Document Detail


Discordant thymectomy in identical twins concordant for myasthenia gravis.
MedLine Citation:
PMID:  21969350     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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:
Kevin R Riggs; Laurie Gutmann; Jack E Riggs
Publication Detail:
Type:  Case Reports; Letter    
Journal Detail:
Title:  Annals of internal medicine     Volume:  155     ISSN:  1539-3704     ISO Abbreviation:  Ann. Intern. Med.     Publication Date:  2011 Oct 
Date Detail:
Created Date:  2011-10-04     Completed Date:  2012-07-02     Revised Date:  2013-06-25    
Medline Journal Info:
Nlm Unique ID:  0372351     Medline TA:  Ann Intern Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  478-9     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Autoantibodies / 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:
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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