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Uterine activity in myotonia dystrophica. Case report

The patient was a 28-year-old woman in her fourth pregnancy. She has had myotonia dystrophica since the age of 4 years diagnosed by electromyographic (EMG) investigations and abnormal serum creatinine phosphokinase levels. One of her sisters also has the disease but not her parents or two other siblings. Her first pregnancy resulted in a growthretarded (1800 g) macerated stillbirth at term 6 years ago. The second pregnancy terminated in spontaneous abortion at 3 months gestation. Her third pregnancy was complicated by massive polyhydramnios with preterm labour at 32 weeks when 2.5 litres of amniotic fluid were drained with membrane rupture. The baby was born by caesarean section for fetal distress under 0.5% bupivicaine lumbar epidural anaesthesia. The baby had an Apgar score of 1 at 1 min, 2 at 5 min, and died 24 h later. Within hours of surgery her temperature rose to 39°C and was controlled by tepid sponging. The possibility of malignant hyperpyrexia was entertained but was not confirmed due to the lack of facilities to perform caffeine sensitivity test. In this, her fourth pregnancy, she was booked at 20 weeks and had eight subsequent antenatal visits. Initial ultrasound confirmed the period of gestation and excluded major fetal abnormality. Serial ultrasound examination for growth of the fetus was satisfactory and there was no evidence of excess amniotic fluid. She complained of generalized weakness during this pregnancy but there was no paraesthesia, sensory defect or visual problems. She had no frontal balding but had poor facial expression and symmetrical wasting of both arms and legs. Cardiovascular and respiratory systems were normal. Investigations revealed normal ECG, haemoglobin, phosphate, calcium, urea potassium, sodium and chloride levels. Serum