Oromandibular dystonia

Joseph Jankovic MD (Dr. Jankovic, Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine, received research funding from Allergan, Allon, Ceregene, Chelsea, EMD Serono, Impax, Ipsen, Lundbeck, Medtronic, Merz, and Teva, and compensation for his services as a consultant or an advisory committee member by Allergan, Auspex, EMD Serono, Lundbeck, Merz, Neurocrine Biosciences, and Teva.)
Originally released December 1, 1998; last updated March 6, 2017; expires March 6, 2020

This article includes discussion of oromandibular dystonia, bruxism, and cranial dystonia. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Oromandibular dystonia is a form of focal dystonia manifested by jaw closure often associated with clenching of the jaws and grinding of teeth (bruxism) and may lead to temporal-mandibular joint syndrome. Other forms of oromandibular dystonia include jaw opening and deviation. The author discusses the cause of this dystonia and its management, focusing on botulinum toxin injections, the treatment of choice.

Key points

 

• Oromandibular dystonia is a form of focal dystonia involving the jaw, tongue, and lower face.

 

• There are 4 types of oromandibular dystonia: jaw closure (associated with trismus and bruxism), jaw opening, jaw deviation, and mixed.

 

• Botulinum toxin injection is considered the first line of treatment for oromandibular dystonia.

Historical note and terminology

Horatio Wood, a United States neurologist, described involuntary facial and oromandibular movements in 1887, 23 years before the French neurologist Henry Meige reported a series of patients who exhibited dystonic contractions of facial and neck muscles (Pandey and Sharma 2017). The term “dystonia” was coined in 1911 by Oppenheim, a German clinician. Although the term "torsion dystonia" has been used in the literature, not every patient with dystonia has "torsion," and hence the simple term “dystonia” is currently preferred. Discoveries of genetic markers in some primary and secondary forms of dystonia have contributed to the development of newer nosology and classification of dystonia (Fahn et al 1998). When no etiology can be identified, the dystonia is referred to as primary dystonia. Primary dystonia can be either sporadic or inherited, and is not associated with any cognitive, pyramidal, cerebellar, or sensory abnormalities. The term "dystonia plus" is used when an associated neurologic abnormality exists, such as parkinsonism, dementia, corticospinal tract signs, and other neurologic disturbances besides dystonia. By definition, all cases of "dystonia plus" are secondary, although the etiology (eg, neuroleptics, Wilson disease, neuroacanthocytosis, trauma) may not always be obvious.

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