dose requirement. Oral and nasal tolerance suppress several animal models of autoimmune diseases including experimental allergic encephalomyelitis (EAE, uveitis, thyroiditis, myasthenia, arthritis and diabetes in the nonobese diabetic (NOD mouse, plus non-autoimmune diseases such as asthma, atherosclerosis, colitis and stroke. Oral tolerance has been tested in human autoimmune diseases including MS, arthritis, uveitis and diabetes and in allergy, contact sensitivity to DNCB, nickel allergy. Positive results have been observed in phase II trials and new trials for arthritis, MS and diabetes are underway. Mucosal tolerance is an attractive approach for treatment of autoimmune and inflammatory diseases because of lack of toxicity, ease of administration over time and Ag-specific mechanism of action. The successful application of oral tolerance for the treatment of human diseases will depend on dose, developing immune markers to assess immunologic effects, route (nasal versus oral, formulation, mucosal adjuvants, combination therapy and early therapy.

  3. INTEGRATED METHODOLOGY OF I.I. MECHNIKOV AND MODERN ADDRESS IMMUNOCORRECTION AT MYASTHENIA

    Directory of Open Access Journals (Sweden)

    Klimova E.M.

    2015-05-01

    neutrophils and completeness of phagocytosis has revealed various degree of disturbances of barrier function of phagocyte cells, and also change of the titer and frequencies of occurrence of herpes viruses CMV and VEB at various clinical phenotypes of myasthenia. Conclusion. It is shown, that degree of expression and frequency of occurrence of persistence of cytomegalovirus and Epstein-Barra virus are interconnected with defects of various stages of phagocytosis, its completeness and activity of the intracellular enzymes which participate in formation of active forms of oxygen, and intensity of formation of monomers of DNA of the microorganisms which are grasped by phagocytes. Disturbance of barrier function of immunity at myasthenia is significant for a debut and disease advance. An estimation of phagocytosis and virus persistence, as trigger factor of myasthenia, is important for a choice of target therapy. Use of evolutionary scientific methodology of I.I. Mechnikov in the synthetic approach for diagnostics of trigger factors and monitoring of stage of formations of pathological process allows to prove individual medical-diagnostic algorithm for correction of various clinical forms of autoimmune diseases. The approaches for address therapy are proved at various clinical phenotypes of myasthenia which includes carrying out of staged monoligand therapy by peptides and enzymes. This therapy referred on activation of intracellular enzymes of neutrophils at incompleteness of phagocytosis, and application of the immunoglobulins which are specific to herpes viruses.

  4. Miastenia gravis familiar: registro de dois irmãos

    Directory of Open Access Journals (Sweden)

    Marcos R. G. de Freitas

    1985-03-01

    Full Text Available A forma familiar da miastenia gravis é condição relativamente rara, ocorrendo em cerca de 3,4% dos pacientes miastênicos. Os autores registram os casos de dois irmãos não-gêmeos, apresentando miastenia gravis com acometimento predominantemente ocular desde o nascimento. Um terceiro irmão faleceu na infância, provavelmente com a mesma afecção. Os pais eram consanguíneos. Estudo da incidência da forma familiar da miastenia e de seus aspectos clínicos, genéticos e terapêuticos complementa os registros.

  5. Miastenia gravis familiar: registro de dois irmãos

    OpenAIRE

    1985-01-01

    A forma familiar da miastenia gravis é condição relativamente rara, ocorrendo em cerca de 3,4% dos pacientes miastênicos. Os autores registram os casos de dois irmãos não-gêmeos, apresentando miastenia gravis com acometimento predominantemente ocular desde o nascimento. Um terceiro irmão faleceu na infância, provavelmente com a mesma afecção. Os pais eram consanguíneos. Estudo da incidência da forma familiar da miastenia e de seus aspectos clínicos, genéticos e terapêuticos complementa os reg...

  6. The autoimmune tautology.

    Science.gov (United States)

    Anaya, Juan-Manuel

    2010-01-01

    Although autoimmune diseases exhibit contrasting epidemiological features, pathology, and clinical manifestations, three lines of evidence demonstrate that these diseases share similar immunogenetic mechanisms (that is, autoimmune tautology). First, clinical evidence highlights the co-occurrence of distinct autoimmune diseases within an individual (that is, polyautoimmunity) and within members of a nuclear family (that is, familial autoimmunity). Second, physiopathologic evidence indicates that the pathologic mechanisms may be similar among autoimmune diseases. Lastly, genetic evidence shows that autoimmune phenotypes might represent pleiotropic outcomes of the interaction of non-specific disease genes.

  7. Thymic hyperplasia in Graves’ disease

    OpenAIRE

    Narendra Kotwal; Yashpal Singh; Anil Menon; Vineet Behera

    2013-01-01

    Graves′ disease is an autoimmune thyroid condition characterized by the production of autoantibodies against the thyrotropin receptor. It is known to be associated with autoimmune conditions such as myasthenia gravis, Addison′s disease, type 1 diabetes mellitus, and vitiligo. We present a case of rare autoimmune association of Graves′ disease with thymic hyperplasia which regressed after treatment with antithyroid drugs. Exact pathophysiology of thymic hyperplasia in Graves′ is not well under...

