Topical Review Article: Transient Neonatal Myasthenia Gravis

Abstract

Transient neonatal myasthenia gravis is a postsynaptic neuromuscular transmission defect occurring in 21% of infants born to women with active (and, less commonly, in remission) acquired myasthenia gravis. Although passive-transfer acetylcholine receptor (AChR) antibodies are found in the majority of these newborns, their pathogenic role is questionable because only some infants are symptomatic. Pathogenesis in infants without AChR antibodies is unknown. There is still no biologic marker for prenatal identification of this subpopulation of newborns, although HLA typing may be a promising tool. Sucking, swallowing, and respiratory difficulties are the most common presenting signs in the first day of life. Final diagnosis is done when administration of acetylcholinesterase agents transiently corrects the neuromuscular transmission defect. Serum AChR antibody titers follow the same pattern as their mothers. Supportive management and anticholinesterase agents prior to feedings are necessary in about 80% of patients. In the majority of infants the condition resolves spontaneously. ( J Child Neurol 1992;7:135-141).