Meige Syndrome

Overview

What is Meige syndrome?

French neurologist Henri Meige first described this syndrome in medical journals in 1910. Meige syndrome is a rare neurological disorder in which a person has involuntary and irregular contractions of the muscles that move the eyelids, lower face, jaw and tongue.

Meige syndrome is one of a family of disorders known as dystonia. The group of disorders that fall under the umbrella of dystonia are all movement malfunctions — but they have different causes, symptoms, progression and treatments. Meige syndrome is a specific form that’s called segmental cranial dystonia.

These muscle contractions can be forceful and often painful. Also, muscles around your eyes may spasm and close, which is called blepharospasm. When this happens, it’s known specifically as idiopathic blepharospasm-oromandibular dystonia syndrome.

Who is most likely to have Meige syndrome?

Women are more likely than men to get Meige syndrome. Meige syndrome tends to appear in middle age, around the age of 40. But it can appear in those age 60+. In rare cases, Meige syndrome has been reported in people younger than 40.

Is Meige syndrome the same thing as Meige disease?

No, they are different. Meige syndrome is a form of dystonia and Meige disease is an inherited lymphatic condition that causes fluid buildup in the lower limbs (arms and legs). The confusion is understandable because both were named after the French neurologist, Henri Meige.

Is Meige syndrome the same thing as Brueghel’s syndrome?

No. Brueghel’s syndrome is a form of jaw dystonia. Brueghel’s syndrome doesn’t include eyelid spasms (blepharospasm).

Is Meige syndrome the same thing as a hemifacial spasm?

No. Meige syndrome is a form of dystonia, while hemifacial spasms are caused by a compressed or irritated facial nerve. Hemifacial spasms affect all of the muscles on only one side of the face and are usually not painful.

Can Meige syndrome result from another disease?

Yes. Meige syndrome is often associated with:

  • Parkinson's disease. Parkinson’s disease is a neurological movement disorder. Common symptoms include tremor, slowness of movement, stiff muscles, unsteady walk and balance and coordination problems. There is no cure for the disease. Most patients can maintain a good quality of life with medications. In some patients, surgery can help improve symptoms.
  • Tardive dyskinesia. Tardive dyskinesia is a neurological syndrome caused by the long-term use of neuroleptic drugs. Neuroleptic drugs are generally prescribed for psychiatric disorders, as well as for some gastrointestinal and neurological disorders.
  • Wilson disease. Wilson disease is a genetic disease that causes copper to accumulate in the body. This condition may cause liver disease.

Symptoms and Causes

What are the symptoms of Meige syndrome?

Blepharospasm, or forced or frequent blinking or twitching, sometimes happens when you’re exposed to bright lights, fatigue, wind or air pollution or tension. Blepharospasm may first affect only one of your eyes, but it will affect both eventually.

Over time, these muscle spasms and contractions may happen more often, and cause your eyelids to close, making it harder to keep your eyes open. Those with Meige syndrome can essentially become blind since their eyes are closed, though their vision is unaffected. You may also have dry eyes. The initial symptom can feel like “sand in the eye."

Oromandibular dystonia, another symptom of Meige syndrome, is a term that means you have forced contractions of your jaw and tongue, making it difficult to open or close your mouth. A clenched mouth and teeth grinding (bruxism) can also occur. Other jaw-related symptoms you might experience include:

  • Facial grimacing.
  • Frowning.
  • Thrusting of the chin (involuntary chin jerks, usually while talking or eating).
  • Displaced jaw (twitching of the jaw).
  • Jaw pain.
  • Headaches.

Spasms can also occur in your tongue, throat and respiratory tract. Spasms in the respiratory tract can make breathing difficult. Spasms of the tongue can make eating and swallowing solid foods challenging.

What causes Meige syndrome?

