Oromandibular

Oromandibular

What is it?
Oromandibular Dystonia is a focal dystonia characterised by forceful contractions of the face, jaw and/or tongue, causing difficulty in opening and closing the mouth and often affecting chewing and speech. Another word to describe dystonia of this kind is cranial dystonia. Cranial dystonia is a broad description for dystonia that affects any part of the head. Dystonia that affects the facial muscles and lips of musicians who play wind instruments is called embouchure dystonia. Dystonia that specially affects the tongue is called lingual dystonia. Oromandibular  dystonia  may be primary or secondary.

 

Terms used to describe oromandibular dystonia include: orofaciomandibular dystonia; orofacialbuccal dystonia; jaw dystonia. tongue dystonia (lingual dystonia); embouchure dystonia; cranial dystonia; adult onset focal dystonia. When oromandibular dystonia occurs with Blepharospasm, it may be referred to as Meige’s syndrome.

 

Symptoms
Oromandibular may be associated with dystonia of the neck muscles (cervical dystonia/spasmodic torticollis), eyelids (blepharospasm), or larynx (spasmodic dysphonia). The combination of upper and lower dystonia is sometimes called cranial-cervical dystonia. Sometimes symptoms of oromandibular dystonia are task-specific and occur only during activities such as speaking or chewing. Paradoxically, in some people,  activities like speaking and chewing reduce symptoms. Difficulty in swallowing is a common aspect of oromandibular dystonia if the jaw is affected, and spasms in the tongue can also make it difficult to swallow.

Drug-induced dystonia often manifests as symptoms in the facial muscles. Secondary oromandibular dystonia may persist during sleep.

Oromandibular dystonia symptoms usually begin later in life, between the ages of 40 and 70 years, and appear to be more common in women than in men.

 

Cause
Oromandibular dystonia is believed to be due to abnormal functioning of the basal ganglia, which are deep brain structures involved with the control of movement. The basal ganglia assist in initiating and regulating movement. What goes wrong in the basal ganglia is still unknown. An imbalance of dopamine, a neurotransmitter in the basal ganglia, may underlie several different forms of dystonia, but much more research needs to be done for a better understanding of the brain mechanisms involved with dystonia.

Oromandibular dystonia may be primary (meaning that is  the only apparent neurological disorder, with or without a family history) or be brought about by secondary causes such as drug exposure or disorders such as Wilson’s disease. Cases of inherited cranial dystonia have been reported, often in conjunction with DYT1 generalised dystonia.

Oromandibular dystonia may be secondary, or symptomatic, occurring in association with other disorders such as tardive dystonia, Wilson’s disease, Parkinson’s disease, and X-linked dystonia-parkinsonian syndrome.

 

Diagnosis
Diagnosis of oromandibular dystonia  is based on information from the affected individual and the physical and neurological examination. At this time, there is no test to confirm diagnosis of oromandibular dystonia and in most cases laboratory tests are normal.

Oromandibular dystonia should not be mistaken for Temporomandibular Joint Disease (TMJ), which is an arthritic condition, and not dystonia.

 

Treatment

Treatment for oromandibular dystonia must be highly customised  to the individual. A multitude of drugs has been studied to determine benefit for people with oromandibular dystonia. About one-third of people’s symptoms improved when treated with oral medications such as Klonopin (clonazepam), Artane (trihexyphenidyl), Diazepam (Valium), tetrabenazine, and Lioresal (baclofen).

Although the symptoms may vary from person to  person, approximately 70% of people with oromandibular dystonia experience some reduction of spasm and improvement of chewing and speech after injection of botulinum toxin into the masseter, temporalis and lateral pterygoid muscles. Botulinum toxin injections are most effective in jaw closure dystonia, while treating jaw opening dystonia may be more challenging. Botulinum toxin injections may also be an option for lingual dystonia. Side effects  such as swallowing difficulties, slurred speech, and excess weakness in injected muscles may occur, but these side effects are usually transient and well tolerated.

Oromandibular dystonia may respond surprisingly well to the use of sensory tricks to temporarily reduce symptoms. For example, gently touching the lips or chin, chewing gum, talking, biting on a toothpick, or placing a finger near an eye or underneath the chin may cause symptoms to subside temporarily. Different sensory tricks work for different people, and if a person finds a sensory trick that works, it usually continues to work.

Speech and swallowing therapy may lessen spasms, improve range of motion, strengthen unaffected muscles and facilitate  speech and swallowing. Regular relaxation practices may benefit overall well being.

 

Support
Dystonia and its emotional offshoots affect every aspect of a person’s life – how we think, the way we act, and how we cope. By educating yourself with information, you have taken the first step in dealing with dystonia.

Stress is an inevitable part of life, and although it clearly does not cause dystonia, it can aggravate dystonia symptoms. Stress-reduction programmes such as relaxation techniques, meditation, and journal writing may be beneficial.

Sometimes depression can be a byproduct of dystonia. Depression may aggravate symptoms and make them worse, but, often, treating depression can result in an improvement of dystonia. It is important to remember that depression is a disorder; it is treatable and not a reflection of one’s self.

Many people are experiencing similar symptoms. Reassurance from family, friends, and others who have dystonia is beneficial.   Sharing experiences at support group meetings offers encouragement, camaraderie, and the latest information about new treatments and medical advances.

 

Dystonia Ireland gratefully acknowledges  the Dystonia Medical Research Foundation for use of this information.