Vol. 125 No.
4 April 2018
The American Academy of Oral Medicine Clinical Practice
Statement: Oromandibular dystonia
Katherine France, DMD, MBE, and
Eric T. Stoopler, DMD, FDS RCSEd, FDS RCSEng, FDS RCPS(Glasg), FDTFEd
The American Academy of Oral Medicine (AAOM) affirms
that oromandibular dystonia (also referred to as orofacial
dystonia) is a movement disorder causing paroxysmal or
sustained contraction of the affected facial muscles.
Oromandibular dystonia may occur alone or as a part
of a segmental or generalized dystonia and may be id-
iopathic or secondary to previous medication or drug use,
trauma, or neurodegenerative disease. The AAOM also
affirms that patients with suspected oromandibular dys-
tonia should be referred to the appropriate oral and/or
medical health care provider(s) for comprehensive eval-
uation and management of the condition. Management
of oromandibular dystonia may include conservative
therapies, medications, botulinum toxin injections, or pe-
ripheral or central surgical approaches.
This Clinical Practice Statement was developed as an
educational tool on the basis of expert consensus of the
American Academy of Oral Medicine (AAOM) leader-
ship. Readers are encouraged to consider the
recommendations in the context of their specific clini-
cal situation and to consult, when appropriate, other
sources of clinical, scientific, or regulatory information
before making a treatment decision.
Originators: Katherine France, DMD, MBE, Eric T.
Stoopler, DMD, FDS RCSEd, FDS RCSEng, FDS
RCPS(Glasg), FDTFEd
Review: AAOM Education Committee
Approval: AAOM Executive Committee
Adopted: December 8, 2017
PURPOSE
The American Academy of Oral Medicine (AAOM)
affirms that oromandibular dystonia (also referred to as
orofacial dystonia) is a movement disorder associated with
uncontrolled contraction of the affected muscles, leading
to abnormal posture and functional difficulties, includ-
ing psychosocial withdrawal. The AAOM also affirms
This Statement was developed as an educational tool on the basis of
expert opinion. Readers are encouraged to consider the recommen-
dations in the context of their specific clinical situation and to consult,
when appropriate, other sources of clinical, scientific, or regulatory
information before making a treatment decision.
Department of Oral Medicine, University of Pennsylvania School of
Dental Medicine, Philadelphia, PA, USA.
Received for publication Dec 8, 2017; returned for revision Jan 3, 2018;
accepted for publication Jan 20, 2018.
2212-4403/$ - see front matter
https://doi.org/10.1016/j.oooo.2018.01.023
that patients with suspected oromandibular dystonia should
be referred to the appropriate oral and/or medical health
care provider(s) for comprehensive evaluation and man-
agement of the condition.
METHODS
This statement is based on a review of the current dental
and medical literature related to oromandibular dystonias.
PubMed and MEDLINE searches were conducted using
the terms “dystonia,” “oral,” “oromandibular dystonia,”
“bruxism,” “orofacial dystonia,” and “Meige’s syn-
drome.” Clinical studies, narrative reviews, case series,
and case reports provided the basis for this statement.
Expert opinions and best current practices were relied
upon when clinical evidence was not available.
BACKGROUND
Dystonia is a type of movement disorder characterized
by sustained, involuntary muscle contractions, often
causing abnormal postures.1 It may include focal, seg-
mental, or generalized muscle overactivity, causing
patterned or twisting movements worsened by function.2
These disorders may be of primary etiology or occur sec-
ondary to infection, trauma, or medications and may
exhibit spontaneous remission, usually within 5 years.3
Dystonia is thought to be underdiagnosed because of its
similarity to other movement disorders, including dys-
kinesia, hemifacial spasm, muscular effects of
temporomandibular disorders, and bruxism. Primary focal
dystonias frequently appear in adulthood, and an esti-
mated 60% of cases are limited to the head and neck
region.3-5
Oromandibular dystonia (OMD; also referred to as
orofacial dystonia) is a focal dystonia defined as uncon-
trolled spasm of the facial musculature with variable cause,
clinical course, affected muscles, and severity.4 OMD most
frequently affects the muscles of mastication or the tongue
muscles, causing involuntary jaw opening, closing, de-
viation, or tongue thrust, among others.4-6 In some cases,
OMD may occur concomitantly with dystonia of other
areas, as in the case of Meige syndrome, which con-
sists of dystonia of the ocular and facial muscles.2,7,8 OMD
is more common among women, with incidence esti-
mates around 6.9 per 100,000 persons in the United
States.5 The pathophysiology of OMD is unknown, but
OMD may be caused by dysfunction in the basal ganglia,
hyperactivity of motor nerves related to signaling
283
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284 France and Stoopler
pathways, reduced spinal cord and brainstem inhibi-
tion, aberrant dopamine signaling, or reduced
neuroplasticity, with the presence of OMD being corre-
lated with a larger putamen and mosaic nerve loss.3-5,9
OMD may also lead to dysphagia, dysarthria, diffi-
culty breathing, weight loss, damage to teeth and oral
mucosa, temporomandibular disorders, pain, social with-
drawal, and impaired sleep quality.3,5 OMD is diagnosed
through a thorough medical and symptom history, clin-
ical and neurologic examinations, and electromyography.
