Myasthenia gravis (MG) is a neuromuscular disorder characterized by weakness and fatigability of skeletal muscles. It is caused by an autoimmune response mediated by specific anti-acetylcholine receptor antibodies. This results in a reduction of available acetylcholine receptors at the neuromuscular junction, impairing muscle activation. Symptoms include weakness that increases with repeated use of muscles and improves with rest. Ocular muscles are often initially affected, followed by oropharyngeal and limb muscles. Treatment focuses on acetylcholinesterase inhibitors, immunosuppression, and thymectomy in some cases.
Myasthenia gravis (MG) is a neuromuscular disorder characterized by weakness and fatigability of skeletal muscles. It is caused by an autoimmune response mediated by specific anti-acetylcholine receptor antibodies. This results in a reduction of available acetylcholine receptors at the neuromuscular junction, impairing muscle activation. Symptoms include weakness that increases with repeated use of muscles and improves with rest. Ocular muscles are often initially affected, followed by oropharyngeal and limb muscles. Treatment focuses on acetylcholinesterase inhibitors, immunosuppression, and thymectomy in some cases.
Myasthenia gravis (MG) is a neuromuscular disorder characterized by weakness and fatigability of skeletal muscles. It is caused by an autoimmune response mediated by specific anti-acetylcholine receptor antibodies. This results in a reduction of available acetylcholine receptors at the neuromuscular junction, impairing muscle activation. Symptoms include weakness that increases with repeated use of muscles and improves with rest. Ocular muscles are often initially affected, followed by oropharyngeal and limb muscles. Treatment focuses on acetylcholinesterase inhibitors, immunosuppression, and thymectomy in some cases.
Myasthenia gravis (MG) is a neuromuscular disorder characterized by weakness and fatigability of skeletal muscles. It is caused by an autoimmune response mediated by specific anti-acetylcholine receptor antibodies. This results in a reduction of available acetylcholine receptors at the neuromuscular junction, impairing muscle activation. Symptoms include weakness that increases with repeated use of muscles and improves with rest. Ocular muscles are often initially affected, followed by oropharyngeal and limb muscles. Treatment focuses on acetylcholinesterase inhibitors, immunosuppression, and thymectomy in some cases.
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KEPANITERAAN ILMU PENYAKIT SARAF RS PGI CIKINI
PERIODE 24 Juni 2013 20 Juli 2013
FAKULTAS KEDOKTERAN UNIVERSITAS KRISTEN INDONESIA JAKARTA
Dosen Pembimbing : dr. Hophoptua N. Manurung, Sp.S
Disusun oleh : Bintari Anindhita (09-58)
Myasthenia gravis (MG) is a neuromuscular disorder characterized by weakness and fatigability of skeletal muscles.
The underlying defect..
antibody-mediated autoimmune attack acetylcholine receptors (AChRs) at neuromuscular junctions
The neuromuscular abnormalities in MG are caused by an autoimmune response mediated by specific anti-AChR antibodies.
There are 3 distinct mechanisms: Rapid endocytosis of the receptors Blockade of the active site of the achr Damage to the postsynaptic muscle membrane The thymus appears to play a role in autoimune response in MG The thymus is abnormal in ~75% of patients with MG In ~65% the thymus is hyperplastic, with the presence of active germinal centers detected histologically An additional 10% of patients have thymic tumors (thymomas). Muscle-like cells within the thymus (myoid cells), which bear AChRs on their surface, may serve as a source of autoantigen and trigger the autoimmune reaction within the thymus gland. Number of available ACHRs The postsynaptic folds are atrophic Efficiency of neuromuscular transmission Fail to trigger muscle action Potentials Weakness of muscle contraction Normally.. The amount of ACh released per impulse declines on repeated activity (termed presynaptic rundown). In the myasthenic patient.. Efficiency of neuromuscular transmission Normal rundown Activation of fewer and fewer muscle fibers by successive nerve impulses Increasing weakness, or myasthenic fatigue The cardinal features weakness and fatigability of muscles. The weakness increases during repeated use (fatigue) and may improve following rest or sleep. Exacerbations and remissions may occur, particularly during the first few years after the onset of the disease. Ocular muscles affected first in about 40% of patients and are ultimately involved in about 85% (Ocular MG) Ptosis Diplopia Weakness of facial or oropharyngeal muscles, resulting in: dysarthria, dysphagia, and limitation of facial movements. Together, oropharyngeal and ocular weakness cause symptoms in virtually all patients with acquired MG. In ~85% of patients, the weakness becomes generalized, affecting the limb muscles as well often proximal and may be asymmetric
Generalized MG A. Ptosis B. Attempted gaze to the right. Only right eye abducts incompletely. C. Demonstrates proximal weakness upon attempt to raise the arms. D. Holding the arms and fingers extended the extensor muscles weaken and finger drop occurs Crisis occur in patients with oropharyngeal or respiratory muscle weakness. Provoked by.. respiratory infection surgical procedures emotional stress systemic illness Anticholinestrase test Acetylcholinesterase Inhibitors First line of therapy Neostigmine bromide (Pyridostigmine) Edrophonium chloride (Tensilon) Immunosuppressive Therapy Prednisone Azathioprine
Plasmapheresis Immunoglobulin Therapy Thymectomy
Drachman, D. B. (2010). Myasthenia Gravis and Other Diseases of the Neuromuscular Junction. In S. L. Hauser, & S. A. Josephson, Harrison's Neurology in Clinical Medicine (2 ed., pp. 559-566). The Mcgraw-Hill Companies.
Goldenberg, W. D., & Shah, A. K. (2013, February 11). Myasthenia Gravis. (N. Lorenzo, Editor) Retrieved July 10, 2013, from Medscape Drugs, Diseases & Procedures: http://emedicine.medscape.com/article/1171206-overview
Penn, A. S., & Rowland, L. P. (2005). Myasthenia Gravis. In L. P. Rowland, Merritt's Neurology (11 ed.). Lippincott Williams & Wilkins.
(Lippincott Williams & Wilkins Handbook Series) Randolph W. Evans MD, Ninan T. Mathew MD FRCP (C) - Handbook of Headache-Lippincott Williams & Wilkins (2005)