Article

Duchenne muscular dystrophy (DMD) is a rapidly progressive neuromuscular
disorder causing weakness of the skeletal, respiratory, cardiac and oropharyngeal
muscles with up to one third of young men reporting difficulty swallowing
(dysphagia). Recent studies on dysphagia in DMD clarify the pathophysiology of
swallowing disorders and offer new tools for its assessment but little guidance
is available for its management. This paper aims to provide a step-by-step
algorithm to facilitate clinical decisions regarding dysphagia management in this
patient population. METHODS: This algorithm is based on 30 years of clinical
experience with DMD in a specialised Centre for Neuromuscular Disorders
(Inkendaal Rehabilitation Hospital, Belgium) and is supported by literature where
available. RESULTS: Dysphagia can worsen the condition of ageing patients with
DMD. Apart from the difficulties of chewing and oral fragmentation of the food
bolus, dysphagia is rather a consequence of an impairment in the pharyngeal phase
of swallowing. By contrast with central neurologic disorders, dysphagia in DMD
accompanies solid rather than liquid intake. Symptoms of dysphagia may not be
clinically evident; however laryngeal food penetration, accumulation of food
residue in the pharynx and/or true laryngeal food aspiration may occur. The
prevalence of these issues in DMD is likely underestimated. CONCLUSIONS: There is
little guidance available for clinicians to manage dysphagia and improve feeding
for young men with DMD. This report aims to provide a clinical algorithm to
facilitate the diagnosis of dysphagia, to identify the symptoms and to propose
practical recommendations to treat dysphagia in the adult DMD population.
Implications for Rehabilitation Little guidance is available for the management
of dysphagia in Duchenne dystrophy. Food can penetrate the vestibule, accumulate
as residue or cause aspiration. We propose recommendations and an algorithm to
guide management of dysphagia. Penetration/residue accumulation: prohibit solid
food and promote intake of fluids. Aspiration: if cough augmentation techniques
are ineffective, consider tracheostomy.

Langue : ANGLAIS