Abstract:
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.
journal_name
Ann Intern Medjournal_title
Annals of internal medicineauthors
Riggs KR,Gutmann L,Riggs JEdoi
10.7326/0003-4819-155-7-201110040-00016subject
Has Abstractpub_date
2011-10-04 00:00:00pages
478-9issue
7eissn
0003-4819issn
1539-3704pii
155/7/478journal_volume
155pub_type
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