Volume 13
Number 1
Apr 2009
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Case Reports
M Fuchs, CW Hoedemaekers, JG van der Hoeven

Dystrophia myotonica is the most common hereditary disease of the neuromuscular system in adults and was first identified by Steinert, Batten and Gibb (1909). It is inherited as an autosomal dominant trait and is a clinical syndrome that affects skeletal muscle, the heart, and the neurological -, gastrointestinal- and endocrine systems. Several complications can occur during the course of the disease which often go unrecognised, and can lead to admission to the ICU. We describe the case history of a man who presented with respiratory failure due to aspiration after gastro-intestinal surgery, and who was diagnosed with dystrophia myotonica type 1 during a prolonged ICU stay.


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