Facial structure and functional findings in patients with progressive muscular dystrophy (Duchenne)

The investigation of 15 patients who have a progressive muscular dystrophy of the Duchenne type (PMD) shows the affection of various groups of muscles of the stomatognathic system, studied by means of electromyography and mechanical force measurements. We observed the following deviations in patients with PMD; transversal overdevelopment and sagittal shortening of the dental arch; reduction of overbite and overjet; sagittal underdevelopment of the cranial, maxillary and mandibular base; retrusion of incisors; concave profile; increase in bizygomatic width; and a difference in time between the attack on the musculi orbicularis oris and that on masticatory muscles. In our study, the activity of the jaw muscles diminished 2 years earlier compared with the perioral muscles. This, and also the enlargement of the hypotonic tongue, causes a transversal expansion of skull and dental arches. This expansion was strongly pronounced in the lower jaw; we invariably observed a posterior crossbite. In contrast to the transversal overdevelopment, we observed a sagittal underdevelopment of skeletal and dental parameters, as well as a retrusion of the incisors. The results of surface electromyography showed the affection of the masseter muscle in patients who had PMD. By means of the Fast-Fourier transformation, we observed a displacement of the median frequency as compared with the lower frequency range.