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Chorea-Acanthocytosis with Feeding Dystonia

  • Roongroj Bhidayasiri
  • Daniel Tarsy
Chapter
Part of the Current Clinical Neurology book series (CCNEU)

Abstract

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.

Keywords

Differential Diagnosis Significant Weight Loss Frequent Form Tongue Protrusion Pharyngeal Phase 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Supplementary material

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.

References

  1. 1.
    Bader B, Walker RH, Vogel M, et al. Tongue protrusion and feeding dystonia: a hallmark of chorea-acanthocytosis. Mov Disord. 2010;25:127–9.PubMedCrossRefGoogle Scholar
  2. 2.
    Kanjanasut N, Jagota P, Bhidayasiri R. The first case report of neuroacanthocytosis in Thailand: utilization of a peripheral blood smear technique for detecting acanthocytes. Clin Neurol Neurosurg. 2010;112:541–3.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  • Roongroj Bhidayasiri
    • 1
    • 2
  • Daniel Tarsy
    • 3
  1. 1.Chulalongkorn Center of Excellence on Parkinson’s Disease and Related DisordersChulalongkorn University HospitalBangkokThailand
  2. 2.Department of NeurologyDavid Geffen School of Medicine at UCLALos AngelesUSA
  3. 3.Department of NeurologyHarvard Medical School Beth Israel Deaconess Medical CenterBostonUSA

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