Myasthenia Gravis: A disorder of neuromuscular transmission characterized by weakness of cranial and skeletal muscles. Autoantibodies directed against acetylcholine receptors damage the motor endplate portion of the NEUROMUSCULAR JUNCTION, impairing the transmission of impulses to skeletal muscles. Clinical manifestations may include diplopia, ptosis, and weakness of facial, bulbar, respiratory, and proximal limb muscles. The disease may remain limited to the ocular muscles. THYMOMA is commonly associated with this condition. (Adams et al., Principles of Neurology, 6th ed, p1459)Myasthenia Gravis, Autoimmune, Experimental: Any autoimmune animal disease model used in the study of MYASTHENIA GRAVIS. Injection with purified neuromuscular junction acetylcholine receptor (AChR) (see RECEPTORS, CHOLINERGIC) components results in a myasthenic syndrome that has acute and chronic phases. The motor endplate pathology, loss of acetylcholine receptors, presence of circulating anti-AChR antibodies, and electrophysiologic changes make this condition virtually identical to human myasthenia gravis. Passive transfer of AChR antibodies or lymphocytes from afflicted animals to normals induces passive transfer experimental autoimmune myasthenia gravis. (From Joynt, Clinical Neurology, 1997, Ch 54, p3)Thymectomy: Surgical removal of the thymus gland. (Dorland, 28th ed)Receptors, Cholinergic: Cell surface proteins that bind acetylcholine with high affinity and trigger intracellular changes influencing the behavior of cells. Cholinergic receptors are divided into two major classes, muscarinic and nicotinic, based originally on their affinity for nicotine and muscarine. Each group is further subdivided based on pharmacology, location, mode of action, and/or molecular biology.Thymoma: A neoplasm originating from thymic tissue, usually benign, and frequently encapsulated. Although it is occasionally invasive, metastases are extremely rare. It consists of any type of thymic epithelial cell as well as lymphocytes that are usually abundant. Malignant lymphomas that involve the thymus, e.g., lymphosarcoma, Hodgkin's disease (previously termed granulomatous thymoma), should not be regarded as thymoma. (From Stedman, 25th ed)Pyridostigmine Bromide: A cholinesterase inhibitor with a slightly longer duration of action than NEOSTIGMINE. It is used in the treatment of myasthenia gravis and to reverse the actions of muscle relaxants.Thymus Neoplasms: Tumors or cancer of the THYMUS GLAND.Thymus Hyperplasia: Enlargement of the thymus. A condition described in the late 1940's and 1950's as pathological thymic hypertrophy was status thymolymphaticus and was treated with radiotherapy. Unnecessary removal of the thymus was also practiced. It later became apparent that the thymus undergoes normal physiological hypertrophy, reaching a maximum at puberty and involuting thereafter. The concept of status thymolymphaticus has been abandoned. Thymus hyperplasia is present in two thirds of all patients with myasthenia gravis. (From Segen, Dictionary of Modern Medicine, 1992; Cecil Textbook of Medicine, 19th ed, p1486)Myasthenia Gravis, Neonatal: A disorder of neuromuscular transmission that occurs in a minority of newborns born to women with myasthenia gravis. Clinical features are usually present at birth or develop in the first 3 days of life and consist of hypotonia and impaired respiratory, suck, and swallowing abilities. This condition is associated with the passive transfer of acetylcholine receptor antibodies through the placenta. In the majority of infants the myasthenic weakness resolves (i.e., transient neonatal myasthenia gravis) although this disorder may rarely continue beyond the neonatal period (i.e., persistent neonatal myasthenia gravis). (From Menkes, Textbook of Child Neurology, 5th ed, p823; Neurology 1997 Jan;48(1):50-4)Autoantibodies: Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them.Blepharoptosis: Drooping of the upper lid due to deficient development or paralysis of the levator palpebrae muscle.Edrophonium: A rapid-onset, short-acting cholinesterase inhibitor used in cardiac arrhythmias and in the diagnosis of myasthenia gravis. It has also been used as an antidote to curare principles.Cholinesterase Inhibitors: Drugs that inhibit cholinesterases. The neurotransmitter ACETYLCHOLINE is rapidly hydrolyzed, and thereby inactivated, by cholinesterases. When cholinesterases are inhibited, the action of endogenously released acetylcholine at cholinergic synapses is potentiated. Cholinesterase inhibitors are widely used clinically for their potentiation of cholinergic inputs to the gastrointestinal tract and urinary bladder, the eye, and skeletal muscles; they are also used for their effects on the heart and the central nervous system.Plasmapheresis: Procedure whereby plasma is separated and extracted from anticoagulated whole blood and the red cells retransfused to the donor. Plasmapheresis is also employed for therapeutic use.Neostigmine: A cholinesterase inhibitor used in the treatment of myasthenia gravis and to reverse the effects of muscle relaxants such as gallamine and tubocurarine. Neostigmine, unlike PHYSOSTIGMINE, does not cross the blood-brain barrier.Torpedo: A genus of the Torpedinidae family consisting of several species. Members of this family have powerful electric organs and are commonly called electric rays.Neuromuscular Junction: The synapse between a neuron and a muscle.Diplopia: A visual symptom in which a single object is perceived by the visual cortex as two objects rather than one. Disorders associated with this condition include REFRACTIVE ERRORS; STRABISMUS; OCULOMOTOR NERVE DISEASES; TROCHLEAR NERVE DISEASES; ABDUCENS NERVE DISEASES; and diseases of the BRAIN STEM and OCCIPITAL LOBE.Thymus Gland: A single, unpaired primary lymphoid organ situated in the MEDIASTINUM, extending superiorly into the neck to