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Neurologic Clinics May 2018Myasthenia gravis (MG) is a rare disease, but the most common disorder of the neuromuscular junction. It is the prototypic autoimmune disease most commonly caused by... (Review)
Review
Myasthenia gravis (MG) is a rare disease, but the most common disorder of the neuromuscular junction. It is the prototypic autoimmune disease most commonly caused by antibodies to the acetylcholine receptor (AChR) leading to characteristic fatigable weakness of the ocular, bulbar, respiratory, axial, and limb muscles. The majority of patients with MG first present with ocular symptoms. Most patients with MG will experience at least 1 exacerbation of symptoms throughout the course of their illness. This article will cover the epidemiology, clinical presentation, classification, and natural history of MG.
Topics: Humans; Myasthenia Gravis
PubMed: 29655448
DOI: 10.1016/j.ncl.2018.01.002 -
Indian Journal of Ophthalmology Oct 2014Myasthenia gravis (MG) is a disease that affects the neuro-muscular junction resulting in classical symptoms of variable muscle weakness and fatigability. It is called... (Review)
Review
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.
Topics: Disease Management; Global Health; Humans; Morbidity; Myasthenia Gravis
PubMed: 25449931
DOI: 10.4103/0301-4738.145987 -
CMAJ : Canadian Medical Association... Sep 2018
Topics: Diagnostic Techniques, Ophthalmological; Electromyography; Humans; Myasthenia Gravis; Thymectomy
PubMed: 30249760
DOI: 10.1503/cmaj.180656 -
Neurologic Clinics Feb 2021Myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) are the most common disorders of neuromuscular transmission in clinical practice. Disorders of the... (Review)
Review
Myasthenia gravis (MG) and Lambert-Eaton myasthenic syndrome (LEMS) are the most common disorders of neuromuscular transmission in clinical practice. Disorders of the neuromuscular junction (NMJ) are characterized by fluctuating and fatigable weakness and include autoimmune, toxic, and genetic conditions. Adults with NMJ disorders are most often antibody mediated, with MG being the most common, having a prevalence of approximately 1 in 10,000, and with women being affected about twice as often as men. This article focuses on advances in management of autoimmune MG and LEMS.
Topics: Adult; Female; Humans; Lambert-Eaton Myasthenic Syndrome; Male; Myasthenia Gravis
PubMed: 33223079
DOI: 10.1016/j.ncl.2020.09.007 -
Annals of the New York Academy of... Jan 2018Generalized myasthenia gravis (gMG) is a rare autoimmune disorder characterized by skeletal muscle weakness caused by disrupted neurotransmission at the neuromuscular... (Review)
Review
Generalized myasthenia gravis (gMG) is a rare autoimmune disorder characterized by skeletal muscle weakness caused by disrupted neurotransmission at the neuromuscular junction (NMJ). Approximately 74-88% of patients with gMG have acetylcholine receptor (AChR) autoantibodies. Complement plays an important role in innate and antibody-mediated immunity, and activation and amplification of complement results in the formation of membrane attack complexes (MACs), lipophilic proteins that damage cell membranes. The role of complement in gMG has been demonstrated in animal models and patients. Studies in animals lacking specific complement proteins have confirmed that MAC formation is required to induce experimental autoimmune MG (EAMG) and NMJ damage. Complement inhibition in EAMG models can prevent disease induction and reverse its progression. Patients with anti-AChR MG have autoantibodies and MACs present at NMJs. Damaged NMJs are associated with more severe disease, fewer AChRs, and MACs in synaptic debris. Current MG therapies do not target complement directly. Eculizumab is a humanized monoclonal antibody that inhibits cleavage of complement protein C5, preventing MAC formation. Eculizumab treatment improved symptoms compared with placebo in a phase II study in patients with refractory gMG. Direct complement inhibition could preserve NMJ physiology and muscle function in patients with anti-AChR gMG.
Topics: Animals; Antibodies, Monoclonal, Humanized; Autoantibodies; Complement Inactivating Agents; Complement Membrane Attack Complex; Complement System Proteins; Humans; Myasthenia Gravis; Myasthenia Gravis, Autoimmune, Experimental; Neuromuscular Junction; Receptors, Cholinergic; Synaptic Transmission
PubMed: 29266249
DOI: 10.1111/nyas.13522 -
Medicina 2019Juvenile myasthenia gravis is a rare autoimmune disease, which has made it difficult to collect data from prospective randomized controlled trials to evaluate the...
Juvenile myasthenia gravis is a rare autoimmune disease, which has made it difficult to collect data from prospective randomized controlled trials to evaluate the efficacy and results of different treatments. Although there are differences between the juvenile myasthenia gravis and that of the adult, the data provided by some researches in adults in the treatment of juvenile myasthenia gravis have been used. The different therapeutic options will be evaluated, with the different evidences that sustain it and a treatment algorithm will be elaborated keeping always in mind that each patient offers us different challenges.
