Meige Syndrome: Causes, Symptoms, Diagnosis, and Treatment

By | 15/01/2019
neurological movement disorder

It’s a rare form of Dystonia, a movement disorder that affects the jaws, the tongue, and the eyes.

Dystonia refers to involuntary and irregular muscle contractions that result in movement disorders.

Meige Syndrome describes a neurological condition that consists of a combination of two types of Dystonia; Blepharospasm, and Oromandibular Dystonia.

Blepharospasm features involuntary eye blinking or eye closure due to muscle spasms or contractions.

Oromandibular Dystonia refers to forceful contractions of the lower face, jaw, and tongue.

By considering such concepts, some people define Meige Syndrome as “Cranio-facial Dystonia.”

This condition consists of involuntary and irregular contractions of the muscles responsible for eyelid opening, lower face, and the jaw.

Causes of Meige Syndrome

Medical experts still don’t know what the root cause of this condition is. Some speculate that there are multiple factors involved in the development of Meige Syndrome.

Researchers theorize that Meige Syndrome is the result of the combination of specific genetic and environmental factors.

Other people think a malfunction in the basal ganglia (a group of structures within the brain involved in the regulation of motor and learning functions) may play a relevant role in the development of this disorder.

Some doctors suggest that Meige Syndrome occurs due to some underlying conditions such as the ones listed here below:

  • Tardive Dyskinesia, a neurological disorder that features involuntary movements of the lower face, lips, and tongue.
  • Wilson disease, which is a rare inherited condition that consists of a copper build-up that usually affects the brain and the liver and results in muscle contractions.
  • Parkinson disease, it refers to a degenerative nervous system disorder that affects nerve cells and results in the impairment of motor functions.

Despite all of these theories, researchers are still looking for the exact cause of this neurological condition.

Symptoms

As stated above, Meige Syndrome features a combination of two forms of Dystonia.

The typical symptoms are frequent or forced blinking due to muscle spasms or contractions. That results in involuntary closure of the eyelids.

Sometimes these symptoms manifest in one eye, but more often than not, Meige Syndrome affects both eyes.

Patients are prone to experience eye irritation as a result of specific stimuli such as bright lights, fatigue, emotional tension, and depending on the environment, air pollution. Such factors may lead to abnormally dry eyes.

Meige Syndrome also includes involuntary and forceful contractions of the jaw and tongue, which makes it difficult for patients to close the mouth.

Some people may experience grinding of the teeth, displacement of the jaw, chin thrusting, and in some cases, repeated pursing of the lips.

Due to the muscle contractions of the tongue and throat, people will have difficulty swallowing solid food.

This condition could even lead to a gradual decline of facial muscle tone, and when the muscle spasms extend to the respiratory tract, patients will likely suffer from Dyspnea (shortness of breath).

Meige Syndrome worsens over time, and the muscle spasms or contractions will keep on happening more often if not treated.

Diagnosis

To determine if a patient has Meige Syndrome, medical experts may resort to clinical evaluation of external signs such as the presence of eye irritation, increased blinking frequency and spasms.

However, there is no particular test to make a diagnosis of this neurological condition. Still, neurologists can examine the patients by analyzing their medical history.

Treatment

There’s no specific cure for Meige Syndrome, which is why most doctors focus on alleviating the symptoms.

Around 33% of the cases of Meige Syndrome get treated with oral medications. However, more often than not, this treatment proves to be moderately efficient at best.

Some of the drugs used to treat this condition may include Clonazepam, Trihexyphenidyl, Diazepam, Baclofen, among others.

Several doctors regard Botulinum Toxin injections as the most effective treatment known so far. Injecting small amounts of this drug into the affected muscles helps to reduce the involuntary contractions.

Other people resort to Low-voltage Deep Brain Stimulation (DBS) because it produced long-lasting improvement in patients with Meige Syndrome. DBS disrupts abnormal patterns of brain activity, which impacts muscle activity.

Treatments that don’t rely on the use of drugs or other medical procedures may involve a series of activities known as sensory tricks. They consist of specific movements such as biting on a toothpick, chewing gums, or talking.

When it comes to this last element, some people found out that speech and swallowing therapy helps patients to lessen spasms, improve range of motion, and strengthen the unaffected muscles.

According to several experts, some patients with this condition have improved without treatment, but therapies and other techniques may speed up the process.