the lower edge of the THYROID GLAND and inferiorly to the fourth costal cartilage. It is necessary for normal development of immunologic function early in life. By puberty, it begins to involute and much of the tissue is replaced by fat.Rats, Inbred LewAutoimmune Diseases: Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides.Receptors, Nicotinic: One of the two major classes of cholinergic receptors. Nicotinic receptors were originally distinguished by their preference for NICOTINE over MUSCARINE. They are generally divided into muscle-type and neuronal-type (previously ganglionic) based on pharmacology, and subunit composition of the receptors.Ocular Motility Disorders: Disorders that feature impairment of eye movements as a primary manifestation of disease. These conditions may be divided into infranuclear, nuclear, and supranuclear disorders. Diseases of the eye muscles or oculomotor cranial nerves (III, IV, and VI) are considered infranuclear. Nuclear disorders are caused by disease of the oculomotor, trochlear, or abducens nuclei in the BRAIN STEM. Supranuclear disorders are produced by dysfunction of higher order sensory and motor systems that control eye movements, including neural networks in the CEREBRAL CORTEX; BASAL GANGLIA; CEREBELLUM; and BRAIN STEM. Ocular torticollis refers to a head tilt that is caused by an ocular misalignment. Opsoclonus refers to rapid, conjugate oscillations of the eyes in multiple directions, which may occur as a parainfectious or paraneoplastic condition (e.g., OPSOCLONUS-MYOCLONUS SYNDROME). (Adams et al., Principles of Neurology, 6th ed, p240)Eye Manifestations: Ocular disorders attendant upon non-ocular disease or injury.Ophthalmoplegia: Paralysis of one or more of the ocular muscles due to disorders of the eye muscles, neuromuscular junction, supporting soft tissue, tendons, or innervation to the muscles.Electromyography: Recording of the changes in electric potential of muscle by means of surface or needle electrodes.Lambert-Eaton Myasthenic Syndrome: An autoimmune disease characterized by weakness and fatigability of proximal muscles, particularly of the pelvic girdle, lower extremities, trunk, and shoulder girdle. There is relative sparing of extraocular and bulbar muscles. CARCINOMA, SMALL CELL of the lung is a frequently associated condition, although other malignancies and autoimmune diseases may be associated. Muscular weakness results from impaired impulse transmission at the NEUROMUSCULAR JUNCTION. Presynaptic calcium channel dysfunction leads to a reduced amount of acetylcholine being released in response to stimulation of the nerve. (From Adams et al., Principles of Neurology, 6th ed, pp 1471)Motor Endplate: The specialized postsynaptic region of a muscle cell. The motor endplate is immediately across the synaptic cleft from the presynaptic axon terminal. Among its anatomical specializations are junctional folds which harbor a high density of cholinergic receptors.Muscles: Contractile tissue that produces movement in animals.Bungarotoxins: Neurotoxic proteins from the venom of the banded or Formosan krait (Bungarus multicinctus, an elapid snake). alpha-Bungarotoxin blocks nicotinic acetylcholine receptors and has been used to isolate and study them; beta- and gamma-bungarotoxins act presynaptically causing acetylcholine release and depletion. Both alpha and beta forms have been characterized, the alpha being similar to the large, long or Type II neurotoxins from other elapid venoms.Antibodies: Immunoglobulin molecules having a specific amino acid sequence by virtue of which they interact only with the ANTIGEN (or a very similar shape) that induced their synthesis in cells of the lymphoid series (especially PLASMA CELLS).Azathioprine: An immunosuppressive agent used in combination with cyclophosphamide and hydroxychloroquine in the treatment of rheumatoid arthritis. According to the Fourth Annual Report on Carcinogens (NTP 85-002, 1985), this substance has been listed as a known carcinogen. (Merck Index, 11th ed)Muscle Weakness: A vague complaint of debility, fatigue, or exhaustion attributable to weakness of various muscles. The weakness can be characterized as subacute or chronic, often progressive, and is a manifestation of many muscle and neuromuscular diseases. (From Wyngaarden et al., Cecil Textbook of Medicine, 19th ed, p2251)Electrophorus: A genus of fish, in the family GYMNOTIFORMES, capable of producing an electric shock that immobilizes fish and other prey. The species Electrophorus electricus is also known as the electric eel, though it is not a true eel.Neuromuscular Junction Diseases: Conditions characterized by impaired transmission of impulses at the NEUROMUSCULAR JUNCTION. This may result from disorders that affect receptor function, pre- or postsynaptic membrane function, or ACETYLCHOLINESTERASE activity. The majority of diseases in this category are associated with autoimmune, toxic, or inherited conditions.Oculomotor Muscles: The muscles that move the eye. Included in this group are the medial rectus, lateral rectus, superior rectus, inferior rectus, inferior oblique, superior oblique, musculus orbitalis, and levator palpebrae superioris.Plasma Exchange: Removal of plasma and replacement with various fluids, e.g., fresh frozen plasma, plasma protein fractions (PPF), albumin preparations, dextran solutions, saline. Used in treatment of autoimmune diseases, immune complex diseases, diseases of excess plasma factors, and other conditions.Immunoglobulin G: The major immunoglobulin isotype class in normal human serum. There are several isotype subclasses of IgG, for example, IgG1, IgG2A, and IgG2B.Multiple Sclerosis: An autoimmune disorder mainly affecting young adults and characterized by destruction of myelin in the central nervous system. Pathologic findings include multiple sharply demarcated areas of demyelination throughout the