Prenatal Immunomodulation Treatment in Neonatal Myasthenia Gravis

Abstract

Neonatal mysthenia gravis (NMG) is a rare cause of arthrogryposis multiplex congenita (AMC) due to diaplacental transfer of maternal acetylcholine receptors (AChR) antibodies. 2 cases of severe NMG complicated by chronic lung disease and pulmonary arterial hypertension are reported. With respect to the severe course of the index patient, prenatal diagnosis and immunomodulation treatment were offered during the 2^nd pregnancy. The combination of prenatal immunoadsorption (IA) therapy, administration of intravenous immunoglobulin (IVIG) and prednisolone failed. Failure may be partly explained by immaturity of the infant. However, considering the successful treatment of fetal/neonatal alloimmune thrombocytopenia (AIT) reported in literature, a treatment approach with IVIG doses up to 1–2 g/kg per week plus prednisone/prednisolone at a higher dose up to 1 mg/kg/d might be more effective.

Zusammenfassung

Die neonatale Myasthenia gravis (NMG) ist eine seltene Ursache einer Arthrogryposis multiplex congenita (AMC) bedingt durch den diaplazentaren Übertritt maternaler Acetylcholinrezeptor (AChR) Antikörper. Wir berichten über 2 Fälle mit schwerer NMG, welche in Folge chronischer Lungenschäden und pulmonaler Hypertension kompliziert verliefen. Aufgrund des schweren klinischen Verlaufs des Indexpatienten wurde eine entsprechende Pränataldiagnostik und pränatale immunmodulierende Therapie während der 2.Schwangerschaft initiiert. Die Kombination aus pränataler Immunadsorption (IA), Gabe von intravenösen Immunglobulin (IVIG) und Prednisolon brachte nicht den gewünschten Erfolg. Das Scheitern kann zum Teil auch in der Frühgeburtlichkeit des Kindes begründet sein. Möglicherweise wäre ein Behandlungsansatz analog zu den in der Literatur beschriebenen erfolgreichen Ansätzen bei fetaler/neonataler Alloimmunthrombozytopenie mit IVIG-Dosen bis zu 1–2 g/kg/Woche und höheren Prednison/Prednisolongaben bis zu 1 mg/kg/d erfolgsversprechender.

Key words

Neonatal mysthenia gravis - arthrogryposis multiplex congenita - acetylcholine receptors antibodies - prenatal immuno­modulation treatment - immunoadsorption - IVIG

Schlüsselwörter

Neonatale Mysthenia gravis - Arthrogryposis multiplex congenita - Acetylcholinrezeptor ­Antikörper - pränatale immun­modulierende Therapie - Immunadsorption - IVIG

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