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Acta Neurologica Scandinavica Feb 2005We give an update on clinical, immunological, and therapeutic advances in the field of myasthenia gravis, including a summary of suggested therapeutic recommendations. (Review)
Review
We give an update on clinical, immunological, and therapeutic advances in the field of myasthenia gravis, including a summary of suggested therapeutic recommendations.
Topics: Cholinesterase Inhibitors; Humans; Immunoglobulins, Intravenous; Immunosuppressive Agents; Myasthenia Gravis; Plasmapheresis; Thymectomy
PubMed: 15644074
DOI: 10.1111/j.1600-0404.2005.00374.x -
Paediatric Drugs 2000Myasthenia in children can be juvenile (autoimmune) or congenital. Juvenile myasthenia (JM) is an autoimmune disorder characterised by fluctuating weakness and fatigue... (Review)
Review
Myasthenia in children can be juvenile (autoimmune) or congenital. Juvenile myasthenia (JM) is an autoimmune disorder characterised by fluctuating weakness and fatigue in the ocular, facial, bulbar or limb muscles. Diagnosis is confirmed by electromyography (EMG), single fibre EMG and the patient's clinical response to anticholinesterase medication. Serology is less helpful in children because acetylcholine receptor antibodies, usually positive in adults, are frequently absent in patients with prepubertal onset of the disease. Treatment methods in JM include anticholinesterase drugs, thymectomy and immunomodulatory agents. Plasmapheresis and intravenous immunoglobulin are used in myasthenic crisis. The prognosis of patients with JM is usually good, clinical remission being achieved in the majority of patients with the current treatment methods.
Topics: Adult; Child; Cholinesterase Inhibitors; Humans; Immunotherapy; Myasthenia Gravis; Thymectomy
PubMed: 10937467
DOI: 10.2165/00128072-200002030-00001 -
Hematology/oncology Clinics of North... Jun 2008The relationship between myasthenia gravis and thymic pathology, including thymoma, is well known. Approximately 10% to 15% of patients who have myasthenia gravis are... (Review)
Review
The relationship between myasthenia gravis and thymic pathology, including thymoma, is well known. Approximately 10% to 15% of patients who have myasthenia gravis are observed to have a thymoma. Myasthenia gravis may be considered as the most common of the paraneoplastic syndromes in patients who have thymoma. This article summarizes the clinical aspects of myasthenia gravis, followed by a review of the less often recognized paraneoplastic disorders noted to occur in patients who have thymoma.
Topics: Cholinesterase Inhibitors; Humans; Immunosuppressive Agents; Myasthenia Gravis; Paraneoplastic Syndromes; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 18514130
DOI: 10.1016/j.hoc.2008.03.004 -
Recenti Progressi in Medicina Jan 1977
Review
Topics: Autoimmune Diseases; Cholinesterase Inhibitors; Humans; Myasthenia Gravis; Neostigmine; Physostigmine; Pyridostigmine Bromide; Thymectomy; Thymoma; Thymus Neoplasms
PubMed: 323942
DOI: No ID Found -
Annals of the New York Academy of... Feb 2018Because of the failure of many promising therapeutics identified in preclinical evaluation, funding sources have established guidelines for increased rigor in animal... (Review)
Review
Because of the failure of many promising therapeutics identified in preclinical evaluation, funding sources have established guidelines for increased rigor in animal evaluations. The myasthenia gravis (MG) community of scientists has developed guidelines for preclinical assessment for potential MG treatments. Here, we provide a focused summary of these recommendations and the role of complement in disease development in experimental models of MG.
Topics: Animals; Autoantibodies; Complement Inactivator Proteins; Complement System Proteins; Disease Models, Animal; Immunization, Passive; Immunoglobulin G; Macaca mulatta; Mice; Mice, Inbred C57BL; Myasthenia Gravis, Autoimmune, Experimental; Rats; Rats, Inbred Lew; Receptor Protein-Tyrosine Kinases; Receptors, Cholinergic
PubMed: 29356015
DOI: 10.1111/nyas.13555 -
Revue Neurologique Dec 2010Myasthenia gravis is a rare, auto-immune disorder of the neuromuscular junction. Onset signs frequently involve ocular muscles, accounting for ptosis and/or diplopia in... (Review)
Review
Myasthenia gravis is a rare, auto-immune disorder of the neuromuscular junction. Onset signs frequently involve ocular muscles, accounting for ptosis and/or diplopia in more than 75% of cases. Among the cases with purely ocular muscle involvement, less than one half will never progress towards a more general form of myasthenia. However, even if they do not share the potentially life-threatening course of generalized myasthenia, purely ocular forms are often responsible for severe impairment in everyday life. The diagnosis is essentially based on fluctuations in the time and topography of the ocular muscle weakness. It still remains uneasy, as investigations such as electromyography, search for antiacetycholine receptor antibodies (positive in 50% of cases of purely ocular myasthenia), and edrophonium chloride test sometimes yield false negative results. Whereas some patients get better while on anticholinesterasic drugs alone, most of them will experience insufficient improvement and need steroids and/or immunosuppressant drugs. There is no indication for plasma exchanges, intravenous immunoglobulin or thymectomy (except in the presence of thymoma). This treatment could well decrease the risk of an evolution towards generalized myasthenia. The reasons underlying the vulnerability of ocular motor muscles in myasthenia are complex and several factors (linked to immunology, anatomy and function) may combine to bring about their specific involvement. In the future, randomized, controlled trials will be necessary, in order to determine a more rational approach of the treatment of ocular myasthenia, which currently lies mostly on retrospective data and the expertise of reference centers implicated in the management of the disease.