Underlying pathology acting as a source of the condi-
tion may be ruled out by using magnetic resonance
imaging of the brain and spinal cord.10 On the basis of
this evaluation, OMD is characterized as primary (idio-
pathic) or secondary, with one common subclass of
primary dystonia being task-specific dystonia, a focal
subset in which signs are directly related to a repeated
action, such as playing a musical instrument.11,12 Al-
though many cases of OMD are idiopathic and can be
managed clinically with standard protocols, secondary
dystonias may be caused by medications, including neu-
rologic agents (dopamine antagonists, typical
antipsychotics, or mood stabilizers) or other drugs,
including cefixime13,14 and illicit drugs (e.g., metham-
phetamine and cocaine), 4 or by neurodegenerative
diseases, such as Parkinson disease or Huntington
disease.1,5 They may also be caused by central injury, such
as traumatic brain injury, stroke, or brain tumor.15 On the
basis of limited data, peripheral injury resulting from
dental treatment, causing local injury or change to oc-
clusion, has been implicated as a cause of secondary
dystonia.16
OMD has no known cure and is most effectively treated
conservatively through physical and speech therapy,
massage, biofeedback, acupuncture, and fabrication of
occlusal appliances.1,6,11,17 Sensory tricks, including pres-
sure against the dystonic muscle (Le Geste Antagoniste),
posturing, speaking, and chewing, have been found to
have beneficial long-term effects.2,5 Although not defini-
tively proven, occlusal appliance use is thought to work
similarly by counteracting the dystonic contraction of af-
fected muscles. Although many medications have been
proposed to treat OMD, none works reliably; pharma-
cologic therapy may be beneficial in only one-third of
patients. Medical therapies that have been tested include
anticholinergic agents, dopamine-depleting agents and
dopamine agonists, anticonvulsants, muscle relaxants,
antipsychotics, benzodiazepines, baclofen, levodopa, and
zolpidem, among others. Anticholinergics, including
benztropine mesylate, currently boast the highest re-
ported success rate.1,3,5,6,8,10,11,18,19
The use of botulinum toxin (BTX) injections is well
established in the treatment of laryngeal and cervical dys-
tonia and has also been studied in OMD, where most
commonly botulinum toxin type A (Botox, Allergan Phar-
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April 2018
maceuticals Ireland, Dublin, Ireland) is used.20 BTX
injection is hypothesized to influence the pathophysiol-
ogy of dystonia and currently represents the first line of
treatment for OMD.8,20,21 Injections into the affected muscle
may be guided by electromyography or ultrasonogra-
phy. Benefits to injection peak, on average, after 4 weeks,
with injections often repeated at 4-month intervals. BTX
is generally considered safe, and although 90% of pa-
tients respond initially to this therapy, development of
tolerance may occur over time, decreasing the efficacy
of this treatment.1,3,5,8,11,20 Although few serious adverse
reactions to BTX have been reported in patients with dys-
tonia, this treatment may cause influenza-like symptoms
as well as xerostomia, dysphagia, or weakness of prox-
imal muscles because of the local spread of the toxin.5
Peripheral and central surgeries have also been pro-
posed in OMD. Peripheral surgeries, including myotomy,
myectomy, rhizotomy, neurotomy, and ramisectomy, have
low success rate and may worsen symptoms.5,11 Central
approaches, including deep brain stimulation and
pallidotomy, have shown promise, as indicated by the
limited evidence available.1,3,5,7,8,22
CLINICAL PRACTICE STATEMENT
1. The AAOM recognizes that:
A. Oromandibular dystonia (also referred to as
orofacial dystonia) is a rare movement disorder
affecting the head and neck region that may present
a challenge in diagnosis and management.
B. Oromandibular dystonia may commonly affect
tongue or facial musculature and cause recurrent
uncontrolled spasm(s).
C. Oromandibular dystonia may occur alone or in
conjunction with other dystonias, including in the
cases of Meige syndrome or generalized dystonia.
D. Oromandibular dystonia may cause secondary
functional and psychological effects, including dys-
phagia, dysarthria, temporomandibular disorders,
and weight loss, which significantly affect quality
of life.
E. Oromandibular dystonia may be idiopathic or may
occur secondary to medications, illicit drug use,
neurodegenerative disease, or central or periph-
eral injury.
i. Task-specific dystonia presents one subset of
primary OMD in which a repeated motion may
lead to development of a dystonia.
2. The AAOM thus encourages oral health care provid-
ers to:
A. Identify patients with signs and symptoms that may
suggest a diagnosis of OMD.
B. Obtain a thorough history and clinical examina-
tion on all patients suspected of having OMD,
including:
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Volume 125, Number 4
i. Previous medication use.
ii. Medical history, particularly as it applies to po-
tentially related diagnoses.