white matter of the central nervous system. Clinical manifestations include visual loss, extra-ocular movement disorders, paresthesias, loss of sensation, weakness, dysarthria, spasticity, ataxia, and bladder dysfunction. The usual pattern is one of recurrent attacks followed by partial recovery (see MULTIPLE SCLEROSIS, RELAPSING-REMITTING), but acute fulminating and chronic progressive forms (see MULTIPLE SCLEROSIS, CHRONIC PROGRESSIVE) also occur. (Adams et al., Principles of Neurology, 6th ed, p903)Antibodies, Antinuclear: Autoantibodies directed against various nuclear antigens including DNA, RNA, histones, acidic nuclear proteins, or complexes of these molecular elements. Antinuclear antibodies are found in systemic autoimmune diseases including systemic lupus erythematosus, Sjogren's syndrome, scleroderma, polymyositis, and mixed connective tissue disease.Muscle, Skeletal: A subtype of striated muscle, attached by TENDONS to the SKELETON. Skeletal muscles are innervated and their movement can be consciously controlled. They are also called voluntary muscles.Ventilators, Mechanical: Mechanical devices used to produce or assist pulmonary ventilation.Neurology: A medical specialty concerned with the study of the structures, functions, and diseases of the nervous system.Lupus Erythematosus, Systemic: A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.Bed Conversion: The reallocation of beds from one type of care service to another, as in converting acute care beds to long term care beds.Prednisone: A synthetic anti-inflammatory glucocorticoid derived from CORTISONE. It is biologically inert and converted to PREDNISOLONE in the liver.Mycophenolic Acid: An antibiotic substance derived from Penicillium stoloniferum, and related species. It blocks de novo biosynthesis of purine nucleotides by inhibition of the enzyme inosine monophosphate dehydrogenase. Mycophenolic acid is important because of its selective effects on the immune system. It prevents the proliferation of T-cells, lymphocytes, and the formation of antibodies from B-cells. It also may inhibit recruitment of leukocytes to inflammatory sites. (From Gilman et al., Goodman and Gilman's The Pharmacological Basis of Therapeutics, 9th ed, p1301)Immunosuppressive Agents: Agents that suppress immune function by one of several mechanisms of action. Classical cytotoxic immunosuppressants act by inhibiting DNA synthesis. Others may act through activation of T-CELLS or by inhibiting the activation of HELPER CELLS. While immunosuppression has been brought about in the past primarily to prevent rejection of transplanted organs, new applications involving mediation of the effects of INTERLEUKINS and other CYTOKINES are emerging.Treatment Outcome: Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.Cardiovascular Deconditioning: A change in cardiovascular function resulting in a reduction in BLOOD VOLUME, and reflex DIURESIS. It occurs frequently after actual or simulated WEIGHTLESSNESS.Physical Fitness: The ability to carry out daily tasks and perform physical activities in a highly functional state, often as a result of physical conditioning.
TE671 cell-based ELISA for anti-acetylcholine receptor antibody determination in myasthenia gravis. (1/830)
BACKGROUND: Acetylcholine receptor (AChR) from human muscles is the antigen used currently in radioimmunoprecipitation assays (RIPAs) for the determination of anti-AChR antibodies in the diagnosis of myasthenia gravis (MG). Our aim was to develop and validate an ELISA using TE671 cells as the source of AChR. METHODS: After TE671 cell homogenization, the crude AChR extract was used for plate coating. Anti-AChR antibodies were determined in 207 MG patients and in 77 controls. RESULTS: The mean intra- and interassay CVs (for two samples with different anti-AChR antibody concentrations) were 9.7% and 15.7%, respectively. Test sensitivity and specificity, for generalized MG, were 79.5% (95% confidence interval, 72.8-85.0%) and 96.1% (89.0-99.1%). The detection limit was 2 nmol/L. Anti-AChR antibody concentrations from 53 MG patients, as tested with our ELISA, showed good agreement with an RIPA with a mean difference (SD) of 1.0 (5.6) nmol/L. CONCLUSION: Our ELISA is a simple screening test for the diagnosis of MG and enables rapid and inexpensive patient follow-up. (+info)Myasthenia gravis and polymyositis as manifestations of chronic graft-versus-host-disease. (2/830)
Myasthenia gravis and polymyositis are each a rare manifestation of immune dysregulation in chronic graft-versus-host disease (cGVHD). We report a 4-year-old boy with idiopathic acquired aplastic anemia who developed myasthenia gravis 22 months and polymyositis 69 months after an allogeneic BMT (5/6 matched, MLC-nonreactive). The occurrence of both syndromes in one patient is unique. Autoimmune dysfunction may be associated with the development of cGVHD as demonstrated by the high incidence of prior aplastic anemia in BMT patients presenting with myasthenia gravis and polymyositis. Recognition of these neurologic manifestations is important in the diagnosis and treatment of cGVHD. (+info)Experimental autoimmune myasthenia gravis and CD5+ B-lymphocyte expression. (3/830)