  8. Disorders of the neuromuscular junction

    NARCIS (Netherlands)

    Kuks, JBM; de Baets, MH

    2000-01-01

    Acquired myasthenic syndromes are mostly autoimmune diseases with antibodies directed to ion channels of the presynaptic (Lambert Eaten Myasthenic Syndrome) or postsynaptic (Myasthenia Gravis) membrane. Although both of these syndromes can be treated rather effectively there is still a need for find

  9. Perspectives on autoimmunity

    Energy Technology Data Exchange (ETDEWEB)

    Cohen, I.R.

    1987-01-01

    The contents of this book are: HLA and Autoimmunity; Self-Recognition and Symmetry in the Immune System; Immunology of Insulin Dependent Diabetes Mellitus; Multiple Sclerosis; Autoimmunity and Immune Pathological Aspects of Virus Disease; Analyses of the Idiotypes and Ligand Binding Characteristics of Human Monoclonal Autoantibodies to DNA: Do We Understand Better Systemic Lupus Erythematosus. Autoimmunity and Rheumatic Fever; Autoimmune Arthritis Induced by Immunization to Mycobacterial Antigens; and The Interaction Between Genetic Factors and Micro-Organisms in Ankylosing Spondylitis: Facts and Fiction.

  10. Autoimmune Hemolytic Anemia.

    Science.gov (United States)

    Liebman, Howard A; Weitz, Ilene C

    2017-03-01

    Autoimmune hemolytic anemia is an acquired autoimmune disorder resulting in the production of antibodies directed against red blood cell antigens causing shortened erythrocyte survival. The disorders can present as a primary disorder (idiopathic) or secondary to other autoimmune disorders, malignancies, or infections. Treatment involves immune modulation with corticosteroids and other agents.

  11. Sirolimus for Autoimmune Disease of Blood Cells

    Science.gov (United States)

    2017-03-16

    Autoimmune Pancytopenia; Autoimmune Lymphoproliferative Syndrome (ALPS); Evans Syndrome; Idiopathic Thrombocytopenic Purpura; Anemia, Hemolytic, Autoimmune; Autoimmune Neutropenia; Lupus Erythematosus, Systemic; Inflammatory Bowel Disease; Rheumatoid Arthritis

  12. Galectin-3 in autoimmunity and autoimmune diseases.

    Science.gov (United States)

    de Oliveira, Felipe L; Gatto, Mariele; Bassi, Nicola; Luisetto, Roberto; Ghirardello, Anna; Punzi, Leonardo; Doria, Andrea

    2015-08-01

    Galectin-3 (gal-3) is a β-galactoside-binding lectin, which regulates cell-cell and extracellular interactions during self/non-self-antigen recognition and cellular activation, proliferation, differentiation, migration and apoptosis. It plays a significant role in cellular and tissue pathophysiology by organizing niches that drive inflammation and immune responses. Gal-3 has some therapeutic potential in several diseases, including chronic inflammatory disorders, cancer and autoimmune diseases. Gal-3 exerts a broad spectrum of functions which differs according to its intra- or extracellular localization. Recombinant gal-3 strategy has been used to identify potential mode of action of gal-3; however, exogenous gal-3 may not reproduce the functions of the endogenous gal-3. Notably, gal-3 induces monocyte-macrophage differentiation, interferes with dendritic cell fate decision, regulates apoptosis on T lymphocytes and inhibits B-lymphocyte differentiation into immunoglobulin secreting plasma cells. Considering the influence of these cell populations in the pathogenesis of several autoimmune diseases, gal-3 seems to play a role in development of autoimmunity. Gal-3 has been suggested as a potential therapeutic agent in patients affected with some autoimmune disorders. However, the precise role of gal-3 in driving the inflammatory process in autoimmune or immune-mediated disorders remains elusive. Here, we reviewed the involvement of gal-3 in cellular and tissue events during autoimmune and immune-mediated inflammatory diseases.

  13. Autoimmune pancreatitis: A review

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Autoimmune pancreatitis has emerged over the last 40 years from a proposed concept to a well established and recognized entity. As an efficient mimicker of pancreatic carcinoma, its early and appropriate recognition are crucial. With mounting understanding of its pathogenesis and natural history, significant advances have been made in the diagnosis of autoimmune pancreatitis. The characteristic laboratory features and imaging seen in autoimmune pancreatitis are reviewed along with some of the proposed diagnostic criteria and treatment algorithms.