C. Refer patients to the appropriate oral and/or
medical health care provider(s) for further eval-
uation and management of patients with suspected
OMD.
i. Evaluation for OMD by the appropriate oral
and/or medical health care provider(s) includes:
a. Electromyography
b. Magnetic resonance imaging of the brain and
spinal cord
c. Use of dystonia rating scales (e.g., Fahn-
Marsden Dystonia Rating Scale, Global
Dystonia Rating Scale) may be considered, but
the diagnostic utility of these scales remains
uncertain at this time.
ii. Management of OMD by the appropriate oral
and/or medical health care provider(s) may
consist of:
a. Physical and speech therapy
b. Massage
c. Biofeedback
d. Acupuncture
e. Fabrication of occlusal appliances
f. Pharmacologic therapy (e.g., anticholinergics)
g. BTX injections (e.g., BTX type A)
h. Surgery (peripheral and/or central)
REFERENCES
1. Jinnah HA, Factor SA. Diagnosis and treatment of dystonia. Neurol
Clin. 2016;33:77-100.
2. Albanese A. How many dystonias? Clinical evidence. Front Neurol.
2017;8:18.
3. Evatt ML, Freeman A, Factor S. Adult-onset dystonia. Handb Clin
Neurol. 2011;100:1841-1872.
4. Balasubramaniam R, Ram S. Orofacial movement disorders. Oral
Maxillofac Surg Clin North Am. 2008;20:273-285.
5. Karp BI, Alter K. Botulinum toxin treatment of blepharospasm,
orofacial/oromandibular dystonia, and hemifacial spasm. Semin
Neurol. 2016;36:84-91.
6. Martinez-Ramirez D, Paz-Gomez V, Rodriguez RL. Response to
zolpidem in oromandibular dystonia: a case report. Parkinson-
ism Relat Disord. 2015;21:154-155.
7. Lyons MK, Birch BD, Hillman RA, Boucher OK, Evidente VGH.
Long-term follow-up of deep brain stimulation for Meige syn-
drome. Neurosurg Focus. 2010;29:E5.
ORIGINAL ARTICLE
France and Stoopler 285
8. Pandey S, Sharma S. Meige’s syndrome: history, epidemiology,
clinical features, pathogenesis and treatment. J Neurol Sci. 2017;
372:162-170.
9. Factor S, Barron KD. Mosaic pattern of gliosis in the neostria-
tum of a North American man with craniocervical dystonia and
Parkinsonism. Mov Disord. 1997;12:783-799.
10. Clark GT, Ram S. Four oral motor disorders: bruxism, dystonia,
dyskinesia and drug-induced dystonic extrapyramidal reactions.
Dent Clin North Am. 2007;51:225-243.
11. Balasubramaniam R, Rasmussen J, Carlson LW, van Sickles JE,
Okeson JP. Oromandibular dystonia revisited: a review and a unique
case. J Oral Maxillofac Surg. 2008;66:379-386.
12. Moura RC, de Carvalho Aguiar PM, Bortz G, Ferraz HB. Clini-
cal and epidemiological correlates of task-specific dystonia in a
large cohort of Brazilian music players. Front Neurol. 2017;8:
73.
13. Fratto G, Manzon L. Use of psychotropic drugs and associated
dental diseases. Int J Psychiatry Med. 2014;48:185-197.
14. Mondet L, Radoubé F, Gras V, Masmoudi K. Cefixime-induced
oromandibular dystonia in an adult: a case report. Curr Drug Saf.
2017;doi:10.2174/1574886312666170310095320. Epub ahead of
print.
15. Fasano A, Tinazzi M. Functional facial and tongue movement dis-
orders. Handb Clin Neurol. 2016;31:353-365.
16. Shankhla C, Lai EC, Jankovic J. Peripherally induced oromandibular
dystonia. J Neurol Neurosurg Psychiatry. 1998;65:722-728.
17. Albanese A, Sorbo FD, Comella C, et al. Dystonia rating scales:
critique and recommendations. Mov Disord. 2013;28:874-883.
18. Miguel R, Mendonça MD, Barbosa R, et al. Tetrabenazine in treat-
ment of hyperkinetic movement disorders: an observational study.
Ther Adv Neurol Disord. 2017;10:81-90.
19. Park JE, Srivanitchapoom P, Maurer CW, et al. Lack of efficacy
of levetiracetam in oromandibular and cranial dystonia. Acta Neurol
Scand. 2017;136:103-108.
20. Del Sorbo F, Albanese A. Botulinum neurotoxins for the treat-
ment of focal dystonias: review of rating tools used in clinical trials.
Toxicon. 2015;107:89-97.
21. Awan KH. The therapeutic usage of botulinum toxin (Botox) in
non-cosmetic head and neck conditions: an evidence based review.
Saudi Pharm J. 2017;25:18-24.
22. Minkin K, Gabrovski K, Dimova P, et al. Bilateral pallidotomy
for Meige syndrome. Acta Neurochir (Wien). 2017;159:1359-
1363.
Reprint requests:
Eric T. Stoopler, DMD, FDS RCSEd, FDS RCSEng, FDS
RCPS(Glasg), FDTFEd
University of Pennsylvania School of Dental Medicine
240 South 40th Street
Philadelphia, PA 19104
USA
Ets@upenn.edu