Myasthenia gravis is one of the typical organ specific autoimmune disease and the CD5+ B-lymphocytes are known to be associated with the secretion of autoimmune antibodies. The authors performed the study to establish an animal model of experimental autoimmune myasthenia gravis (EAMG) by immunizing the nicotinic acetylcholine receptor (AChR) and to understand CD5+ B-lymphocyte changes in peripheral blood of EAMGs. Lewis rats weighing 150-200 g were injected subcutaneously three times with 50 microg AChR purified from the electric organ of Torpedo marmorata and Freund's adjuvant. The EAMG induction was assessed by evaluating clinical manifestations. The CD5+ B-lymphocyte was double stained using monoclonal PE conjugated anti-CD5+ and FITC conjugated anti-rat CD45R antibodies and calculated using a fluorescence-activated cell sorter (FACS). In three out of ten Lewis rats injected with purified AChR, the EAMG models were established. The animals showed definite clinical weakness responded to neostigmine; they had difficulty in climbing the slope, or easily fell down from a vertical cage. The range of CD5+ B-lymphocytes of peripheral blood in the EAMG models was 10.2%-17.5%, which was higher than in controls. In conclusion, the EAMG models were successfully established and the CD5+ B-lymphocyte expression in peripheral blood increased in EAMGs. This provided indirect evidence of the autoimmune pathomechanism of human myasthenia gravis. (+info)Congenital myasthenia gravis: clinical and HLA studies in two brothers. (4/830)
Two brothers with congenital myasthenia gravis are described. In both, ptosis and ophthalmoplegia responded poorly to oral anticholinesterase therapy and to thymectomy. The brothers had two different HLA haplotypes and neither had the HLA-A1-B8-DW3 haplotypes which are commonly associated with myathenia gravis in adult-onset cases. (+info)The value of thymectomy in myasthenia gravis: a computer-assisted matched study. (5/830)
In the absence of a prospective randomized study of patients treated conservatively or with thymectomy, a computer-assisted retrospective matches study was devised. Of 563 patients treated for myasthenia gravis without thymoma up to 1965, 104 had thymectomy. With computer assistance, each surgical patient was matched with a medical patient on the basis of age, sex, and severity and duration of disease. On this basis 80 of the 104 surgical patients could be matched satisfactorily. There were 16 males and 64 females in each of the matched surgically treated and medical control groups. A complete remission was experienced by 27 of the 78 patients in the surgical group as compared to 6 of the medical group. Improvement was noted by 26 of 78 surgically treated patients and 13 of 78 receiving medical treatment. Survival for patients having thymectomy. Thirty-four patients in the medical group had died as compared to 11 in the surgical group. Comparison of survival in relation to sex, duration of symptoms, or age (less than 30 or less than 30 years) did not show a significant difference. Until more effective treatment is available for myasthenia gravis, thymectomy deserves consideration for both sexes, and with increased age or long duration of symptoms. (+info)Mice with IFN-gamma receptor deficiency are less susceptible to experimental autoimmune myasthenia gravis. (6/830)
IFN-gamma can either adversely or beneficially affect certain experimental autoimmune diseases. To study the role of IFN-gamma in the autoantibody-mediated experimental autoimmune myasthenia gravis (EAMG), an animal model of myasthenia gravis in humans, IFN-gammaR-deficient (IFN-gammaR-/-) mutant C57BL/6 mice and congenic wild-type mice were immunized with Torpedo acetylcholine receptor (AChR) plus CFA. IFN-gammaR-/- mice exhibited significantly lower incidence and severity of muscle weakness, lower anti-AChR IgG Ab levels, and lower Ab affinity to AChR compared with wild-type mice. Passive transfer of serum from IFN-gammaR-/- mice induced less muscular weakness compared with serum from wild-type mice. In contrast, numbers of lymph node cells secreting IFN-gamma and of those expressing IFN-gamma mRNA were strongly augmented in the IFN-gammaR-/- mice, reflecting a failure of negative feedback circuits. Cytokine studies by in situ hybridization revealed lower levels of lymphoid cells expressing AChR-reactive IL-1beta and TNF-alpha mRNA in AChR + CFA-immunized IFN-gammaR-/- mice compared with wild-type mice. No differences were found for AChR-reactive cells expressing IL-4, IL-10, or TGF-beta mRNA. These results indicate that IFN-gamma promotes systemic humoral responses in EAMG by up-regulating the production and the affinity of anti-AChR autoantibodies, thereby contributing to susceptibility to EAMG in C57BL/6-type mice. (+info)Scanning a DRB3*0101 (DR52a)-restricted epitope cross-presented by DR3: overlapping natural and artificial determinants in the human acetylcholine receptor. (7/830)
A recurring epitope in the human acetylcholine receptor (AChR) alpha subunit (alpha146-160) is presented to specific T cells from myasthenia gravis patients by HLA-DRB3*0101-"DR52a"-or by DR4. Here we first map residues critical for DR52a in this epitope by serial Ala substitution. For two somewhat similar T cells, this confirms the recently deduced importance of hydrophobic "anchor" residues at peptide p1 and p9; also of Asp at p4, which complements this allele's distinctive Arg74 in DRbeta. Surprisingly, despite the 9 sequence differences in DRbeta between DR52a and DR3, merely reducing the bulk of the peptide's p1 anchor residue (Trp149-->Phe) allowed maximal cross-presentation to both T cells by DR3 (which has Val86 instead of Gly). The shared K71G73R74N77 motif in the alpha helices of DR52a and DR3 thus outweighs the five differences in the floor of the peptide-binding groove. A second issue is that T cells selected in vitro with synthetic AChR peptides rarely respond to longer Ag preparations, whereas those raised with recombinant subunits consistently recognize epitopes processed naturally even from whole AChR. Here we compared one T cell of each kind, which both respond to many overlapping alpha140-160 region peptides (in proliferation assays). Even though both use Vbeta2 to recognize peptides bound to the same HLA-DR52a in the same register, the peptide-selected line nevertheless proved to depend on a recurring synthetic artifact-a widely underestimated problem. Unlike these contaminant-responsive T cells, those that are truly specific for natural AChR epitopes appear less heterogeneous and therefore more suitable targets for selective immunotherapy. (+info)Detection of antibodies directed against the cytoplasmic region of the human acetylcholine receptor in sera from myasthenia gravis patients. (8/830)
