Meige Syndrome

What is Meige syndrome?

Meige syndrome is a rare involuntary movement condition that affects your brain and nerves (neurological condition). It causes muscle spasms you can’t control. It may make your jaw twitch or force you to close your eyes. These muscle spasms can be painful.

Meige syndrome usually affects muscles in your:

• Lower face.
• Jaw.
• Tongue.
• Eyes.

Meige syndrome is a type of dystonia. Dystonia is a group of conditions that cause uncontrollable muscle movements. Healthcare providers sometimes call Meige syndrome, segmental cranial dystonia or idiopathic blepharospasm-oromandibular dystonia syndrome.

Is Meige syndrome rare?

Yes, Meige syndrome is a rare form of dystonia.

What are the symptoms of Meige syndrome?

Meige syndrome affects how you move your face and jaw. You might move your face without meaning to or being able to control it. The most common symptoms include:

• Facial grimacing.
• Frowning.
• Thrusting your chin (involuntary chin jerks, usually while talking or eating).
• Displaced jaw (jaw twitching).
• Jaw pain (shock or stabbing feeling).
• Difficulty opening or closing your mouth.
• A clenched mouth.
• Grinding teeth (bruxism).

You may experience spasms in your tongue, throat and respiratory tract. Tongue spasms can make eating and swallowing solid foods challenging. Spasms in your respiratory tract can make breathing difficult. Call 911 (or your local emergency services number) if you have trouble breathing.

Meige syndrome symptoms that affect your eyes include:

• Frequent or uncontrolled blinking.
• Eye pain.
• Difficulty keeping your eyes open.
• Dry eyes or a “sand in your eyes” feeling.

Early symptoms may only affect one of your eyes, but it might eventually affect both eyes. The symptoms may happen more often as you age. This can lead to difficulty seeing, as you’re unable to keep your eyes open but there’s no change to how your eyes function.

What causes Meige syndrome?

The cause of Meige syndrome is unknown. Experts think it could be a combination of genetic factors (unpredictable changes to your DNA that happen during fetal development) and environmental factors (triggers).

Some researchers think Meige syndrome affects the basal ganglia region in your brain. This part of your brain regulates your movement.

What triggers Meige syndrome symptoms?

Certain factors in your environment can make Meige syndrome symptoms worse. These are known as triggers, including:

• Bright lights.
• Feeling tired.
• Stress.
• Wind.
• Air pollution.

Is Meige syndrome caused by other conditions?

Meige syndrome can happen alongside (or along with) other conditions like:

• Parkinson's disease. Parkinson’s disease is a neurological movement disorder. Common symptoms include tremors, slowness of movement, stiff muscles, unsteady walking, and balance and coordination problems.
• Tardive dyskinesia. Tardive dyskinesia is a neurological condition caused by the long-term use of certain medications prescribed for psychiatric conditions, as well as for some gastrointestinal and neurological conditions.
• Wilson disease. Wilson disease is a genetic condition that causes copper to accumulate in your body. It can cause liver disease and abnormal involuntary movement.

What are the risk factors for Meige syndrome?

Meige syndrome can affect anyone, but it’s more likely to affect women than men.

Most people with Meige syndrome are diagnosed between the ages of 40 and 70, but it can develop at any age.

What are the complications of Meige syndrome?

Meige syndrome can cause the following complications:

• Headaches.
• Difficulty seeing (unable to open eyes).
• Malnutrition.
• Depression.

How is Meige syndrome diagnosed?

A healthcare provider will diagnose Meige syndrome after a physical exam, a neurological exam and testing (like blood or imaging tests) to rule out similar conditions. There isn’t a specific test available to diagnose Meige syndrome. Your healthcare provider will make a diagnosis by the presentation of your symptoms.

How is Meige syndrome treated?

