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Seminars in Pediatric Neurology May 2017Myasthenia gravis is a disorder of neuromuscular transmission that leads to fatigue of skeletal muscles and fluctuating weakness. Myasthenia that affects children can be... (Review)
Review
Myasthenia gravis is a disorder of neuromuscular transmission that leads to fatigue of skeletal muscles and fluctuating weakness. Myasthenia that affects children can be classified into the following 3 forms: transient neonatal myasthenia, congenital myasthenic syndromes, and juvenile myasthenia gravis (JMG). JMG is an autoimmune disorder that has a tendency to affect the extraocular muscles, but can also affect all skeletal muscles leading to generalized weakness and fatigability. Respiratory muscles may be involved leading to respiratory failure requiring ventilator support. Diagnosis should be suspected clinically, and confirmatory diagnostic testing be performed, including serum acetylcholine receptor antibodies, repetitive nerve stimulation, and electromyography. Treatment for JMG includes acetylcholinesterase inhibitors, immunosuppressive medications, plasma exchange, intravenous immunoglobulins, and thymectomy. Children with myasthenia gravis require monitoring by a pediatric ophthalmologist for the development of amblyopia from ptosis or strabismus.
Topics: Adolescent; Child; Child, Preschool; Humans; Infant; Infant, Newborn; Myasthenia Gravis
PubMed: 28941526
DOI: 10.1016/j.spen.2017.04.003 -
Revista de Neurologia Sep 2022Myasthenia gravis is characterized by skeletal muscle weakness, the most common initial presentation includes ocular weakness with asymmetric ptosis and binocular... (Review)
Review
INTRODUCTION
Myasthenia gravis is characterized by skeletal muscle weakness, the most common initial presentation includes ocular weakness with asymmetric ptosis and binocular diplopia. Around 19-50% of pregnant women with myasthenia gravis will experience a worsening of the disease. The objective of this article was to review the current information regarding the interrelation between MG and pregnancy; as well as its approach.
DEVELOPMENT
Bibliographic search in databases such as PubMed, ScienceDirect, SciELO, Google Scholar and medRxiv. Original articles, reviews, series and case reports between 2013 and 2022 are included.
CONCLUSIONS
Myasthenia gravis would not significantly effects on pregnancy; however, pregnancy can exacerbate the disease, especially during the first trimester or after delivery. The approach to pregnant women with myasthenia gravis must be multidisciplinary and involves the adjustment of pharmacological treatment and constant monitoring.
Topics: Blepharoptosis; Diplopia; Eye; Female; Humans; Muscle Weakness; Myasthenia Gravis; Pregnancy
PubMed: 35880965
DOI: 10.33588/rn.7505.2022207 -
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 -
La Revue Du Praticien Mar 2023
Topics: Humans; Myasthenia Gravis
PubMed: 37289123
DOI: No ID Found -
Disease-a-month : DM Nov 1990Myasthenia Gravis is a disorder of neuromuscular function resulting from an immunologically based premature destruction of acetylcholine receptors. The disease is... (Review)
Review
Myasthenia Gravis is a disorder of neuromuscular function resulting from an immunologically based premature destruction of acetylcholine receptors. The disease is characterized clinically by variable weakness accentuated by repetitive muscular activity and usually responding to the administration of acetylcholinesterase inhibitors. Myasthenia Gravis is a complex disease and requires understanding of the many facets of its natural history and immunological basis to ensure optimal individual patient management. The long-term goal is control of the immunological imbalance; treatment regimens include thymectomy, corticosteroids, azathioprine, and plasmapheresis. The common use of acetylcholinesterase inhibitors provides symptomatic relief during variable daily muscular activity. Disability due to myasthenia gravis is to a large extent reversible and death is preventable. Early recognition of myasthenia gravis and appropriate treatment are often rewarded by remission that may be permanent.