The nicotinic acetylcholine receptor (AChR) is the autoantigen in the human autoimmune disease myasthenia gravis (MG). Anti-AChR antibodies in MG sera bind mainly to conformational epitopes, therefore the determination of their specificities requires the use of native AChR. Antibody competition studies suggest that most MG antibodies are directed against the extracellular part of the molecule, whereas antibodies directed against the cytoplasmic region of the AChR have not been detected. To determine whether even small quantities of such antibodies exist in MG sera, we performed competition experiments based on the inhibition by MG sera of the binding of MoAbs to the human AChR, rather than inhibition by MoAbs of the binding of MG sera performed earlier. When MoAbs directed against cytoplasmic epitopes on the alpha or beta subunits (alpha 373-380 and beta 354-360) were used as test MoAbs, 17% or 9% of MG sera inhibited the binding of the anti-alpha or anti-beta subunit MoAbs, respectively, by > or = 50%. Non-specific inhibition was excluded. These results suggest the presence, in several MG sera, of antibodies directed against cytoplasmic regions of the AChR; yet these antibodies seemed to represent a relatively small proportion of the total anti-AChR antibodies. The corresponding epitopes may be involved in the inducing mechanisms in certain MG cases, and knowledge of the presence of such antibodies may be useful in understanding the autoimmune mechanism involved in MG. (+info)SymptomsOcular myastheniaWeaknessMyasthenicDiagnosisPeople with myastheniaNeurologyFoundation of AmericaCases of myasthenia gravisCause of myasthenia gravisSymptom of myasthenia gravisCongenital myasthTreat myasthenia gravisThymus glandReceptorsDisordersRare autoimmuneTreatmentMusclesOccursNerveDiagnose myastheniaNeonatal myastheniaAffectsTest for myasthenia gravisTypes of myasthenia gravisCauses of Myasthenia GravisFatigue in myasthenia gravisSubjects with myasthenia gravisTreatments for myasthenia gravisNeuromuscular diseasesAntibodies against the acetylcholine receptorMysathenia gravis
- Here's what you should know about myasthenia gravis symptoms and diagnosis. (vetinfo.com)
- Your vet can treat the symptoms of myasthenia gravis by injecting your dog with edrophonium chloride and other drugs that increase levels of acetylcholine in the brain. (vetinfo.com)
- After the two-week neostigmine treatment phase, symptoms of myasthenia gravis (measured as improvement in at least one muscle function) improved in nine of the 10 participants. (cochrane.org)
- Researchers are examining the blood of patients from 22 centers specializing in the treatment of mysathenia gravis, the most common communication problem between brain and muscle, to determine what percentage of patients have one or both of the new antibodies and to characterize their clinical symptoms, said Dr. Lin Mei, chairman of the Department of Neuroscience and Regenerative Medicine at the Medical College of Georgia at Augusta University. (eurekalert.org)
- Antibodies to agrin and LRP4 have already been found in some patients, and LPR4 antibodies cause myasthenia gravis-like symptoms when injected into lab animals. (eurekalert.org)
- These patients may very well have slightly different symptoms because of where these antibodies are acting," said Dr. Michael H. Rivner, MCG neurologist specializing in neuromuscular disease who directs the Electrodiagnostic Medicine Laboratory at Augusta University Medical Center and follows about 250 patients with myasthenia gravis. (eurekalert.org)
- They went back to the laboratory and showed the antibodies to LRP4 cause myasthenia gravis symptoms in animal models. (eurekalert.org)
- The first noticeable symptoms of myasthenia gravis may be weakness of the eye muscles, difficulty in swallowing, or slurred speech. (medications.com)
- Thymectomy, the surgical removal of the thymus gland (which often is abnormal in myasthenia gravis patients), improves symptoms in certain patients and may cure some individuals, possibly by re-balancing the immune system. (medications.com)
- Moreover about 30 percent of people who have a thymoma, a thymus tumor, also suffer from myasthenia gravis and removal of the tumor alleviates the myasthenic symptoms. (enewspf.com)
- With Myasthenia gravis, it's common for returning symptoms to worsen with each flare up. (steadyhealth.com)
- Thymectomy, the surgical removal of the thymus gland (which often is abnormal in those with myasthenia gravis), improves symptoms in certain individuals Other therapies include plasmapheresis, a procedure in which abnormal antibodies are removed from the blood, and high-dose intravenous immune globulin, which temporarily modifies the immune system and provides the body with normal antibodies from donated blood. (abingtonneurology.com)
- The most common symptoms of myasthenia gravis are double vision (diplopia), drooping eyelids (ptosis), and muscle weakness that usually gets worse after exercise or at the end of the day and improves with rest. (safein4.com)
- Eventually, almost everyone with myasthenia gravis will experience eye symptoms. (safein4.com)
- Dr. Tahseen Mozaffar discusses the symptoms of generalized myasthenia gravis (gMG), diagnostic challenges, prognosis, and new therapeutic options for managing gMG. (healthprofessionalradio.com.au)
- Myasthenia gravis symptoms tend to progress over time if left untreated. (integratedneurologyservices.com)
- The main symptoms of myasthenia gravis are the weakness in the voluntary skeletal muscle. (integratedneurologyservices.com)
- Other common symptoms of myasthenia gravis are drooping eyelids, facial paralysis, problems walking up stairs, trouble talking, double vision, and a hoarse voice. (integratedneurologyservices.com)
