Myasthenia gravis with polyhydramnios in the fetus of an asymptomatic mother

Abstract

Transient myasthenia gravis was observed in an infant whose mother had no signs of neuro-muscular disease. The case presented with severe polyhdramnios at 31 weeks. Sonographic and invasive work-up showed only an absence of fetal swallowing. At birth, the infant had severe muscle weakness and respiratory distress. He had high titres of anti-acetylcholine receptor (anti-AChR) antibodies of maternal origin. Anti-AChR antibodies were also found in stored samples of fetal serum and amniotic fluid. To our knowledge, this is the first case of neonatal myasthenia gravis for which neither past nor present evidence of disease could be obtained in the mother. Anti-AChR antibody testing should be considered in cases of unexplained polyhydramnios.

Transiente neonatale Myasthenia gravis

“…Umgekehrt scheint das MyasthenieRisiko für Neugeborene von Müttern, die zum Zeitpunkt der Geburt in Remission sind, geringer zu sein [12,17,21,26,29,35,36]. Unklar ist bisher aber, warum nicht jedes Neugeborene einer Myasthenie-Patientin erkrankt bzw.…”

“…Als mögliche Erklärung hierfür und gleichzeitig auch für die fehlende eindeutige Korrelation zwischen Schwere und Dauer der mütterlichen und kindlichen Erkrankung wird in der Literatur einerseits das Vorhandensein eines weiteren Faktors (wie z. B. α-Fetoprotein) neben den AChR-AK postuliert, der entweder den Ausbruch der Erkrankung beim Neugeborenen triggert oder die für den Ausbruch der Erkrankung verantwortlichen Antikörper intrauterin bis zum Geburtstermin inhibiert, und andererseits die Neusynthese von AChR-AK durch das Neugeborene selbst diskutiert [12,17,19,23,29,30,35,36].…”

“…Bei schweren Verlaufsformen entstehen bereits intrauterin Gelenkkontrakturen (Arthrogryposis multiplex congenita) [26,31,33,36].Auch kann wegen der eingeschränkten fetalen Schlucktätigkeit ein Polyhydramnion entstehen [22,26,27,36].…”

Congenital Myasthenia Gravis in Smooth-Haired Miniature Dachshund Dogs

“…16 Rare cases of neonatal MG have been reported where mothers are clinically asymptomatic. 17,18 Human patients with congenital MG with episodic apnea secondary to mutation of choline acetyl transferase gene have been reported to show improvement with time; however, are they are generally not clinically normal. 1 Acquired MG has been reported in only 1 puppy 5 and never in littermates younger than 3 months of age.…”

Myasthenia Gravis and Pregnancy

“…In these cases, lack of fetal movement causes joint contractures and paralysis of the fetal diaphragm results in the development of pulmonary hypoplasia [13]. In extreme cases, fetal arthrogryposis multiplex congenita may be observed in pregnant myasthenic patients.…”