[High resolution manometry study of pharyngeal function in patients with myotonic dystrophy].

BACKGROUND

Patients with myotonic dystrophy (MD) are known to suffer from oropharyngeal dysphagia and esophageal motility disorders, which are often the cause of aspiration pneumonia. So far only little is known about the pharyngeal contractility and the function of the upper esophageal sphincter in these patients, in particular only few data are available for manometric investigations allowing assessment of the pharyngeal pressure build-up during swallowing. The aim of this study was to collect such data in patients with MD using high resolution manometry.

METHOD

In two patients with MD high resolution manometry studies were performed during swallowing and phonation to determine pressure-dependent parameters. The results were compared with normal values from healthy subjects.

RESULTS

In both patients a reduced pressure in the entire pharynx during swallowing was determined. The duration of the contraction in the velopharynx and tongue base region was shortened. The structural course of the swallowing process and the opening and closing functions of the upper esophageal sphincter were regular. During realization of closed vowels a reduced pressure build-up in the velopharyngeal region was observed.

CONCLUSION

The force of contraction and the associated pharyngeal pressure build-up during swallowing were reduced resulting in an incomplete clearing of the pharynx. Beside myopathic disorders, neuromuscular disorders also have to be considered. The functional course of the swallowing process and the swallowing pattern was retained. The reduced pressure build-up in the velopharyngeal region can be considered as the cause for rhinophonia. To evaluate the pharyngeal function in patients with MD, high resolution manometry is a useful tool for assessing the pharyngeal function besides the basic diagnostics.