- Symptoms of myasthenia gravis usually progress to maximum severity in three years, either stabilizing or improving after that. (integratedneurologyservices.com)
- Myasthenia Gravis can cause different symptoms amongst dogs. (simplewag.com)
- BACKGROUND: The principal symptoms of myasthenia gravis (MG), muscle weakness and fatigue due to impaired neuromuscular transmission, are caused by autoantibodies to the muscle nicotinic acetylcholine receptor (AChR). (unipi.it)
- The condition may be restricted to certain muscle groups, particularly those of the eyes (ocular myasthenia gravis), or may become more generalized (generalized myasthenia gravis), involving multiple muscle groups. (rarediseases.org)
- In some affected individuals, the disease process may be limited to certain eye muscles, which is often described as "ocular myasthenia gravis. (rarediseases.org)
- Autoantibodies (binding, blocking, and/or modulating) to postsynaptic AChRs are detectable in the serum of 90% of patients with generalized MG and in 55% to 70% of patients with ocular myasthenia. (labcorp.com)
- this is called ocular myasthenia gravis. (safein4.com)
- Myasthenia gravis affects the motor nervous system, causing muscle weakness. (vetinfo.com)
- When canine myasthenia gravis occurs, the number of nerve receptors for acetylcholine in your dog's brain diminishes, leaving your dog susceptible to muscle weakness. (vetinfo.com)
- Most dogs experience the muscle weakness of myasthenia gravis in the legs, especially the hind legs. (vetinfo.com)
- Your vet will test your dog for the muscle weakness associated with myasthenia gravis. (vetinfo.com)
- Myasthenia gravis is disease that causes weakness in the muscles under your control. (aarda.org)
- Myasthenia gravis is a neuromuscular disorder primarily characterized by muscle weakness and muscle fatigue. (rarediseases.org)
- Myasthenia gravis is an autoimmune disorder manifested by muscle weakness caused by the loss or dysfunction of acetylcholine receptors (AChR) of skeletal muscle. (labcorp.com)
- Myasthenia Gravis (MG) is a chronic, autoimmune disorder of neuromuscular transmission, resulting in muscle weakness. (yale.edu)
- The term "myasthenia" is Latin for muscle weakness, and "gravis" for grave or serious. (yale.edu)
- Myasthenia gravis (MG) with antibodies to muscle‐specific kinase (MuSK) is characterized by fluctuating fatigable weakness. (chemie.de)
- Myasthenia gravis is a chronic autoimmune neuromuscular disease characterized by varying degrees of weakness of the skeletal (voluntary) muscles of the body. (medications.com)
- The hallmark of myasthenia gravis is muscle weakness that increases during periods of activity and improves after periods of rest. (medications.com)
- Some case of myasthenia gravis may go into remission temporarily, and muscle weakness may disappear so that medications can be discontinued. (medications.com)
- In a few cases, the severe weakness of myasthenia gravis may cause respiratory failure, which requires immediate emergency medical care. (medications.com)
- In a global study of myasthenia gravis, an autoimmune disease that causes muscle weakness and fatigue, researchers found that surgical removal of an organ called the thymus reduced patients' weakness, and their need for immunosuppressive drugs. (enewspf.com)
- Affecting 36,000 to 60,000 patients in the United States, myasthenia gravis describes a group of chronic autoimmune neuromuscular diseases which causes varying degrees of muscle weakness and fatigue. (enewspf.com)
- Myasthenia gravis (MG) is defined as a disorder of the neuromuscular junction with fluctuating weakness of voluntary muscles associated with exhaustibility. (prolekare.cz)
- Though the reason behind the deficiency is different, either disease can lead to problems speaking , chewing , and swallowing (caused by weakness in Myasthenia gravis and nerve damage in multiple sclerosis). (steadyhealth.com)
- With treatment, most individuals with myasthenia can significantly improve their muscle weakness. (abingtonneurology.com)
- The name myasthenia gravis is of Latin and Greek origin, which means, "grave muscle weakness. (integratedneurologyservices.com)
- The majority of the cases are similar to the generalized, acquired myasthenia gravis, still there are some myasthenic familial congenital patients, too. (nih.gov)
- Myasthenia gravis and Lambert-Eaton myasthenic syndrome. (prolekare.cz)
- This is called a myasthenic crisis, and it's responsible for most of the small number of deaths due to myasthenia gravis. (safein4.com)
- If the doctor suspects myasthenia gravis, several diagnostic tests are available to confirm the diagnosis, including a special blood test that can detect the presence of immune molecules or acetylcholine receptor antibodies. (medications.com)
- We reviewed the evidence about the effect of aceytlcholinesterase inhibitor drugs in people with myasthenia gravis. (cochrane.org)
- In spite of the meaning of the name, people with myasthenia gravis have a normal life expectancy. (integratedneurologyservices.com)
- Our results support the idea that thymectomy is a valid treatment option for a major form of myasthenia gravis," said Gil Wolfe, M.D., Professor and Irvin and Rosemary Smith Chair of Neurology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, New York, and a leader of the study. (enewspf.com)
- The study draws from the county-based prospective myasthenia gravis register implemented by the Neurology Department at Hospital General de Vic in 1991. (elsevier.es)
- In our ongoing series this month, June being Generalized Myasthenia Gravis Awareness Month, we are joined this afternoon with Dr. Tahseen Mozaffar, Vice Chair of Education and Professor Department of Neurology at the University of California joining us today to further educate us about this rare neuromuscular disease. (healthprofessionalradio.com.au)