Topics: Diagnosis, Differential; Humans; Myasthenia Gravis
PubMed: 2209357
DOI: 10.1016/0011-5029(90)90006-d -
Lancet (London, England) Jun 2001
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Annals of Internal Medicine Aug 1974
Review
Topics: Adolescent; Adult; Aged; Anti-Arrhythmia Agents; Anti-Bacterial Agents; Autonomic Agents; Electromyography; Female; Glucocorticoids; Humans; Lactates; Male; Middle Aged; Muscles; Myasthenia Gravis; Neuromuscular Junction; Prednisone; Respiration; Synaptic Transmission; Thymus Gland
PubMed: 4367701
DOI: 10.7326/0003-4819-81-2-225 -
Asia-Pacific Journal of Ophthalmology... 2018Myasthenia gravis is a relatively common neuromuscular disorder, with ocular myasthenia gravis being a subset defined as myasthenia gravis limited to the orbicularis,... (Review)
Review
Myasthenia gravis is a relatively common neuromuscular disorder, with ocular myasthenia gravis being a subset defined as myasthenia gravis limited to the orbicularis, levator, and extraocular muscles. Patients with ocular myasthenia gravis can have disabling diplopia or functional blindness from ptosis and in most cases treatment is required. Like generalized myasthenia gravis, there are a variety of treatments available that include pyridostigmine, immunosuppression, intravenous immunoglobulin, plasmapheresis, thymectomy, lid crutches, ptosis surgery, and extraocular muscle surgery. Unfortunately, there is limited data on the use of individual treatments in ocular myasthenia gravis and no data comparing treatments. Using a combination of available data on treatment of generalized myasthenia gravis, data on treatment of ocular myasthenia gravis, best practices, and clinical experience we will provide a rational framework for treatment of ocular myasthenia gravis.
Topics: Eye Movements; Humans; Immunosuppression Therapy; Myasthenia Gravis; Oculomotor Muscles; Ophthalmologic Surgical Procedures; Plasmapheresis; Prognosis; Visual Acuity
PubMed: 30044061
DOI: 10.22608/APO.2018301 -
Disease-a-month : DM Feb 1997Adult-onset myasthenia gravis is an acquired autoimmune disorder of neuromuscular transmission in which acetylcholine receptor antibodies attack the postsynaptic... (Review)
Review
Adult-onset myasthenia gravis is an acquired autoimmune disorder of neuromuscular transmission in which acetylcholine receptor antibodies attack the postsynaptic membrane of the neuromuscular junction. Although the cause of this disease is unknown, the role of immune responses in its pathogenesis is well established. Circulating acetylcholine receptor antibodies are present in 80% to 90% of patients with the generalized form of myasthenia gravis. Most patients have ptosis, diplopia, dysarthria and dysphagia. The weakness and fatigue worsen on exertion and improve with rest. Respiratory muscle and limb weakness are rare at the onset of the disease. For the past two decades, there has been considerable progress in understanding the diagnosis and management of myasthenia gravis. The diagnosis is based on clinical presentation, neurologic examination, and confirmation by means of electrophysiologic testing and immunologic studies. Myasthenia gravis mimics many neuromuscular diseases and even illnesses such as depression and chronic fatigue syndrome. One should always exclude drug-induced myasthenia gravis for all patients. With the introduction of new modalities of treatment, particularly immunosuppressive or immunomodulating drugs, plasma exchange and thymectomy, the morbidity and mortality of myasthenia gravis have decreased dramatically to the point that myasthenia gravis should not be considered as serious a disease as it once was. Although the several therapeutic options are usually effective and have meant independence in daily life to many patients with myasthenia gravis, well-designed, controlled, prospective studies are still lacking.
Topics: Adult; Female; Humans; Male; Middle Aged; Myasthenia Gravis
PubMed: 9118787
DOI: 10.1016/s0011-5029(97)90033-x -
Current Opinion in Ophthalmology Dec 1996Ocular myasthenia gravis may mimic any pupil-spared, painless, nonproptotic ophthalmoplegia with or without ptosis. This review references recent papers regarding... (Review)
Review
Ocular myasthenia gravis may mimic any pupil-spared, painless, nonproptotic ophthalmoplegia with or without ptosis. This review references recent papers regarding clinical features, diagnostic techniques, and treatment for ocular myasthenia gravis.
Topics: Combined Modality Therapy; Eye Diseases; Humans; Myasthenia Gravis; Syndrome
PubMed: 10166551
DOI: 10.1097/00055735-199612000-00006