We report six sibs with arthrogryposis multiplex congenita and a Pena-Shokeir phenotype, born to a healthy woman who was discovered to have asymptomatic myasthenia gravis (MG). This is the first report of anti-acetylcholine receptor (AChR) antibodies causing fetal akinesia/hypokinesia sequence in th
Teratogen update: Maternal myasthenia gravis as a cause of congenital arthrogryposis
✍ Scribed by Polizzi, A. ;Huson, S.M. ;Vincent, A.
- Book ID
- 101322803
- Publisher
- John Wiley and Sons
- Year
- 2000
- Tongue
- English
- Weight
- 186 KB
- Volume
- 62
- Category
- Article
- ISSN
- 0040-3709
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✦ Synopsis
Background: Arthrogryposis multiplex congenita (AMC) is defined as nonprogressive congenital contractures that generally result from lack of fetal movement in utero. AMC is a feature of many congenital disorders caused by genetic, environmental, or other factors. One rare cause of AMC is maternal myasthenia gravis (MG). This is an autoimmune disorder, caused by antibodies to the nicotinic acetylcholine receptor (AChR), and resulting in weakness of voluntary muscles. In 10 -15% of babies born to MG mothers, transient signs of MG are noted after placental transfer of anti-AChR antibodies. In a few cases, AMC predominates.
Methods:
We review the role of antibodies to AChR in MG and in AMC associated with maternal antibodies to AChR.
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