Oromandibular dystonia


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Oromandibular dystonia

This page contains information about oromandibular dystonia (dystonia of the face, jaw or tongue ) for medical professionals. For information on oromandibular dystonia for patients and carers please click here.

Oromandibular dystonia is characterised by forceful contractions of the face, jaw or tongue, which can cause difficulty opening or closing the mouth, often affecting chewing and speech. Oromandibular dystonia is a neurological movement disorder and should be diagnosed and treated by a neurologist specialising in movement disorders or an Ear, Nose and Throat (ENT) surgeon.

Classification

Where torsion dystonia is the only clinical sign (apart from tremor) and there is no identifiable exogenous cause or other inherited or degenerative disease, oromandibular dystonia is classified as a primary pure dystonia. Symptoms of primary oromandibular dystonia usually begin later in life, between the ages of 40 and 70 years so it is classified as a late-onset dystonia. It is more common in women than men.

Oromandibular dystonia is a focal dystonia which means it affects only one region of the body. However, in some cases it is part of a dystonia affecting two or more body regions in which case the dystonia is classified as a multifocal or segmental dystonia. When oromandibular occurs together with blepharospasm, this is called Meige’s syndrome.

Oromandibular dystonia can also appear as a symptom of tardive dystonia. Tardive dystonia is a secondary dystonia, which means the dystonia has an identifiable exogenous cause - in this case, exposure to drugs. Tardive dystonia is usually multifocal and its onset can be at any age.

Symptoms

The symptoms of oromandibular dystonia are strange movements of the face and mouth. In some but not all cases, eating and swallowing can be difficult. 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.

Diagnosis

Oromandibular dystonia is often misdiagnosed by GPs. Conditions that it is commonly mistaken for include damage to the jaw, tic, bruxism (teeth grinding), dental problem, orthopaedic problem or a psychological problem. 

Indications for a GP that the problem may be oromandibular dystonia rather one of these other conditions include:

  • No damage shows up on x-rays
  • Does not clear up over time
  • Movement still present when patient unaware of being observed

Treatment

Although the symptoms of oromandibular dystonia may vary from person to person, many people experience some reduction of spasm and improvement of chewing and speech through regular botulinum toxin injections. Injections into the muscles that move the mouth can be difficult, as a very precise dose needs to be given to avoid weakening the muscle too much and some muscles can be difficult to inject. Because of these difficulties muscles are usually injected using electromyography (EMG).

A few different drugs can help reduce the severity of symptoms in people with oromandibular dystonia. Although some people get great benefit from such drugs, they are not effective in everyone, and some people experience side effects.

Oromandibular dystonia may respond to the use of sensory tricks to temporarily reduce symptoms. For example, gently touching the lips or chin, chewing gum, talking or placing a finger near an eye or underneath the chin may cause symptoms to subside temporarily. Different sensory tricks work for different people.

Speech and language therapy

A number of dystonias can affect speech. Where speech difficulties occur referral should be made to a speech and language therapist (SALT). Patients with  oromandibular dystonia with articulation difficulties can be given mouth and swallowing exercises to help them reduce the risk of choking by chewing and swallowing safely.

Wuthin the NHS there are very few SALT posts who have experience of dystonia, so not everyone who may need to see one is referred. However, if a health professional is concerned about a patient’s speech and swallowing issues they should always seek the advice of a SALT.

Dietary support

A number of types of dystonia can affect nutrition. Dysphagia (difficulty swallowing) can arise from oromandibular when it can be difficult to chew or move food around the mouth to prepare it for swallowing.

Dystonia can also lead to an inadequate diet:

  • Those who have excessive movements may also find it almost impossible to keep still whilst eating, making hand to mouth feeding very difficult. As a result, finger foods and feeding cups are often used but these may not provide enough nutrition.
  • Texture modified diets (e.g. puree diets) may be recommended due to dysphagia and can have negative impact on nutritional status due to their poor nutritional content.

Nutrition can be monitored using a screening tool (e.g. MUST) during home/clinic visits or inpatient admissions. A referral to the dietitian should be made if weight loss has occurred or if malnutrition is a concern. A dietetic referral is also required when dysphagia is present. The dietitian can suggest appropriate use of texture modified diets, food fortification and nutritional supplement drinks (e.g. Build Up™, Complan™, Ensure Plus™ and Fortisip™) to supplement diet.

 

Last reviewed March 2012

Disclaimer
The Dystonia Society provides the information on this page as general information only. It is not intended to provide instruction and you should not rely on this information to determine diagnosis, prognosis or a course of treatment. It should not be used in place of a professional consultation with a doctor.
The Dystonia Society is not responsible for the consequences of your decisions resulting from the use of this information, including, but not limited to, your choosing to seek or not to seek professional medical care, or from choosing or not choosing specific treatment based on the information. You should not disregard the advice of your physician or other qualified health care provider because of any information you receive from us. If you have any health care questions, please consult the relevant medical practitioner.

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