Topics: Child; Cholinesterase Inhibitors; Humans; Immunosuppressive Agents; Myasthenia Gravis; Steroids; Thymectomy
PubMed: 31603848
DOI: No ID Found -
Nature Reviews. Neurology May 2016Myasthenia gravis (MG) is an autoimmune disorder caused by autoantibodies that target the neuromuscular junction, leading to muscle weakness and fatigability. Currently... (Review)
Review
Myasthenia gravis (MG) is an autoimmune disorder caused by autoantibodies that target the neuromuscular junction, leading to muscle weakness and fatigability. Currently available treatments for the disease include symptomatic pharmacological treatment, immunomodulatory drugs, plasma exchange, thymectomy and supportive therapies. Different autoantibody patterns and clinical manifestations characterize different subgroups of the disease: early-onset MG, late-onset MG, thymoma MG, muscle-specific kinase MG, low-density lipoprotein receptor-related protein 4 MG, seronegative MG, and ocular MG. These subtypes differ in terms of clinical characteristics, disease pathogenesis, prognosis and response to therapies. Patients would, therefore, benefit from treatment that is tailored to their disease subgroup, as well as other possible disease biomarkers, such as antibodies against cytoplasmic muscle proteins. Here, we discuss the different MG subtypes, the sensitivity and specificity of the various antibodies involved in MG for distinguishing between these subtypes, and the value of antibody assays in guiding optimal therapy. An understanding of these elements should be useful in determining how to adapt existing therapies to the requirements of each patient.
Topics: Autoantibodies; Biomarkers; Humans; Myasthenia Gravis; Neuromuscular Junction
PubMed: 27103470
DOI: 10.1038/nrneurol.2016.44 -
Neuromuscular Disorders : NMD Feb 2020Myasthenia gravis is an autoimmune disease characterized by dysfunction of the neuromuscular junction. Current treatment is based on lifestyle advice, symptomatic... (Review)
Review
Myasthenia gravis is an autoimmune disease characterized by dysfunction of the neuromuscular junction. Current treatment is based on lifestyle advice, symptomatic treatment, immunosuppressive drugs and thymectomy. Corticosteroids remain the cornerstone of treatment beside symptomatic medication due to their low cost, wide availability and fast mode of action. However, long term steroid use carries substantial risks of severe adverse side effects. Therefore, non-steroidal immunosuppressive drugs are commonly added to the treatment. Unfortunately, they have a delayed-onset effect and evidence of their efficacy appears to be difficult to obtain. Several trials using drugs that have had clear positive results in other immunological disorders have failed in myasthenia. This failure may in part be related to difficulties in the design of clinical trial for myasthenia, which has a fluctuating disease course involving weakness that may be difficult to assess quantitively. This problem is exacerbated by the tendency of most clinical trials to select patients with a stable, but severe disease. Future trials should: select patients with weakness and fatigability that is completely explained by their myasthenia gravis, use a design that avoids the exclusion of patients with recent changes in medication, and explore the possibilities to completely avoid the use of corticosteroids.
Topics: Adrenal Cortex Hormones; Antibodies, Monoclonal; Exercise Therapy; Humans; Immunosuppressive Agents; Myasthenia Gravis; Proteasome Inhibitors; Thymectomy
PubMed: 32007304
DOI: 10.1016/j.nmd.2019.12.003 -
Revue Neurologique Mar 2021Myasthenia gravis is an autoimmune disease characterised by fluctuating muscle weakness, which worsens during activity. It affects particularly scapular and pelvic... (Review)
Review
Myasthenia gravis is an autoimmune disease characterised by fluctuating muscle weakness, which worsens during activity. It affects particularly scapular and pelvic girdles, axial and bulbar muscles. Myasthenia gravis is twice more frequent in women and symptoms often appear in the second and third decade of life. Thus, a growing number of women affected by this condition become pregnant. To minimise the effects of myasthenia gravis on pregnancy and the newborn, and to avoid myasthenia crisis in the post-partum, the pregnancy must be planned as far as possible. During pregnancy, treatment must be reviewed due to the threat of teratogenic effects (mycophenolate mofetil, rituximab), and the follow-up must be multidisciplinary.
Topics: Female; Humans; Infant, Newborn; Myasthenia Gravis; Pregnancy; Pregnancy Complications
PubMed: 33648779
DOI: 10.1016/j.neurol.2020.09.015 -
Neurologic Clinics May 2018Ocular myasthenia is a form of myasthenia gravis in which weakness is restricted to the ocular muscles and may produce significant visual disability. Patients present... (Review)
Review
Ocular myasthenia is a form of myasthenia gravis in which weakness is restricted to the ocular muscles and may produce significant visual disability. Patients present with fluctuating ptosis, diplopia, or a combination of both. Examination may show any type of ocular motility deficit ranging from isolated muscle palsy to complete ophthalmoplegia. Cogan lid twitch, enhanced ptosis, peek sign, and saccadic fatigue are specific examination findings that support the clinical diagnosis of myasthenia gravis. Confirmation of the diagnosis is challenging with autoantibody serology, and repetitive nerve stimulation studies are often negative.
Topics: Humans; Myasthenia Gravis; Oculomotor Muscles
PubMed: 29655447
DOI: 10.1016/j.ncl.2018.01.003