- At Integrated Neurology Services, we provide extensive treatment options to help patients with Myasthenia gravis get the care they need and deserve. (integratedneurologyservices.com)
- Until this study, the only evidence was from non-randomized studies that contained significant bias issues," said Wolfe, an expert on neuromuscular disorders with a special focus on MG, who was awarded the 2015 Doctor of the Year award from the Myasthenia Gravis Foundation of America. (buffalo.edu)
- Introduction: The aim of this study was to elucidate the effectiveness of oral prednisolone (PSL) according to dosing regimen in 472 patients with myasthenia gravis (MG). Methods: We compared the clinical characteristics and PSL treatment between 226 patients who achieved minimal manifestations (MM) or better and 246 patients who remained improved (I) or worsened, according to the MG Foundation of America postintervention status. (elsevier.com)
- In some severe cases of Myasthenia Gravis, dogs may be hospitalized until they are stabilized with Anti-acetylcholinesterase medications. (simplewag.com)
- Mei's lab found a link between agrin and LRP4 in 2008, reported LRP4 antibodies as a new cause of myasthenia gravis in 2013 and that agrin antibodies were associated with the disease the next year. (eurekalert.org)
- I shopped online for a cane to help me deal with the double vision and altered depth perception that I experience as a symptom of Myasthenia Gravis (MG). I was thrilled to find a metallic purple one. (jeriaielloart.com)
- Congenital myasthenia gravis is hereditary , and occurs when a dog is born without the appropriate number of acetylcholine receptors in his brain. (vetinfo.com)
- Acetylcholine receptor autoantibodies are not typically found in congenital myasthenia gravis. (labcorp.com)
- Congenital Myasthenia Gravis is incredibly rare, but it can happen. (simplewag.com)
- Puppies born with an ACh-receptor (acetylcholine receptors) deficiency are likely to have congenital Myasthenia Gravis. (simplewag.com)
- Congenital Myasthenia Gravis typically becomes apparent in puppies that are approximately six to eight weeks old. (simplewag.com)
- In severe cases of Congenital Myasthenia Gravis, puppies can experience paralysis or even death. (simplewag.com)
- The Dachshund is the most common dog breed that contracts Congenital Myasthenia Gravis. (simplewag.com)
- Perhaps what is most interesting is that Congenital Myasthenia Gravis in Dachshunds often resolves on its own! (simplewag.com)
- Other drugs used to treat myasthenia gravis include corticosteroids like prednisone , pyridostigmine and azathioprine. (vetinfo.com)
- Some drugs that are used to treat myasthenia gravis act on acetylcholinesterase to stop the breakdown of acetylcholine. (cochrane.org)
- A new study led by UB faculty says surgery to remove the thymus gland in patients with myasthenia gravis (MG), a relatively rare autoimmune disease that impacts neuromuscular function, provides long-term health and financial benefits. (wbfo.org)
- Wolfe said they've known for decades going back to early 1900's the thymus gland plays a role in myasthenia gravis generation. (wbfo.org)
- It's believed that these antibodies are formed in the thymus gland, which may have a tumor (in about fifteen percent of patients with Myasthenia gravis) or could be oversized (should be small in adults). (steadyhealth.com)
- The study to be published August 11 (available online on August 10 at 5 p.m.) found that surgical removal of the thymus gland from patients with myasthenia gravis, a rare autoimmune disease that affects neuromuscular function, provides significant benefit in patients who do not have a chest tumor. (buffalo.edu)
- Acquired canine myasthenia gravis is a type of autoimmune disorder that attacks and damages aceltycholine receptors. (vetinfo.com)
- Myasthenia gravis results from an abnormal immune reaction in which the body's natural immune defenses (i.e., antibodies) inappropriately attack and gradually destroy certain receptors in muscles that receive nerve impulses (antibody-mediated autoimmune response). (rarediseases.org)
- Clinical correlations of antibodies that bind, block, or modulate human acetylcholine receptors in myasthenia gravis. (labcorp.com)
- In myasthenia gravis, antibody-mediated blockade of acetylcholine receptors at the neuromuscular junction abolishes the naturally occurring 'safety factor' of synaptic transmission. (cochrane.org)
- In myasthenia gravis, antibodies produced by the body's own immune system block, alter, or destroy the receptors for acetylcholine. (medications.com)
- Myasthenia gravis (MG) is an autoimmune disease involving the thymus in which 85 percent of patients have antibodies to muscle acetylcholine receptors (AchR-Ab) that interfere with neuromuscular transmission. (nih.gov)
- Acquired Myasthenia Gravis in dogs is an immune-mediated disease , which is when the dog's own antibodies destroy ACh-receptors, causing an acetylcholine receptors deficiency. (simplewag.com)
- This is a study that the myasthenia gravis community has needed for a long time," said Robin Conwit, M.D., program director, at NIH's National Institute of Neurological Disorders and Stroke (NINDS). (enewspf.com)
- The increasing demand on diagnostic assays that are sensitive and specific for pathogenic antibodies, and the interest in identifying new antigens, prompted the development of cell-based assays for the detection of autoantibodies in myasthenia gravis and other autoimmune disorders. (ox.ac.uk)
- Myasthenia gravis is a rare autoimmune condition in which antibodies produced by the immune system attack the connection between nerves and muscles (the neuromuscular junction). (cochrane.org)
- If you have myasthenia gravis, it is important to follow your treatment plan. (aarda.org)