  14. Timectomía por miastenia gravis en el Hospital Dr. Rafael Angel Calderón Guardia: Reporte de 24 casos

    Directory of Open Access Journals (Sweden)

    José A Mainieri Hidalgo

    2000-06-01

    Full Text Available Antecedentes: La miastenia gravis es una enfermedad autoinmune cuya etiología aún no está claramente definida. Desde 1912 se describió mejoría del cuadro posterior a timectomía. Este procedimiento es actualmente una de las formas aceptadas para su tratamiento. El objetivo del presente trabajo fue estudiar los resultados de la timectomía para el tratamiento de la miastenia gravis en el Hospital Rafael Angel Calderón Guardia. Materiales y Métodos: Estudio retrospectivo, basado en la revisión de los expedientes de 24 pacientes, a quienes se les realizó timectomía como parte de su tratamiento de miastenia gravis entre enero de 1992 y mayo de 1999, en el Hospital Dr. Rafael Angel Calderón Guardia. Resultados: Se encontró que un 75% de los pacientes presentaron una franca mejoría, 17% permaneció en igual condición y un 8% empeoró. No se observó diferencia en la evolución por sexo, edad o la presencia de timoma, sin embargo en los pacientes que presentaban únicamente síntomas oculares se dio una tendencia a persistir sintomáticos. Se observó que la preparación pre-operatoria del paciente con gamaglobulina y la modernización de la técnica anestésica disminuyó notablemente la necesidad de ventilación mecánica. Conclusión: La timectomía fue útil en el tratamiento del 75% de los pacientes, especialmente aquellos con no sólo ptosis palpebral y permitió disminuir la dosis de medicamentos en 12 de los 24 casos estudiados.Background: Miastenia gravis is an autoimmune disease, its etiology has not been defined clearly. The improvement of the clinical manifestations after thymectomy was first describes in 1912; and this procedure has become in one of the therapeutic alternativas for the disease nowadays. The aim of the present study was to investigase the results of thymectomy for miastenia gravis in the Hospital Dr. R. A. Calderón Guardia. Materials and Methods: Retrospective study, based in the revision of the clinical

  15. [Syndrome overlap: autoimmune hepatitis and autoimmune cholangitis].

    Science.gov (United States)

    Guerra Montero, Luis; Ortega Alvarez, Félix; Marquez Teves, Maguin; Asato Higa, Carmen; Sumire Umeres, Julia

    2016-01-01

    Autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis and autoimmune cholangitis are chronic autoimmune liver disease, usually present separate, the cases where characteristics of two of the above is observed liver disease is commonly referred to as Overlap Syndromes (OS). Although there is no consensus on specific criteria for the diagnosis of OS identification of this association is important for initiating appropriate treatment and prevent its progression to cirrhosis or at least the complications of cirrhosis and death. We report the case of awoman aged 22 cirrhotic which debuted are edematous ascites, severe asthenia and jaundice compliant diagnostics SS criteria and initially present any response to treatment with ursodeoxycholic acid and oral corticosteroids, but ultimately finished performing a transplant orthotopic liver.

  16. THE AUTOIMMUNE ECOLOGY.

    Directory of Open Access Journals (Sweden)

    Juan-Manuel eAnaya

    2016-04-01

    Full Text Available Autoimmune diseases (ADs represent a heterogeneous group of disorders that affect specific target organs or multiple organ systems. These conditions share common immunopathogenic mechanisms (i.e., the autoimmune tautology, which explain the clinical similarities they have among them as well as their familial clustering (i.e., coaggregation. As part of the autoimmune tautology, the influence of environmental exposure on the risk of developing ADs is paramount (i.e., the autoimmune ecology. In fact, environment, more than genetics, shapes immune system. Autoimmune ecology is akin to exposome, that is all the exposures - internal and external - across the lifespan, interacting with hereditary factors (both genetics and epigenetics to favor or protect against autoimmunity and its outcomes. Herein we provide an overview of the autoimmune ecology, focusing on the immune response to environmental agents in general, and microbiota, cigarette smoking, alcohol and coffee consumption, socioeconomic status, gender and sex hormones, vitamin D, organic solvents and vaccines in particular. Inclusion of the autoimmune ecology in disease etiology and health will improve the way personalized medicine is currently conceived and applied.

  17. Bistability in autoimmune diseases

    DEFF Research Database (Denmark)

    Rapin, Nicolas; Mosekilde, Erik; Lund, Ole

    2011-01-01

    Autoimmune diseases damage host tissue, which, in turn, may trigger a stronger immune response. Systems characterized by such positive feedback loops can display co-existing stable steady states. In a mathematical model of autoimmune disease, one steady state may correspond to the healthy state...

