Myasthenia gravis und Schwangerschaft

Zusammenfassung

Fragen zur Familienplanung und Schwangerschaft sind von großer praktischer Bedeutung in der Behandlung von Myasthenie-Patientinnen, da die Erstmanifestation häufig in die entscheidende Lebensphase der Familienplanung fällt. Schwangerschaft, Geburt und Stillen sind zwar mit einigen Besonderheiten verbunden, stellen unter Beachtung entsprechender Vorsichtsmaßnahmen in der Regel aber kein nennenswert erhöhtes Risiko im Vergleich zur Normalpopulation dar. Im Idealfall sollte die Schwangerschaft bei Myasthenie-Patientinnen nach Absprache mit dem behandelnden Neurologen bzw. der behandelnden Neurologin erfolgen, sodass eine Remission vor Eintritt der Schwangerschaft erreicht werden kann. Pyridostigmin und Glukokortikosteroide (Prednison, Prednisolon, Methylprednisolon) sind sicher einsetzbare Standard-Therapeutika in der Schwangerschaft. Es wird angestrebt, eine bestehende Therapie mit Azathioprin vor einer Schwangerschaft umzustellen. Bei ungeplanter Schwangerschaft kann nach dem gegenwärtigen Kenntnisstand die Therapie mit Azathioprin und Cyclosporin A fortgeführt werden. Schwangerschaften unter Mycophenolat Mofetil, Methotrexat, sowie Tacrolimus müssen konsequent vermieden werden. Gegebenenfalls sollte eine Umstellung auf Glukokortikosteroide oder intravenöse Immunglobuline erfolgen. Intravenöse Immunglobuline oder Plasmapheresen werden bei Exazerbationen bzw. myasthener Krise auch in der Schwangerschaft eingesetzt. Fetale Missbildungen wie Arthrogryposis multiplex congenita oder das fetale Acetylcholin Rezeptor-Inaktivierungssyndrom (FARIS-Syndrom) sind sehr selten und können durch hochauflösende Ultraschalluntersuchungen frühzeitig erkannt werden. Die Myasthenia gravis an sich stellt keine Indikation für eine Sectio caesarea dar. Die Entbindung sollte jedoch in einem Zentrum mit kooperierender Geburtshilfe, Neonatologie und Neurologie durchgeführt werden, da das peripartale und perinatale Risiko erhöht und nicht vorhersagbar ist. Die neonatale Myasthenie kann bei einem Fünftel der Neugeborenen myasthener Mütter auftreten und wird mit Pyridostigmin oder bei schweren Verläufen mittels Plasmapherese behandelt. Beispielhaft werden typische Fallkonstellationen für die individuelle Beratung und Betreuung von Myasthenie-Patientinnen mit Kinderwunsch oder bereits eingetretener Schwangerschaft präsentiert.

Abstract

Family planning issues and pregnancy are frequent concerns in the medical care of patients with myasthenia gravis since disease onset often coincides with this time period on life. Pregnancy, delivery and breastfeeding represent in fact special situations in this group of patients; however, they are not associated with higher risks of complications compared to normal population. Pregnancy should be planned in consultation with the treating neurologist so that disease remission can be achieved before the patient gets pregnant. During pregnancy, pyridostigmine and glucocorticosteroids may be used. Treatment with azathioprine, methotrexate, mycophenolate mofetil, cyclosporine A as well as tacrolimus should be terminated and if necessary changed to glucocorticosteroids or intravenous immunoglobulins. Under certain circumstances, azathioprine as well as cyclosporine A may be continued. Severe exacerbations can be treated with intravenous immunoglobulins and plasma exchange. Fetal deformities such as arthrogryposis multiplex congenita or the fetal acetylcholine receptor inactivation syndrome (FARIS) are very rare conditions and can be recognized in early pregnancy by high-definition ultrasound. Myasthenia gravis is no indication for cesarean section. However, delivery should be accomplished in a centre with collaborating obstetrics, neonatology and neurology. Approximately 20% of infants born to myasthenic mothers develop neonatal myasthenia and will be treated with pyridostigmine and in severe cases with plasma exchange. Here we discuss typical case constellations providing a basis for medical care and individual counseling of myasthenia gravis patients, either already pregnant or presenting with questions related to family planning.

Schlüsselwörter

Myasthenia Gravis - Schwangerschaft - Stillzeit - Geburt - Therapie

Keywords

Myasthenia Gravis - pregnancy - nursing - birth - therapy

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