Marked hypotonia in an infant of a mother with Devic disease.

J Child Neurol

Neonatology Department, Meir Medical Center, Sackler School of Medicine, Tel Aviv University, Kfar Saba, Israel.

Published: June 2010

AI Article Synopsis

  • * The newborn tested positive for anti-acetylcholine receptor antibodies, leading to a suspicion of neonatal myasthenia gravis, a condition that can cause muscle weakness in infants.
  • * After intravenous immunoglobulin treatment, the infant showed significant improvement, raising the possibility that the mother's elevated antibodies could have affected both children, especially since her previous son likely had unrecognized transient myasthenia gravis.

Article Abstract

A full-term female neonate was born with severe hypotonia and weakness. Her mother had been treated for neuromyelitis optica (Devic disease) for 6 years. Her previous son, born 10 years earlier and before she developed the disease, also had marked hypotonia that gradually improved over several weeks. A suspicion of neonatal myasthenia gravis arose, as a search of the literature revealed the occasional detection of anti-acetylcholine receptor antibodies in patients with Devic disease. A neostigmine test was mildly positive in the baby, but anti-acetylcholine receptor antibodies were elevated. Aquaporin 4 antibodies typical of neuromyelitis optica were not detected in the infant. Because of clinical deterioration, intravenous immunoglobulin was administered with substantial improvement. Anti-acetylcholine antibodies were markedly elevated in the mother's serum, although she showed no clinical signs of myasthenia gravis. It is very likely that her previous baby also had unrecognized transient myasthenia gravis.

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Source
http://dx.doi.org/10.1177/0883073809343316DOI Listing

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