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Transiente neonatale Myasthenia gravis

Transient neonatal myasthenia gravis

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Zusammenfassung

10–20% der Kinder von Müttern mit Myasthenia gravis entwickeln selbst eine transiente neonatale Myasthenia gravis, da die für die mütterliche Erkrankung verantwortlichen Antikörper die Plazentaschranke überwinden können. Wir berichten über 2 Geschwister, Kinder einer Patientin mit Myasthenia gravis und eines gesunden Vaters. Im 3. Schwangerschaftstrimenon der ersten Schwangerschaft trat ein Polyhydramnion auf. Das Neugeborene zeigte die ausgeprägten klinischen Symptome einer Myasthenie mit muskulärer Hypotonie, respiratorischer Insuffizienz, schwachem Schreien, Amimie und Trinkschwäche. Initial war eine maschinelle Beatmung notwendig. Die Bestätigung der Diagnose erfolgte durch repetitive Reizung mit typischem Decrement im Elektromyogramm (EMG) sowie Bestimmung des Azetylcholinrezeptorantikörpertiters im Serum. Bis zum Ende des 3. Lebensmonats wurde eine Therapie mit Pyridostigmin (Azetylcholinesterasehemmer) durchgeführt, ab dem 26. Lebenstag erfolgte die Betreuung ambulant. Die myasthene Symptomatik bildete sich vollständig zurück. Nebenwirkungen der medikamentösen Therapie traten nicht auf. Die 2. Schwangerschaft verlief unauffällig, das Neugeborene war gesund. Bemerkenswert an den hier vorgestellten Fällen ist—neben dem unterschiedlichen Schwangerschaftsverlauf—die schwere Ausprägung der myasthenen Symptomatik bei dem ersten Neugeborenen, die initial intensivmedizinische Maßnahmen erforderte, dann aber von einer raschen Erholung und kompletten Rückbildung der Symptome gefolgt wurde. Außerdem beschreiben wir die Steuerung der Pyridostigmintherapie anhand eines klinischen Scores.

Abstract

In 10–20% of cases, the offspring of mothers suffering from myasthenia gravis also develop transient neonatal myasthenia gravis since maternal antibodies are able to cross the placenta. We report the course of two newborns of a mother with MG and a healthy father. The first pregnancy was complicated during the 3rd trimester by hydramnios. The newborn presented with generalized muscle weakness, respiratory distress, weak crying response, amimia, and poor sucking. Mechanical ventilation was necessary. The diagnosis was confirmed by the results of repetitive muscle stimulation showing a typical decrement in the EMG and measurement of serum antiacetylcholine receptor antibodies. The infant was treated with pyridostigmine (cholinesterase inhibitor) for 3 months. After 26 days of hospitalization, the patient was released and followed up regularly. Myasthenic symptoms completely resolved. Side effects of the treatment were not observed. The course of the second pregnancy was normal. This second newborn was healthy. Our case report is remarkable for the very different presentation of two children of the same mother with myasthenia gravis during pregnancy, with one child developing severe transient neonatal myasthenia gravis after delivery, initially requiring ICU treatment followed by quick recovery, and one child being healthy. We also present a score to monitor the clinical course and to adjust anticholinesterase therapy accordingly.

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Notes

  1. In seltenen Fällen (ca. 15%) werden keine AChR-AK, sondern AK gegen eine muskelspezifische Kinase (MuSK) gefunden [37].

  2. Aus den oben genannten Messungen lässt sich eine "Eliminationshalbwertszeit" von etwa 12 (11,93) Tagen für die AchR-AK berechnen.

  3. Abweichend hiervon finden Tagher et al. eine Prävalenz von 54% [31].

  4. Innerhalb von 10–15 min nach der Gabe von Edrophonium (0,5–1 mg i. m. oder s. c.) bzw. innerhalb von etwa 30 min nach der Gabe von Neostigmin (0,15 mg i. m.) tritt eine deutliche Besserung der myasthenen Symptomatik auf.

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Authors and Affiliations

  1. Zentrum für Kinderheilkunde, Universität zu Köln

    Ch. Licht, A. Kribs, P. Herkenrath, D. V. Michalk & B. Roth

  2. Klinik und Poliklinik für Neurologie, Universität zu Köln

    P. Model & W. F. Haupt

  3. Zentrum für Kinderheilkunde, Universität zu Köln, Joseph-Stelzmann-Straße 9, 50931 , Köln

    Ch. Licht

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  1. Ch. Licht
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  2. P. Model
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Correspondence to Ch. Licht.

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Licht, C., Model, P., Kribs, A. et al. Transiente neonatale Myasthenia gravis. Monatsschr Kinderheilkd 152, 555–560 (2004). https://doi.org/10.1007/s00112-003-0669-2

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