- AUGUSTA, Ga. - A study of patients from across the nation with myasthenia gravis is helping determine the incidence of two new antibodies believed to cause the disease, and whether these patients need different treatment strategies. (eurekalert.org)
- The purpose of the current study is to assess safety/tolerability and key pharmacodynamic (PD) effects that are considered to be associated with clinical benefit (reduction of total IgG and anti-AChR-IgG) in Myasthenia Gravis patients following treatment with RVT-1401 (also known as IMVT-1401) compared to placebo. (clinicaltrials.gov)
- With treatment, the outlook for most patients with myasthenia is bright: they can expect to lead normal or nearly normal lives. (medications.com)
- In the treatment of my myasthenia gravis, would mestinon timespan be as good for me as distigmine? (drugs.com)
- 7. Mertens HG, Balzereit F, Leipert M. The treatment of severe myasthenia gravis with immunosuppressive agents. (prolekare.cz)
- In fact, Myasthenia gravis actually seems quite similar to some aspects of multiple sclerosis and distinguishing one from the other is essential to prescribing the right treatment plan. (steadyhealth.com)
- In myasthenia gravis the response to acetylcholinesterase inhibitors in observational studies is probably so clear that a randomised controlled trial depriving participants in a placebo arm of treatment would be difficult to justify. (unboundmedicine.com)
- 1 . Mehndiratta MM, Pandey S, Kuntzer T. Acetylcholinesterase inhibitor treatment for myasthenia gravis. (unboundmedicine.com)
- Treatment of myasthenia gravis: a comparison of the natural course and current therapies. (unboundmedicine.com)
- Acetylcholinesterase inhibitor treatment for myasthenia gravis is a sample topic from the Evidence-Based Medicine Guidelines . (unboundmedicine.com)
- Evidence Central , evidence.unboundmedicine.com/evidence/view/EBMG/457462/all/Acetylcholinesterase_inhibitor_treatment_for_myasthenia_gravis. (unboundmedicine.com)
- Myasthenia gravis is caused by a defect in the transmission of nerve impulses to muscles. (medications.com)
- Myasthenia gravis is an autoimmune condition distinguished by the fast-weakening and fatigue of voluntary muscles and muscle groups . (steadyhealth.com)
- While MS attacks the central nervous system (CNS) and depletes the protective myelin coating and exposes the nerves to damage, Myasthenia gravis causes communication issues in the peripheral nervous system, with its effects seen at the connection sites on the muscles that receive signals. (steadyhealth.com)
- The muscles around the eyes are particularly likely to be affected by myasthenia gravis, and eye problems are the first sign of the disease in about 40% of cases. (safein4.com)
- Congenital myasthenia usually occurs in infants but may become evident in adulthood. (rarediseases.org)
- Similar to human autoimmune myasthenia gravis (MG), canine MG occurs spontaneously and is associated with autoantibodies against the nicotinic acetylcholine receptor (AChR). (elsevier.com)
- Congenital myasthenia is caused by genetic defects of muscle and nerve communication (neuromuscular transmission), and not an abnormal immune system. (rarediseases.org)
- Myasthenia gravis (MG) results from an immune-mediated disruption of communication between nerve and muscle - the neuromuscular junction. (buffalo.edu)
- Your vet will need a complete medical history and a thorough physical exam in order to diagnose myasthenia gravis. (vetinfo.com)
- Transitory (neonatal) myasthenia in new-born babies should be separated from the familial cases. (nih.gov)
- Canine Myasthenia Gravis commonly affects adult dogs. (simplewag.com)
- Your vet may choose to test for myasthenia gravis by injecting your dog with edrophonium chloride. (vetinfo.com)
- There are two common types of myasthenia gravis. (vetinfo.com)
- As we briefly mentioned above, there are two main types of myasthenia gravis in dogs: acquired and congenital. (simplewag.com)
- The specific causes of Myasthenia Gravis in dogs have been researched by professional veterinarians for years. (simplewag.com)
- Generalized fatigue in myasthenia gravis results in physical deconditioning that reduces fitness and increases risk of obesity, hypertension, elevated cholesterol and type 2 diabetes. (clinicaltrials.gov)
- Generalized fatigue in myasthenia gravis results in a sedentary lifestyle and profound physical deconditioning, leading to reduced fitness and increased risk of obesity, hypertension, dyslipidemia, and type 2 diabetes. (clinicaltrials.gov)
- Hypotheses are that a) subjects with myasthenia gravis are physically inactive with reduced fitness level and high incidence of obesity and poor cardiovascular disease risk profile, and b) a 3 month multi-modal home exercise program that includes aerobic, resistive, and pulmonary training components will improve physical activity, strength, fitness, dyspnea, body composition and reduce cardiovascular disease risk. (clinicaltrials.gov)
- A Cochrane review 1 included one study with a total of 10 subjects with myasthenia gravis (MG). It used acetylcholinesterase inhibitor, intranasal neostigmine, including 3 participants with ocular and 7 with generalised myasthenia gravis. (unboundmedicine.com)
- Scientists are evaluating new and improving current treatments for myasthenia gravis. (abingtonneurology.com)
- And as part of this, I just want to mention that we have a large practice in neuromuscular diseases and we have a large clinic with patients with myasthenia gravis. (healthprofessionalradio.com.au)
- Still, the cause of the disease remained a mystery until 1960, when Simpson proposed that myasthenia gravis was caused by antibodies against the acetylcholine receptor. (yale.edu)
- Canine mysathenia gravis is a neuromuscular disorder that can limit your dog's mobility and inhibit major body functions. (vetinfo.com)