| Discordant thymectomy in identical twins concordant for myasthenia gravis. | |
MedLine Citation:
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PMID: 21969350 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Thymectomy is standard therapy fornonthymomatousmyasthenia gravis despite the absence of randomized clinical trials (1). Myasthenia gravis is uncommonly reported in monozygous twins; disease concordance occurs in approximately one third of such identical twin pairs; and treatment for myasthenia gravis, when described,is usually concordant in identical twin pairs (2). OBJECTIVE: To report an 11-year clinical course of a pair of identical twins concordant for generalized acetylcholine receptor antibody–positive nonthymomatous myasthenia gravis in whom only 1 was treated with extended transsternal thymectomy. CASE REPORT: Twin A was a 19-year-old white woman who presented with an 8-week history of intermittent leg weakness, causing her to fall during activities, such as climbing stairs. On examination,she had moderately severe fatigable proximal muscle weakness and ptosis. Her weakness improved with intravenous edrophonium administration.Initial binding acetylcholine receptor antibody titer was 1.22 nmol/L (normal value, 0.03 nmol/L). Repetitive 2-Hz nerve(median, ulnar, and facial) stimulation studies demonstrated up to a 16% decremental response. Chest computed tomography showed residual thymic tissue without thymoma. An extended transsternal thymectomy was performed 11 weeks after the onset of symptoms. |
Authors:
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Kevin R Riggs; Laurie Gutmann; Jack E Riggs |
Publication Detail:
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Type: Case Reports; Letter |
Journal Detail:
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Title: Annals of internal medicine Volume: 155 ISSN: 1539-3704 ISO Abbreviation: Ann. Intern. Med. Publication Date: 2011 Oct |
Date Detail:
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Created Date: 2011-10-04 Completed Date: 2012-07-02 Revised Date: 2013-06-25 |
Medline Journal Info:
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Nlm Unique ID: 0372351 Medline TA: Ann Intern Med Country: United States |
Other Details:
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Languages: eng Pagination: 478-9 Citation Subset: AIM; IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Autoantibodies
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blood Cholinesterase Inhibitors / therapeutic use* Diseases in Twins / drug therapy*, immunology, surgery* Edrophonium / therapeutic use Female Humans Myasthenia Gravis / drug therapy*, immunology, surgery* Pyridostigmine Bromide / therapeutic use Receptors, Cholinergic / immunology Remission Induction Thymectomy* Twins, Monozygotic* Young Adult |
| Chemical | |
Reg. No./Substance:
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0/Autoantibodies; 0/Cholinesterase Inhibitors; 0/Receptors, Cholinergic; 101-26-8/Pyridostigmine Bromide; 312-48-1/Edrophonium |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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