  18. The Autoimmune Ecology.

    Science.gov (United States)

    Anaya, Juan-Manuel; Ramirez-Santana, Carolina; Alzate, Maria A; Molano-Gonzalez, Nicolas; Rojas-Villarraga, Adriana

    2016-01-01

    Autoimmune diseases (ADs) represent a heterogeneous group of disorders that affect specific target organs or multiple organ systems. These conditions share common immunopathogenic mechanisms (i.e., the autoimmune tautology), which explain the clinical similarities they have among them as well as their familial clustering (i.e., coaggregation). As part of the autoimmune tautology, the influence of environmental exposure on the risk of developing ADs is paramount (i.e., the autoimmune ecology). In fact, environment, more than genetics, shapes immune system. Autoimmune ecology is akin to exposome, that is all the exposures - internal and external - across the lifespan, interacting with hereditary factors (both genetics and epigenetics) to favor or protect against autoimmunity and its outcomes. Herein, we provide an overview of the autoimmune ecology, focusing on the immune response to environmental agents in general, and microbiota, cigarette smoking, alcohol and coffee consumption, socioeconomic status (SES), gender and sex hormones, vitamin D, organic solvents, and vaccines in particular. Inclusion of the autoimmune ecology in disease etiology and health will improve the way personalized medicine is currently conceived and applied.

  19. Things to Consider If You Have MG and Are Thinking about Getting Pregnant

    Science.gov (United States)

    ... diagnosis and optimal care of individuals affected by myasthenia gravis and closely related disorders and to improve their ... the authors, but not necessarily those of the Myasthenia Gravis Foundation of America (MGFA). Any reference to a ...

  20. Eyelid drooping

    Science.gov (United States)

    ... include: Tumor around or behind the eye Diabetes Horner syndrome Myasthenia gravis Stroke Swelling in the eyelid, ... Read More Amblyopia Brain tumor - children Cancer Diabetes Horner syndrome Myasthenia gravis Stroke Review Date 8/20/ ...

  1. Swallowing Disorders

    Science.gov (United States)

    ... Huntington disease, and myasthenia gravis. Muscular dystrophy and myotonic dystrophy are accompanied by dysphagia, which is also the ... Huntington disease, and myasthenia gravis. Muscular dystrophy and myotonic dystrophy are accompanied by dysphagia, which is also the ...

  2. Autoimmune Cholangitis: A Variant Syndrome of Autoimmune Hepatitis

    OpenAIRE

    Brij Sharma; Sujeet Raina; Rajesh Sharma

    2014-01-01

    Autoimmune cholangitis (AIC) or autoimmune cholangiopathy is a chronic inflammation of liver and a variant syndrome of autoimmune hepatitis (AIH). We present a case of an adult female who had biochemical features of cholestasis and transaminasemia but aminotransferases were not in the hepatitis range and had histological evidence of bile duct injury which was subsequently diagnosed as autoimmune cholangitis.

  3. Psoriasis and autoimmunity.

    Science.gov (United States)

    Sticherling, Michael

    2016-12-01

    Psoriasis is one of the most common chronic inflammatory human skin diseases. Though clinically well characterized, the exact etiological and pathogenic mechanisms are still not known in detail. Current knowledge indicates distinct overlap to other inflammatory as well as autoimmune disorders. However, the one or more relevant autoantigens could not be characterized so-far. On the other side, several autoimmune diseases were shown to be associated with psoriasis. In addition, serological autoimmune phenomena, namely diverse circulating specific autoantibodies could be demonstrated in the past. A matter of current debate is if psoriasis is a primary autoimmune disease or secondarily evolving into autoimmunity as seen in other chronic inflammatory diseases. Related to this aspect is the concept of autoinflammation versus autoimmunity where psoriasis shares mechanisms of both entities. Though T-cells remain among the most important cellular players in the pathogenesis of psoriasis and current therapeutic strategies successfully target these cells or their products irrespective of these concepts, autoimmunity if relevant will add to the treatment armamentarium by using protective and prophylactic antigen-specific modalities.

  4. Immunometabolism and autoimmunity.

    Science.gov (United States)

    Freitag, Jenny; Berod, Luciana; Kamradt, Thomas; Sparwasser, Tim

    2016-11-01

    A continuous increase in the prevalence of autoimmune diseases is to be expected in the aging societies worldwide. Autoimmune disorders not only cause severe disability and chronic pain, but also lead to considerable socio-economic costs. Given that the current treatment options are not curative, have substantial side effects and a high percentage of non-responders, innovative options to the existing therapeutic armament against autoimmune diseases are urgently required. Accumulating evidence suggests that changes in the metabolism of immune cells are associated with, and contribute to the pathogenesis of autoimmunity. Additionally, some autoimmune diseases share alterations in metabolic pathways, key metabolites or metabolic byproducts such as reactive oxygen species. Other examples for metabolic changes in autoimmune settings include modifications in amino acid and cholesterol levels or glucose catabolism. Thus, the emerging field of immunometabolism may hold the potential to discover new therapeutic targets. Here, we discuss recent findings describing metabolic changes in autoimmune arthritis, multiple sclerosis as well as type 1 diabetes, focusing on pathophysiological aspects.