Your provider will suggest treatments to relieve Meige syndrome symptoms or slow their progression (how quickly they get more severe). Some common Meige syndrome treatments include:

• Medications. Common medications that treat Meige syndrome include clonazepam (Klonopin®), trihexyphenidyl (Artane®), diazepam (Valium®) and baclofen (Lioresal®).
• Botulinum toxin (Botox®) injections. A healthcare provider will inject botulinum toxin into the muscles around your eye and jaw to temporarily weaken these muscles. This will reduce involuntary spasms. You may need injections every three months or when your healthcare provider recommends it.
• Deep brain stimulation. Deep brain stimulation (DBS) works by disrupting abnormal patterns of brain activity. A healthcare provider will place a thin metal electrode into your brain and attach it to an implanted computerized pulse generator. Think of the pulse generator as a pacemaker for your brain. Your healthcare provider will program this device during office visits and adjust it to help you manage your symptoms.
• Speech and swallowing therapy. Speech therapy can help strengthen the muscles in your jaw to reduce the spasm severity.

Can I treat Meige syndrome at home?

Some people with Meige syndrome find sensory tricks they can do at home helpful in reducing their symptoms. Sensory tricks are movements that distract the part of your brain that causes symptoms of Meige syndrome. Sensory tricks may include:

• Chewing gum.
• Biting a toothpick.
• Touching your lips or chin.
• Looking down.

In addition, you can avoid environmental triggers like bright lights or wind by wearing sunglasses or avoiding going outside when it’s windy.

Are there side effects of the treatment?

Talk to your healthcare provider about the side effects of treatment. Each type of medication may have side effects you should look out for, like drowsiness, shortness of breath or dry mouth.

Botulinum toxin injections may cause pain, swelling or bruising at the injection site or too much weakness.

Can Meige syndrome be prevented?

There aren’t any known ways to prevent Meige syndrome. You can reduce your risk of triggering your symptoms by avoiding things that make your symptoms worse, like bright lights or wind.

What can I expect if I have Meige syndrome?

Meige syndrome can affect your lifestyle and make it difficult for you to do routine things like enjoying the sunshine or riding in a vehicle with the windows down. Some people feel uncomfortable participating in social activities because they’re self-conscious about how muscle spasms affect their appearance. Many people with Meige syndrome find comfort in speaking with a mental health professional. This is also important because your symptoms may become more challenging to manage with time.

While there’s no cure for Meige syndrome, treatment can help you get back to the activities you enjoy.

What is the life expectancy for Meige syndrome?

Meige syndrome doesn’t directly affect your life expectancy. The condition isn’t fatal. Meige syndrome, however, may impact your quality of life. Talk to your healthcare provider about treatment options to help you manage your symptoms.

When should I see a healthcare provider?

Talk to your healthcare provider if you experience side effects after treatment or if your symptoms get worse after treatment. They can help find a treatment option that’s right for you and your symptoms.

In addition, if your symptoms make it difficult for you to eat, let your healthcare provider know as soon as possible to prevent malnutrition.

When should I go to the ER?

Call 911 or your local emergency services number if you have trouble breathing.

What questions should I ask my doctor?

• Do I have Meige syndrome or another form of dystonia?
• What type of treatment do you recommend?
• Are there side effects of the treatment?
• Are there clinical trials or new forms of treatment available?
• My symptoms are affecting my self-esteem. Should I speak with a mental health professional?

What is the difference between Meige syndrome and other movement conditions?

Meige syndrome is a form of dystonia that affects your movement. Other conditions that affect your movement could include:

• Jaw opening dystonia. This is a form of jaw dystonia. Jaw opening dystonia doesn’t affect your eyelids (blepharospasm).
• Hemifacial spasms. Hemifacial spasms affect all the muscles on only one side of your face. They’re usually not painful. Some people are found to have a compressed facial nerve on MRI of their brain.

Is Meige syndrome the same as Meige disease?

No. The conditions are different. They’re easy to confuse because they were both named after the French neurologist Henri Meige. Meige disease is an inherited lymphatic condition that causes fluid buildup in your limbs (arms and legs).