Unexpectedly prolonged weaning from mechanical ventilation in a patient with previously undiagnosed dystrophia myotonica type 1

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Netharland Journal of Critical Care

2009, Volume 13, Issue 2 : 84-87

Article

Unexpectedly prolonged weaning from mechanical ventilation in a patient with previously undiagnosed dystrophia myotonica type 1

Fuchs M.

Hoedemaekers C.W.

der Hoeven J.G.

Abstract

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. Copyright © 2009, Nederlandse Vereniging voor Intensive Care. All Rights Reserved.

Keywords

Morbus steinert

Respiratory failure

Weaning

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