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[Acute respiratory failure caused by myotonic dystrophy]
I A Hynås1, T Ganes, A Naalsund
1Rikshospitalet 0027 Oslo. inger.anette.hynas@rikshospitalet.no
Background:
Respiratory insufficiency is not always caused by pulmonary diseases or diseases involving the pulmonary vasculature.
Material And Method:
We present a case history of a 29-year-old patient with acute respiratory insufficiency following a gynaecological operation.
Results:
Ventilation/perfusion scintigraphy and spiral-CT of thorax excluded pulmonary embolism. Chest X-ray demonstrated elevated diaphragm and slight right-sided atelectatic changes. Spirometry revealed a restrictive ventilatory capacity, which explained the patient's hypoxaemia. Phrenic nerve conduction studies were normal on both sides. Electromyography showed severe myotonic discharges in all examined muscles including intercostal musculature and the diaphragm.
Interpretation:
Myotonic dystrophy was the cause of respiratory failure in this patient. The case demonstrates that respiratory failure may be caused by neuromuscular diseases, hence the importance of adequate neurophysiological investigation in such patients.