A study of the facilitation of respiration in myotonic dystrophy

Dystrophia myotonica or myotonic dystrophy is a progressive neuromuscular disorder in which patients demonstrate an irregular respiratory pattern and are particularly subject to cardiopulmonary compromise. The aim of the present study was to investigate the effects of both proprioceptive neuromuscular facilitation (PNF) and staged basal expansion (SBE) breathing exercises in subjects with myotonic dystrophy in two different positions: high support sitting and left side-lying.

A randomized, double-blind study design was used. Seven non-congenital myotonic dystrophy subjects took part in the study. Six 'treatment' levels were applied to each subject: resting in high support sitting; resting in left side-lying; PNF of deep breathing in high support sitting; PNF of deep breathing in left side-lying; SBE in high support sitting and SBE in left side-lying. The outcome measures employed were arterial oxygen saturation (SpO2) and heart rate, as measured by oximetry and thoraco-abdominal motion (TAM), and respiratory rate, as measured by a pneumograph.

The PNF technique was found to be the main contributor to improvement in SpO2 for subjects with myotonic dystrophy, where a 2.2% increase was found in the high support sitting position and a 2.6% increase was found in the left side-lying position. There was an increase of between 377% and 556% in TAM during application of both treatment techniques, in either the high support sitting or left side-lying positions. Respiratory rate declined between 15% and 30% immediately after treatment application and heart rate dropped slightly by between 0.2% and 4.1%.

The present study provides objective evidence that application of these respiratory physiotherapy interventions elicits an improvement in respiratory function in subjects with myotonic dystrophy. Further research into the physiological effects of these techniques could explore the mechanisms responsible for improvement in respiratory indices.