  5. Autoimmune autonomic disorders.

    Science.gov (United States)

    Mckeon, Andrew; Benarroch, Eduardo E

    2016-01-01

    Autoimmune autonomic disorders occur because of an immune response directed against sympathetic, parasympathetic, and enteric ganglia, autonomic nerves, or central autonomic pathways. In general, peripheral autoimmune disorders manifest with either generalized or restricted autonomic failure, whereas central autoimmune disorders manifest primarily with autonomic hyperactivity. Some autonomic disorders are generalized, and others are limited in their anatomic extent, e.g., isolated gastrointestinal dysmotility. Historically, these disorders were poorly recognized, and thought to be neurodegenerative. Over the last 20 years a number of autoantibody biomarkers have been discovered that have enabled the identification of certain patients as having an autoimmune basis for either autonomic failure or hyperactivity. Peripheral autoimmune autonomic disorders include autoimmune autonomic ganglionopathy (AAG), paraneoplastic autonomic neuropathy, and acute autonomic and sensory neuropathy. AAG manifests with acute or subacute onset of generalized or selective autonomic failure. Antibody targeting the α3 subunit of the ganglionic-type nicotinic acetylcholine receptor (α3gAChR) is detected in approximately 50% of cases of AAG. Some other disorders are characterized immunologically by paraneoplastic antibodies with a high positive predictive value for cancer, such as antineuronal nuclear antibody, type 1 (ANNA-1: anti-Hu); others still are seronegative. Recognition of an autoimmune basis for autonomic disorders is important, as their manifestations are disabling, may reflect an underlying neoplasm, and have the potential to improve with a combination of symptomatic and immune therapies.

  6. Autoimmune-mediated peripheral neuropathies and autoimmune pain.

    Science.gov (United States)

    Klein, Christopher J

    2016-01-01

    Peripheral neuropathies have diverse acquired and inherited causes. The autoimmune neuropathies represent an important category where treatment is often available. There are overlapping signs and symptoms between autoimmune neuropathies and other forms. Making a diagnosis can be challenging and first assisted by electrophysiologic and sometimes pathologic sampling, with autoimmune biomarkers providing increased assistance. Here we provide a review of the autoimmune and inflammatory neuropathies, their available biomarkers, and approaches to treatment. Also discussed is new evidence to support a mechanism of autoimmune pain.

  7. Autoimmunity in Immunodeficiency

    Science.gov (United States)

    Todoric, Krista; Koontz, Jessica B.; Mattox, Daniel; Tarrant, Teresa K.

    2013-01-01

    Primary immunodeficiencies (PID) comprise a diverse group of clinical disorders with varied genetic defects. Paradoxically, a substantial proportion of PID patients develop autoimmune phenomena in addition to having increased susceptibility to infections from their impaired immunity. Although much of our understanding comes from data gathered through experimental models, there are several well-characterized PID that have improved our knowledge of the pathways that drive autoimmunity. The goals of this review will be to discuss these immunodeficiencies and to review the literature with respect to the proposed mechanisms for autoimmunity within each put forth to date. PMID:23591608

  8. [Non-autoimmune thyroiditis].

    Science.gov (United States)

    Rizzo, Leonardo F L; Mana, Daniela L; Bruno, Oscar D

    2014-01-01

    The term thyroiditis comprises a group of thyroid diseases characterized by the presence of inflammation, including autoimmune and non-autoimmune entities. It may manifest as an acute illness with severe thyroid pain (subacute thyroiditis and infectious thyroiditis), and conditions in which the inflammation is not clinically evident evolving without pain and presenting primarily thyroid dysfunction and/or goiter (drug-induced thyroiditis and Riedel thyroiditis). The aim of this review is to provide an updated approach on non-autoimmune thyroiditis and its clinical, diagnostic and therapeutic aspects.

  9. Autoimmunity in visual loss.

    Science.gov (United States)

    Petzold, Axel; Wong, Sui; Plant, Gordon T

    2016-01-01

    There are a number of autoimmune disorders which can affect visual function. There are a very large number of mechanisms in the visual pathway which could potentially be the targets of autoimmune attack. In practice it is the retina and the anterior visual pathway (optic nerve and chiasm) that are recognised as being affected in autoimmune disorders. Multiple Sclerosis is one of the commonest causes of visual loss in young adults because of the frequency of attacks of optic neuritis in that condition, however the basis of the inflammation in Multiple Sclerosis and the confirmation of autoimmunity is lacking. The immune process is known to be highly unusual in that it is not systemic and confined to the CNS compartment. Previously an enigmatic partner to Multiple Sclerosis, Neuromyelitis Optica is now established to be autoimmune and two antibodies - to Aquaporin4 and to Myelin Oligodendrocyte Glycoprotein - have been implicated in the pathogenesis. The term Chronic Relapsing Inflammatory Optic Neuropathy is applied to those cases of optic neuritis which require long term immunosuppression and hence are presumed to be autoimmune but where no autoimmune pathogenesis has been confirmed. Optic neuritis occurring post-infection and post vaccination and conditions such as Systemic Lupus Erythematosus and various vasculitides may cause direct autoimmune attack to visual structures or indirect damage through occlusive vasculopathy. Chronic granulomatous disorders such as Sarcoidosis affect vision commonly by a variety of mechanisms, whether and how these are placed in the autoimmune panoply is unknown. As far as the retina is concerned Cancer Associated Retinopathy and Melanoma Associated Retinopathy are well characterised clinically but a candidate autoantibody (recoverin) is only described in the former disorder. Other, usually monophasic, focal retinal inflammatory disorders (Idiopathic Big Blind Spot Syndrome, Acute Zonal Occult Outer Retinopathy and Acute Macular

