Meige Syndrome


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Meige Syndrome / Cranial Dystonia

Meige Syndrome (also known as Cranial Dystonia or Oral Facial Dystonia) and is actually a combination of two forms of dystoniaEye Dystonia (Blepharospasm) and Mouth, Tongue or Jaw Dystonia (Oromandibular).  Meige is named after Henry Meige, the French neurologist who first described the symptoms in detail in 1910. Symptoms usually begin between the ages of 40 and 70 years and appear to be more common in women than men. 

Cause

Blinking is regulated and co-ordinated in the base of the brain, in the basal ganglia, the midbrain, and/or the brainstem.  However, it is considered unlikely that a single defect in any of these areas is the primary cause of the condition.

Current medical research suggests that the condition is caused by a defect in a large network of brain cells.   It is possible that the condition is caused by an abnormality in a control centre in the network that triggers blinking.  This control centre is located in the basal ganglia, a group of nuclei in the brain associated with motor co-ordination.  The muscles that receive their impulses from this control centre are the Orbicularis oculi muscle (surrounding the eye), the Corrugator muscle (which regulates the frown), and the Procerus muscle (which causes the skin to wrinkle horizontally at the root of the nose and controls blinking).

The main sensory nerve in the face (Trigeminal nerve) transmits stimuli to the control centre in the basal ganglia.  When this centre is defective, its response to stimuli such as bright light, or corneal or eyelid irritation caused by wind, pain, emotion or stress, may be amplified.  As a result, descending impulses from the basal ganglia trigger the more pronounced blinking associated with the disorder.

In some cases the disorder is induced by medical drugs, notably those used to treat Parkinson’s disease.  If this cause of the condition is suspected, a reduced dose may alleviate the problem.

Diagnosis

The diagnosis is made on the basis of external signs, for example the presence of eye irritation, increased blinking frequency and spasms. There are no special tests or methods of examination that confirm the diagnosis, and there is no established system of classification to describe the progression of the disorder.

Treatment

Although no cure currently exists for dystonia, there are several treatment options available to alleviate the symptoms.  Botulinum Toxin injections in the facial musculature are considered the most effective treatment to date.  Although the effects are temporary, the intramuscular injections provide rapid symptom relief.  The majority of individuals with Blepharospasm report significant improvement after botulinum toxin treatment.  T

People with Meige Syndrome may find it beneficial to avoid stressors  that exacerbate the symptoms, such as bright sunlight or windy environments.  If sunlight triggers excessive blinking, dark sunglasses and a peaked cap or visor  may alleviate the problem.  Glasses also shield the eyes from wind.  Another advantage of wearing sunglasses (a blue tint may be easier on the eye) is that they make blinking less noticeable.  Artificial tear drops are a common treatment for dry eyes at this stage of the condition.

There are some sensory tricks that can help to alleviate the symptoms, for example chewing gum, whistling, a toothpick or a boiled sweet, singing when walking can help to keep the eyes open, reading aloud, knitting, dancing, pressing fingers against the side of the face (need to find a spot that suits you), plus,  any activity that involves looking down also proves helpful, i.e. cooking and gardening.

 

 

To read case studies of individuals with meige syndrome that have been featured in Dystoniamatters! click on the links below:

Sylvia Benham

Yvan Rioux

 

Last reviewed: October 2011

 

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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