The cause of Meige syndrome is unknown. Several genetic and environmental factors may be involved. Some researchers have suggested that a fault in a region of the brain known as the basal ganglia — the portion of the brain that controls movement — may be involved in Meige syndrome. It’s possible that it’s a combination of both. Sometimes you’re born with a genetic marker for a disorder and then something in your environment — like bright sunlight, wind or stress — may trigger it.

Diagnosis and Tests

How is Meige syndrome diagnosed?

There are no tests that can diagnose Meige syndrome. A diagnosis is made by a neurologist after a careful examination and detailed medical history. If your symptoms are similar to those of someone who suffers from Meige syndrome, your provider may diagnose it and see how you respond to prescribed treatments.

Management and Treatment

How is Meige syndrome treated?

Your provider may offer several treatments for Meige syndrome to help soothe your spasms.

Medications can be the first line of defense for Meige syndrome. Pills used to treat Meige syndrome include Clonazepam (Klonopin®), Trihexyphenidyl (Artane®), Diazepam (Valium®) and Baclofen (Lioresal®). Success with oral medications is limited, however.

Injections of botulinum toxin (Botox®) are the preferred treatment for Meige syndrome. Botulinum toxin is injected into the muscles around the eye and jaw to temporarily paralyze these muscles to reduce the involuntary spasms. Injections are usually given every three months. About 70% of patients report a reduction in symptoms after Botox injections.

Deep Brain Stimulation is a low-voltage way to produce sustained and long-lasting improvement of dystonia symptoms in some patients with Meige syndrome. DBS involves placing a thin metal electrode into a specific area of the brain and attaching it to an implanted computerized pulse generator. Think of the pulse generator as a pacemaker for the brain. Settings are programmed by a doctor during office visits and can be adjusted to optimally control symptoms. DBS works by disrupting abnormal patterns of brain activity.

Speech and swallowing therapy may reduce the severity of your spasms. Talk to your doctor to see if a referral to a therapist is right for you.

Stereotactic brain surgery may be an option if you have a severe case that doesn’t respond to other treatments. However, more research is needed to determine if this surgery is safe and effective for people with Meige syndrome.

Things you can try on your own that may help:

Chewing gum has been reported to help with symptoms.

Wearing sunglasses may help since some spasms are triggered by sun and wind.

Looking down has been reported to help some sufferers, while looking up may trigger it.

A note from Cleveland Clinic:

If you suffer from symptoms similar to those of Meige syndrome, see your doctor and describe your condition. Your doctor can refer you to specialists who can help you make the lifestyle changes and get the treatments necessary to make your symptoms less frequent and more manageable.

Last reviewed by a Cleveland Clinic medical professional on 06/30/2020.

References

  • National Organization for Rare Disorders. Meige Syndrome. (https://rarediseases.org/rare-diseases/meige-syndrome/) Accessed 5/21/2020.
  • National Institutes of Health. Meige Syndrome. (https://rarediseases.info.nih.gov/diseases/7008/meige-syndrome) Accessed 5/21/2020.
  • National Library of Medicine. Meige Syndrome. (https://www.ncbi.nlm.nih.gov/books/NBK513358/) Accessed 5/21/2020.
  • LeDoux MS. Meige syndrome: what’s in a name? Parkinsonism Relat Disord 2009; 15: 483-489.
  • Czyz CN, Burns JA, Petrie TP, et al. Long-term botulinum toxin treatment of benign essential blepharospasm, hemifacial spasm, and Meige syndrome. Am J Ophthalmol 2013; 156: 173-177.
  • Reese R, Gruber D, Schoenecker T, et al. Long-term clinical outcome in meige syndrome treated with internal pallidum deep brain stimulation. Mov Disord 2011; 26:691-698.
  • Sako W, Morigaki R, Mizobuchi Y, Tsuzuki T, Ima H, Ushio Y, Nagahiro S, Kaji R, Goto S. Bilateral pallidal deep brain stimulation in primary Meige syndrome. Parkinsonism Relat Disord 2011; 17: 123-125.

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