Chorea-Acanthocytosis with Feeding Dystonia
Chorea-acanthocytosis is the most frequent form of neuroacanthocytosis. In addition to more generalized chorea, it frequently causes orofacial dyskinesias associated with marked dysarthria, tongue dystonia, and dysphagia (see Chap. 72). Protrusion tongue dystonia is particularly common in ChAc which is absent when the mouth is at rest but occurs during orolingual manipulation of food. Forward protrusion movements of the tongue push food out of the mouth, thereby making chewing and swallowing very difficult. This lingual action dystonia is highly specific for ChAc in contrast to the more spontaneous tongue protrusions associated with other orofacial dyskinesias. Holding nonfood objects in the mouth such as toothpicks does not provoke tongue dystonia and may be used as a sensory trick to avoid involuntary jaw closure.
KeywordsDifferential Diagnosis Significant Weight Loss Frequent Form Tongue Protrusion Pharyngeal Phase
Chorea-acanthocytosis w. FD.mp4 (MP4 24,107KB)
This patient displays mild generalized chorea and tongue protrusion dystonia. While eating, he displays involuntary tongue movements accompanied by involuntary jaw opening. Forceful involuntary tongue protrusion is followed by voluntary retraction of the tongue. The patient tries to bypass the oral phase by pressing his lips together to close his mouth while chewing, by extending his head if the tongue protrudes, or by extending his neck so that the food falls onto the posterior portion of the tongue.