MYOTONIC-DYSTROPHY IN PREGNANCY - A REPORT OF 2 CASES WITHIN ONE FAMILY

Myotonic dystrophy, also called the Curschmann-Steinert syndrome, is a n autosomal dominant inherited neuromuscular disorder characterized by progressive muscular dystrophy, muscle weakness and myotonia, which c an affect both mother and child. Complications may arise during pregna ncy, delivery, including anaesthetic problems, and in the neonatal per iod. During pregnancy hydramnion can be a first sign of the disease le ading to premature labor and also muscle weakness and myotonia can agg ravate complicating the course of delivery. The affected neonate may d isplay severe hypotonia, facial diplegia and respiratory distress. The clinical diagnosis can be confirmed by direct DNA analysis in serum a nd in chorionvillus biopsy material. In this case report two sisters w ith myotonic dystrophy are described, their pregnancies, deliveries an d the outcome of their affected babies. (C) 1997 Elsevier Science Irel and Ltd.