Myotonic Dystrophy
Physiology
- Myopathy
- Hypoventilation leads to hypoxia/ acidosis (with resultant pulmonary vasoconstriction)
- Diaphragm is more susceptible to fatigue in the myotonic dystrophies
Diagnosis
- Respiratory muscle dyskinesia (better performance during rhythmic than static efforts)
- Abnormal expiratory muscle activity
- Prolongation of diaphragmatic activity during expiration elicited by expiratory resistive loads
Clinical
- Pulmonary HTN/Cor pulmonale
References
