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Severe neonatal myasthenia due to maternal anti-MuSK antibodies

Authors :
Béhin, Anthony
Mayer, Michèle
Kassis-Makhoul, Bouchera
Jugie, Myriam
Espil-Taris, Caroline
Ferrer, Xavier
Chatenoud, Lucienne
Laforêt, Pascal
Eymard, Bruno
Source :
Neuromuscular Disorders. Jun2008, Vol. 18 Issue 6, p443-446. 4p.
Publication Year :
2008

Abstract

Abstract: Anti-MuSK antibodies have been reported in about 40–50% of patients with seronegative myasthenia gravis. Curiously, this condition has never been reported in association with fetal or transient neonatal myasthenia gravis, despite a known female predominance. We report the case of a 22-year-old woman who developed seronegative, mild steroid-responsive myasthenia gravis. When aged 26, she gave birth to a baby boy with neonatal myasthenia gravis characterized by hypotonia, stridor and sucking difficulties. Intubation was required for a few weeks. Anti-MuSK antibodies were assessed and found positive in both patients. Progressive hydramnios during the last trimester, with a decrease in spontaneous fetal mobility in the last weeks, long-lasting stridor, ptosis and occasional difficulties in swallowing liquids till two years of age, despite anti-MuSK antibodies becoming negative, suggest a fetal onset. The possible pathophysiology of this disorder, based on recent findings on the expression and function of MuSK protein, is reviewed. [Copyright &y& Elsevier]

Details

Language :
English
ISSN :
09608966
Volume :
18
Issue :
6
Database :
Academic Search Index
Journal :
Neuromuscular Disorders
Publication Type :
Academic Journal
Accession number :
32732161
Full Text :
https://doi.org/10.1016/j.nmd.2008.03.006