Quantitative and qualitative deterioration in skeletal muscles and nutritional considerations during critical illness in a patient with myotonic dystrophy

Background

An optimal nutritional strategy has not been established for the patients with muscle atrophy. Moreover, acute critical illness causes drastic changes in skeletal muscles, complicating nutritional treatment. Here, we present a case of myotonic dystrophy suffered acute respiratory distress syndrome (ARDS). We aimed to describe the changes in skeletal muscle as well as nutrition therapy.

Methods

A female in her 30s with myotonic dystrophy received mechanical ventilation for severe ARDS. Her respiratory status improved by day 15 of admission, but she developed severe weakness. We evaluated the cross-sectional area and mean computed tomography (CT) value of the psoas muscle at the level of the third lumber vertebra using abdominal CT scans performed on days 1 and 15. We also performed bioelectrical impedance analyses (BIA) on days 37 and 44. The patient received enteral nutrition from day 4, which was increased to administer 21.1 kcal/kg of energy and 1.05 g/kg of protein per day as the maintenance dose.

Results

The cross-sectional area remarkably decreased from 8.59 cm² on day 1–7.39 cm² on day 15. The CT values decreased from 47.2 HU to 13.6 HU. BIA performed during the recovery phase showed impaired muscle quality (phase angle of 2.6 or 2.9°). The muscle volume was preserved with maintenance doses of enteral nutrition.

Conclusion

Acute muscle volume loss and deteriorating muscle quality was observed during acute critical illness in a patient with myotonic dystrophy. The patient received a standard dose of energy and protein, and the muscle volume was preserved.