  10. Autoimmune liver diseases

    Institute of Scientific and Technical Information of China (English)

    Pietro Invernizzi; Ian R Mackay

    2008-01-01

    The liver was one of the earliest recognized sites among autoimmune diseases yet autoimmune hepatitis,primary biliary cirrhosis,primary sclerosing cholangitis,and their overlap forms,are still problematic in diagnosis and causation.The contributions herein comprise 'pairs of articles' on clinical characteristics,and concepts of etiopathogenesis,for each of the above diseases,together with childhood autoimmune liver disease,overlaps,interpretations of diagnostic serology,and liver transplantation.This issue is timely,since we are witnessing an ever increasing applicability of immunology to a wide variety of chronic diseases,hepatic and non-hepatic,in both developed and developing countries.The 11 invited expert review articles capture the changing features over recent years of the autoimmune liver diseases,the underlying immunomolecular mechanisms of development,the potent albeit still unexplained genetic influences,the expanding repertoire of immunoserological diagnostic markers,and the increasingly effective therapeutic possibilities.

  11. Autoimmunity against laminins.

    Science.gov (United States)

    Florea, Florina; Koch, Manuel; Hashimoto, Takashi; Sitaru, Cassian

    2016-09-01

    Laminins are ubiquitous constituents of the basement membranes with major architectural and functional role as supported by the fact that absence or mutations of laminins lead to either lethal or severely impairing phenotypes. Besides genetic defects, laminins are involved in a wide range of human diseases including cancer, infections, and inflammatory diseases, as well as autoimmune disorders. A growing body of evidence implicates several laminin chains as autoantigens in blistering skin diseases, collagenoses, vasculitis, or post-infectious autoimmunity. The current paper reviews the existing knowledge on autoimmunity against laminins referring to both experimental and clinical data, and on therapeutic implications of anti-laminin antibodies. Further investigation of relevant laminin epitopes in pathogenic autoimmunity would facilitate the development of appropriate diagnostic tools for thorough characterization of patients' antibody specificities and should decisively contribute to designing more specific therapeutic interventions.

  12. Miastenia gravis congênita e oftalmoplegia externa

    Directory of Open Access Journals (Sweden)

    Moreira Ana Tereza Ramos

    2001-01-01

    Full Text Available A miastenia gravis é uma doença crônica, caracterizada por fatigabilidade anormal de músculos estriados, podendo acometer grupos musculares isolados ou tornar-se generalizada. Os autores descrevem um caso de miastenia gravis congênita generalizada e oftalmoplegia parcial em um paciente de 10 anos de idade, portador de sintomas sistêmicos motores e de ausência na adução, abdução e elevação em ambos os olhos e com ptose palpebral bilateral, sendo reduzida à função de infraversão. O paciente foi diagnosticado aos dois anos e seis meses, sendo iniciado tratamento com piridostigmina em doses subterapêuticas, sem alteração importante no quadro clínico. Aos quatro anos de idade, procurou esta instituição, sendo ajustada à dose da medicação, seguindo-se melhora significativa dos sintomas motores sistêmicos, melhora parcial da ptose palpebral e sem alteração na oftalmoplegia externa.

  13. Autoimmunity in 2014.

    Science.gov (United States)

    Selmi, Carlo

    2015-10-01

    Our PubMed search for peer-reviewed articles published in the 2014 solar year retrieved a significantly higher number of hits compared to 2013 with a net 28 % increase. Importantly, full articles related to autoimmunity constitute approximately 5 % of immunology articles. We confirm that our understanding of autoimmunity is becoming a translational paradigm with pathogenetic elements rapidly followed by new treatment options. Furthermore, numerous clinical and pathogenetic elements and features are shared among autoimmune diseases, and this is well illustrated in the recent literature. More specifically, the past year witnessed critical revisions of our understanding and management of antiphospholipid syndrome with new exciting data on the pathogenicity of the serum anti-beta2 glycoprotein autoantibody, a better understanding of the current and new treatments for rheumatoid arthritis, and new position papers on important clinical questions such as vaccinations in patients with autoimmune disease, comorbidities, or new classification criteria. Furthermore, data confirming the important connections between innate immunity and autoimmunity via toll-like receptors or the critical role of T regulatory cells in tolerance breakdown and autoimmunity perpetuation were also reported. Lastly, genetic and epigenetic data were provided to confirm that the mosaic of autoimmunity warrants a susceptible individual background which may be geographically determined and contribute to the geoepidemiology of diseases. The 2014 literature in the autoimmunity world should be cumulatively regarded as part of an annus mirabilis in which, on a different level, the 2014 Annual Meeting of the American College of Rheumatology in Boston was attended by over 16,000 participants with over selected 3000 abstracts.

