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What is it?
Oromandibular Dystonia is a focal dystonia characterised by forceful contractions of the jaw and tongue, causing difficulty in opening and closing the mouth and often affecting chewing and speech.
Symptoms
Oromandibular may be associated with dystonia of the cervical muscles (cervical dystonia/spasmodic torticollis), eyelids (blepharospasm), or larynx (spasmodic dysphonia). When oromandibular is combined with blepharospasm, it may be referred to as Meige’s Syndrome named after Henry Meige, the French neurologist who first described the symptoms in detail in 1910.
The symptoms usually begin between the ages of 40 and 70 years old and appear to be more common in women than in men.
Oromandibular dystonia may be a continuous disorder that persists even during sleep, or it may be task-specific, occurring only during activities such as speaking or chewing.
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.
If oromandibular dystonia causes any type of impairment, it is because muscle contractions interfere with normal function. Features such as cognition, strength, and the senses, including vision and hearing are normal. While dystonia is not fatal, it is a chronic disorder, and prognosis is difficult to predict.
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.
Cases of inherited cranial dystonia have been reported, usually in conjunction with early-onset generalised dystonia which is associated with the DYT1 gene.
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 blepharospasm 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, 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
Currently there is no cure for dystonia, but treatments are available to help to ease the symptoms related to the disorder including spasms, pain, and disturbed postures. Working with your doctor, an individualized strategy for treatment can be developed.
The approach for treatment of dystonia is usually three-tiered: oral medications, botulinum toxin injections, and surgery. These therapies may be used alone or in combination. Complementary care may also have a role in the treatment management, depending on the form of dystonia, and supportive therapy may provide an important adjunct to medical treatment.
Medications
A multitude of drugs has been studied to determine benefit for people with oromandibular dystonia, but none appear to be uniformly effective.
About one-third of people’s symptoms improved when treated with oral medications such as Rivotril (clonazepam), Artane (benzhexol), diazepam (Valium), tetrabenazine, and Lioresal (baclofen), but the degree of improvement is usually unsatisfactory and at the expense of side effects.
Botulinum Toxin Injections
Botulinum toxin injections are most effective in jaw-closure dystonia, while treating jaw-opening dystonia is more challenging, benefiting fewer people. It is a therapeutic muscle-relaxing agent that helps reduce the uncontrollable muscular contractions associated with dystonia. It is injected into specific muscles where it acts to weaken muscle activity sufficiently to reduce a spasm but not enough to cause paralysis.
Dystonia in the jaw can affect various muscles. Botulinum toxin injections are most effective when the correct muscles are injected. If the jaw muscles are injected, the facial muscles should not be affected, but injections into the face muscles may affect expressions.
About 70 percent of people with oromandibular experience some reduction of spasm and improvement of chewing and speech after injection of botulinum toxin into the masseter, temporalis, and lateral pterygoid muscles.
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.
Surgery
The structure of the jaw is so complex that at this time denervation (cutting a nerve) surgery for oromandibular dystonia cannot be done.
Complementary Therapy
The use of sensory tricks may also be effective in dealing with oromandibular dystonia. Some of the most common “tricks” include touching the lips or chin, chewing gum, talking, biting on a toothpick, or putting a finger near an eye or underneath the chin to keep the jaw closed. Different sensory tricks work for different people, and if a person finds a sensory trick that works, it usually continues to work.
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. Dystonia Ireland has a support group in Dublin and it is our intention eventually to set up similar groups throughout Ireland. Sharing experiences at support group meetings offers encouragement, camaraderie, and the latest information about new treatments and medical advances.
With written permission, this information is reproduced from materials published and copyrighted by the Dystonia Medical Research Foundation, Chicago, IL, USA www.dystonia-foundation.org
