title: Marked hypotonia in an infant of a mother with Devic disease.
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.
authors.affiliation: Neonatology Department, Meir Medical Center, Sackler School of Medicine, Tel Aviv University, Kfar Saba, Israel. nathan.watemberg@clalit.org.il
authors.forename: Eyal
authors.initials: E
authors.lastname: Zifman
authors.forename: Ita
authors.initials: I
authors.lastname: Litmanovitz
authors.forename: Gil
authors.initials: G
authors.lastname: Segal
authors.forename: Rivka
authors.initials: R
authors.lastname: Regev
authors.forename: Nathan
authors.initials: N
authors.lastname: Watemberg
mesh_terms: D001323:Autoantibodies / Q000276:immunology; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007231:Infant, Newborn; D008431:Maternal-Fetal Exchange; D009123:Muscle Hypotonia / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D020941:Myasthenia Gravis, Neonatal / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy; D009471:Neuromyelitis Optica / Q000276:immunology*; D011247:Pregnancy; D011950:Receptors, Cholinergic / Q000276:immunology
chemical_list: D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D011950:Receptors, Cholinergic