Palatal Myoclonus Associated with Orofacial Buccal Dystonia

ABSTRACT

Palatal myoclonus is a rare condition in which there are rhythmic jerky movements of the soft palate and sometimes of the other muscles innervated by the brainstem A particularly annoying symptom is a rhythmic clicking sound in the ear due to the opening and closing of the Eustachian tube. Orofacial buccal dystonia is a focal dystonia with sustained spasms of the masticatory, facial or lingual muscles. The frequent symptoms of this disease have mainly been reported to be involuntary and possibly painful jaw opening, closing, deflecting and retruding, or a combination of the above. However, the subtle and unnoticeable involuntary movement of multiple facial muscles, which might be an infrequent symptom of orofacial buccal dystonia, makes this disease hard to diagnose. Understanding the functional orofacial anatomy that is responsible for the clinical signs and symptoms is necessary for making a proper diagnosis. Here we report on a rare case of palatal myoclonus that was associated with orofacial buccal dystonia, and such a case has not been previously reported. We describe the diagnostic approach and excellent treatment results after Botulinum toxin A (Dysport) injection and proper counseling.