Topics: Autoimmune Diseases; Diagnosis, Differential; Electrophysiological Phenomena; Eye Diseases; Humans; Myasthenia Gravis; Thymectomy
PubMed: 21075410
DOI: 10.1016/j.neurol.2010.08.004 -
Journal of Clinical Neuroscience :... Feb 2014The 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitors, or statins, are commonly prescribed for prevention of cardiovascular morbidity. A rare side... (Review)
Review
The 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitors, or statins, are commonly prescribed for prevention of cardiovascular morbidity. A rare side effect of statin medication is the induction of autoimmune illnesses, including myasthenia gravis (myasthenia). Here we present two patients with seropositive myasthenia that developed 4 weeks after initiation of atorvastatin, increasing the total reported patients to seven. Reviewing recent literature we highlight the connections between statins, auto-immunity and myasthenia. Statins may favour T-cell phenotypes that reduce cell-mediated immunity but could increase antibody-mediated humoral immunity.
Topics: Aged; Atorvastatin; Autoimmunity; Female; Heptanoic Acids; Humans; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Male; Middle Aged; Myasthenia Gravis; Pyrroles
PubMed: 24433954
DOI: 10.1016/j.jocn.2013.11.009 -
JAMA Nov 1983
Review
Topics: Adrenal Cortex Hormones; Adult; Animals; Autoantibodies; Child; Disease Models, Animal; Electromyography; Female; Humans; Immunosuppressive Agents; Male; Myasthenia Gravis; Prednisone; Rats; Receptors, Cholinergic; Thymectomy
PubMed: 6355534
DOI: 10.1001/jama.250.18.2516 -
Seminars in Neurology 2000Ocular myasthenia gravis is a not uncommon autoimmune disorder causing diplopia, ptosis, and weakness of lid closure. The predilection of myasthenia for the ocular... (Review)
Review
Ocular myasthenia gravis is a not uncommon autoimmune disorder causing diplopia, ptosis, and weakness of lid closure. The predilection of myasthenia for the ocular muscles may be related to differences between limb and extraocular muscles in either physiological function or antigenicity. Clinically, ocular myasthenia can mimic any form of pupil-sparing ocular motility disorder. Dynamic abnormalities of myasthenic eye movements may reflect the primary hallmarks of the disease, which are fatigability and variability in strength, or secondary adaptive effects by the central nervous system. Tests to confirm the diagnosis include edrophonium challenge, repetitive nerve stimulation, single-fiber electromyography (EMG) of the frontalis, and assays for antibody directed against the acetylcholine receptor: all are less sensitive for ocular myasthenia than for generalized myasthenia. There is a higher incidence of other autoimmune conditions in myasthenia, notably thymoma and thyroid dysfunction. The differential diagnosis includes other diseases of the neuromuscular junction, such as Lambert-Eaton syndrome and botulism. Treatment consists of symptomatic use of acetylcholinesterase inhibitors and immunosuppression with steroids or azathioprine. Between 50 and 70% of patients with ocular myasthenia will eventually develop generalized disease: there is some retrospective data that steroids or azathioprine may reduce this by about 75%. The role of thymectomy in ocular myasthenia remains unclear.
Topics: Humans; Myasthenia Gravis; Ocular Motility Disorders; Oculomotor Muscles
PubMed: 10874773
DOI: 10.1055/s-2000-6829 -
Fortschritte Der Neurologie-Psychiatrie Jan 2004
Review
Topics: Electrophysiology; Humans; Myasthenia Gravis; Neurosurgical Procedures
PubMed: 14745689
DOI: 10.1055/s-2003-812457