Breathing Capacity Can Be Restored in Duchenne Muscular Dystrophy

The respiratory system in patients with Duchenne muscular dystrophy (DMD) dysfunctions. The deficit can be restored by activating the brain.

Duchenne muscular dystrophy (DMD) patients have deficiencies in respiratory capacity and by activating the brain breathing can be restored in these patients, reveals a new study.

Duchenne muscular dystrophy (DMD) is a fatal genetic neuromuscular disease, which can lead to respiratory failure. Respiratory muscle dysfunction is recognized in DMD, however a thorough assessment of nervous system control of respiratory muscles is lacking.

Muscular Dystrophy / Duchenne Muscular Dystrophy - Overview
Overview about muscular dystrophy, a muscle disease characterized by muscle weakness and muscle wasting.

‘Breathing can be restored in patients with Duchenne muscular dystrophy (DMD) by activating the brain.’

The study was published in The Journal of Physiology.

Lead investigators David Burns and Ken O'Halloran at University College Cork, in conjunction with collaborator labs at the University of Calgary and Trinity College Dublin, performed experiments in mice lacking dystrophin, the muscle protein that malfunctions in DMD.

These mice have been shown to be a useful pre-clinical model of DMD as they demonstrate many of the hallmark features of respiratory muscle dysfunction in DMD patients.

In the young dystrophin-deficient mice, the respiratory control system was impaired on multiple levels. Importantly, the researchers found that the brain was compensating for this by increasing activation of the diaphragm muscle.

This study only offers a snapshot of the deficits and compensations at one time point in the progression of the disease. It is essential the findings from the study in the dystrophin-deficient mice are confirmed in human DMD patients before its potential applications in these patients can be considered.

Age Matters in Regenerating Muscle in Duchenne Muscular Dystrophy
A team of scientists have published details of how a class of drugs called HDACis drive muscle-cell regeneration in the early stages of dystrophic muscles, but fail to work in late stages.

David Burns, one of the lead investigators on the study, said:

'Further study of physiological neuromuscular mechanisms that compensate for the absence of dystrophin is needed. A better understanding may provide insight with potential applications to a range of neuromuscular diseases, beyond DMD.'

Facial Muscle Study Sheds Light on Duchenne Muscular Dystrophy
In patients with neuromuscular disorders, weakness is apparent in the orbicularis oculi, a group of facial muscles that control the eyelids.

Source-Eurekalert