  14. Vaccines, adjuvants and autoimmunity.

    Science.gov (United States)

    Guimarães, Luísa Eça; Baker, Britain; Perricone, Carlo; Shoenfeld, Yehuda

    2015-10-01

    Vaccines and autoimmunity are linked fields. Vaccine efficacy is based on whether host immune response against an antigen can elicit a memory T-cell response over time. Although the described side effects thus far have been mostly transient and acute, vaccines are able to elicit the immune system towards an autoimmune reaction. The diagnosis of a definite autoimmune disease and the occurrence of fatal outcome post-vaccination have been less frequently reported. Since vaccines are given to previously healthy hosts, who may have never developed the disease had they not been immunized, adverse events should be carefully accessed and evaluated even if they represent a limited number of occurrences. In this review of the literature, there is evidence of vaccine-induced autoimmunity and adjuvant-induced autoimmunity in both experimental models as well as human patients. Adjuvants and infectious agents may exert their immune-enhancing effects through various functional activities, encompassed by the adjuvant effect. These mechanisms are shared by different conditions triggered by adjuvants leading to the autoimmune/inflammatory syndrome induced by adjuvants (ASIA syndrome). In conclusion, there are several case reports of autoimmune diseases following vaccines, however, due to the limited number of cases, the different classifications of symptoms and the long latency period of the diseases, every attempt for an epidemiological study has so far failed to deliver a connection. Despite this, efforts to unveil the connection between the triggering of the immune system by adjuvants and the development of autoimmune conditions should be undertaken. Vaccinomics is a field that may bring to light novel customized, personalized treatment approaches in the future.

  15. Lithium associated autoimmune thyroiditis.

    OpenAIRE

    Shimizu, M; Hirokawa, M.; T. Manabe; Shimozuma, K; Sonoo, H; Harada, T.

    1997-01-01

    A case of autoimmune thyroiditis after long term treatment with lithium is described in a 29 year old Japanese woman with manic depression. Positive serum antithyroglobulin and antimicrosomal antibodies, diffuse goitre, and microscopic chronic thyroiditis, as well as the clinical history of long term lithium treatment were suggestive of lithium associated autoimmune thyroiditis. Microscopically, there was a mild degree of interstitial fibrosis and a moderate degree of lymphocytic infiltration...

  16. The epigenetics of autoimmunity

    Science.gov (United States)

    Meda, Francesca; Folci, Marco; Baccarelli, Andrea; Selmi, Carlo

    2011-01-01

    The etiology of autoimmune diseases remains largely unknown. Concordance rates in monozygotic twins are lower than 50% while genome-wide association studies propose numerous significant associations representing only a minority of patients. These lines of evidence strongly support other complementary mechanisms involved in the regulation of genes expression ultimately causing overt autoimmunity. Alterations in the post-translational modification of histones and DNA methylation are the two major epigenetic mechanisms that may potentially cause a breakdown of immune tolerance and the perpetuation of autoimmune diseases. In recent years, several studies both in clinical settings and experimental models proposed that the epigenome may hold the key to a better understanding of autoimmunity initiation and perpetuation. More specifically, data support the impact of epigenetic changes in systemic lupus erythematosus, rheumatoid arthritis, multiple sclerosis and other autoimmune diseases, in some cases based on mechanistical observations. We herein discuss what we currently know and what we expect will come in the next future. Ultimately, epigenetic treatments already being used in oncology may soon prove beneficial also in autoimmune diseases. PMID:21278766

  17. Autoimmunity in 2015.

    Science.gov (United States)

    Selmi, Carlo

    2016-08-01

    Compared to the clear trend observed in previous years, the number of peer-reviewed articles published during 2015 and retrieved using the "autoimmunity" key word declined by 4 %, while remaining 5 % of immunology articles. On the other hand, a more detailed analysis of the published articles in leading immunology and autoimmunity journals revealed exciting scenarios, with fascinating lines of evidence being supported by convincing data and likely followed by rapid translational or clinical developments. As examples, the study of the microbiome, the development of new serum or other tissue biomarkers, and a more solid understanding of disease pathogenesis and tolerance breakdown mechanisms have been central issues in the past year. Furthermore and similar to the oncology field, progress in the understanding of single autoimmune condition is becoming most specific with psoriatic and rheumatoid arthritis being ideal paradigms with treatment options diverging after decades of common therapies, as illustrated by IL17-targeting approaches. The ultimate result of these advances is towards personalized medicine with an ideal approach being tailored on a single patient, based on a finely tuned definition of the immunogenetics, epigenetics, microbiome, and biomarkers. Finally, experimental reports suggest that cancer-associated immune mechanisms or the role of T and B cell subpopulations should be better understood in autoimmune diseases. While we hailed the 2014 literature in the autoimmunity world as part of an annus mirabilis, we should not be mistaken in the strong stimulus of research in autoimmunity represented by the 2015 articles that will be summarized in this article.

