Management of oromandibular dystonia on a chorea acanthocytosis: a brief review of the literature and a clinical case

Cranio. 2016 Sep;34(5):332-7. doi: 10.1179/2151090315Y.0000000027. Epub 2016 Apr 11.


Background: Chorea acanthocytosis is an extremely rare neurodegenerative condition characterized by neuropsychiatric disturbances, movement disorders, neuropathy, seizures, and acanthocytosis. In this case report, the authors will present the management of the oromandibular movement disorders associated with this disease.

Case description: This case report describes the focal management of the severe orofacial manifestations associated with this condition. The therapeutic approach adopted to reduce the severe oromandibular movements, dysphagia, and the numerous oral ulcers was selective electromyography (EMG)-guided botulinum toxin application to the inferior head of the lateral pterygoid muscles and masseters. This would be applied to control severe and sudden oromandibular dystonia.

Results: Through this procedure, the authors were able to reduce these severe oral manifestations, which had a major impact on the patient's quality of life, and temporarily improve vital functions, such as mastication, deglutition, and speech articulation.

Conclusions: Electromyography-guided botulinum toxin application may be a useful tool in the multimodal management of this condition.

Keywords: Botulinum toxin; Chorea acanthocytosis; Electromyography; Movement disorders; Neurodegenerative diseases; Oromandibular dystonia.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Botulinum Toxins / administration & dosage*
  • Dystonia / diagnosis
  • Dystonia / therapy*
  • Electromyography* / drug effects
  • Female
  • Follow-Up Studies
  • Humans
  • Injections, Intramuscular
  • Mandibular Diseases / diagnosis
  • Mandibular Diseases / therapy*
  • Masseter Muscle / drug effects
  • Neuroacanthocytosis / diagnosis
  • Neuroacanthocytosis / therapy*
  • Oral Ulcer / diagnosis
  • Oral Ulcer / therapy
  • Pterygoid Muscles / drug effects


  • Botulinum Toxins