Abstract

GUIMARAES, A.S.; SUAZO, G.I.  and  NAGAHASHI MARIE, S.K.. Orofacial myotonic phenomenon in Steiner´s myotonic dystrophy patients. Av Odontoestomatol [online]. 2010, vol.26, n.3, pp.139-142. ISSN 2340-3152.

Steinert´s myotonic dystrophy (DM1) is a hereditary disease, characterized by multisystem disorders associated with muscle dysfunction. One of the most frequent clinical manifestations is the myotonic phenomenon (MP), which produces a slowdown of post-isometric muscle relaxation. In this study, the presence of MP in the opening and closing oral movements, movements of the tongue, and other perioral muscles in 50 adult patients with DM1 molecular diagnostics was analyzed. We found MP in 32 of the 50 patients analyzed with DM1 (64%), among which 21 cases (65.6%) were affected with impaired movements of the tongue (highest prevalence), followed by the oral opening movements, which affected 14 patients, and oral closing movements presented in 7 patients. Only 2 patients expressed the symptom of oral opening and closing movements, and in 1 patient, buccinator muscle activity was observed. This study demonstrates that the muscles in the orofacial region are frequently affected in patients with DM1, and hence, the clinician should recognize the MP in these patients.

Keywords : Steinert´s myotonic dystrophy; muscular dystrophy; myotonia.

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