  18. Vaccines and autoimmunity.

    Science.gov (United States)

    De Martino, M; Chiappini, E; Galli, L

    2013-01-01

    Vaccines have eradicated or controlled many infectious diseases, saving each year millions of lives and quality of life of many other millions of people. In spite of the success of vaccines over the last two centuries, parents (and also some health care workers) gloss over the devastating consequences of diseases, which are now avoided thanks to vaccines, and direct their attention to possible negative effects of immunization. Three immunological objections are raised: vaccines cause antigenic overload, natural immunity is safer and better than vaccine-induced immunity, and vaccines induce autoimmunity. The last point is examined in this review. Theoretically, vaccines could trigger autoimmunity by means of cytokine production, anti-idiotypic network, expression of human histocompatibility leukocyte antigens, modification of surface antigens and induction of novel antigens, molecular mimicry, bystander activation, epitope spreading, and polyclonal activation of B cells. There is strong evidence that none of these mechanisms is really effective in causing autoimmune diseases. Vaccines are not a source of autoimmune diseases. By contrast, absolute evidence exists that infectious agents can trigger autoimmune mechanisms and that they do cause autoimmune diseases.

  19. Autoimmunity in 2013.

    Science.gov (United States)

    Selmi, Carlo

    2014-08-01

    The peer-reviewed publications in the field of autoimmunity published in 2013 represented a significant proportion of immunology articles and grew since the previous year to indicate that more immune-mediated phenomena may recognize an autoimmune mechanism and illustrated by osteoarthritis and atherosclerosis. As a result, our understanding of the mechanisms of autoimmunity is becoming the paradigm for translational research in which the progress in disease pathogenesis for both tolerance breakdown and inflammation perpetuation is rapidly followed by new treatment approaches and clinical management changes. The similarities across the autoimmune disease spectrum outnumber differences, particularly when treatments are compared. Indeed, the therapeutics of autoimmune diseases are based on a growing armamentarium that currently includes monoclonal antibodies and small molecules which act by targeting molecular markers or intracellular mediators with high specificity. Among the over 100 conditions considered as autoimmune, the common grounds are well illustrated by the data reported for systemic lupus erythematosus and rheumatoid arthritis or by the plethora of studies on Th17 cells and biomarkers, particularly serum autoantibodies. Further, we are particularly intrigued by studies on the genomics, epigenetics, and microRNA at different stages of disease development or on the safe and effective use of abatacept acting on the costimulation of T and B cells in rheumatoid arthritis. We are convinced that the data published in 2013 represent a promising background for future developments that will exponentially impact the work of laboratory and clinical scientists over the next years.

  20. Postoperative Survival for Patients with Thymoma Complicating Myasthenia Gravis
- Preliminary Retrospective Results of the ChART Database

    Directory of Open Access Journals (Sweden)

    Fangrui WANG

    2016-07-01

    Full Text Available Background and objective It is so far not clear that how myasthenia gravis (MG affected the prognosis of thymoma patients. The aim of this assay is to compare the postoperative survival between patients with thymoma only and those with both thymoma and MG. Methods The Chinese Alliance for Research in Thymomas (ChART registry recruited patients with thymoma from 18 centers over the country on an intention to treat basis from 1992 to 2012. Two groups were formed according to whether the patient complicated MG. Demographic and clinical data were reviewed, Patients were followed and their survival status were analyzed. Results There were 1,850 patients included in this study, including 421 with and 1,429 without MG. Complete thymectomy were done in 91.2% patients in MG group and 71.0% in non-MG group (P<0.05. There were more percentage of patients with the histology of thymoma AB, B1, or B2 (P<0.05 in MG group, and more percentage of patients with MG were in Masaoka stage I and II. The 5 year and 10 year OS rates were both higher in MG group (93% vs 88%; 83% vs 81%, P=0.034 respectively. The survival rate was significantly higher in patients with MG when the Masaoka staging was III/IV (P=0.003. Among patients with advanced stage thymoma (stage III, IVa, IVb, the constituent ratios of III, IVa, IVb were similar between MG and Non-MG group. Histologically, however, there were significantly more proportion of AB/B1/B2/B3 in the MG group while there were more C in the non-MG group (P=0.000. Univariate analyses for all patients showed that MG, WHO classification, Masaoka stage, surgical approach, chemotherapy and radiotherapy and resectability were significant factors, and multivariate analysis showed WHO Classification, Masaoka stage, and resectability were strong independent prognostic indicators. Conclusion Although MG is not an independent prognostic factor, the survival of patients with thymoma was superior when MG was present, especially in late