Meige Syndrome: Causes, Symptoms, Treatment

Meige syndrome is a rare form of dystonia – a nervous system disorder in which you have frequently forced spasms of the lower facial muscles, eyes, tongue, and jaw. The frequently forced spasms may feel like a stabbing pain or an electrical shock. 

As this condition is said to be a rare form of dystonia, Meige syndrome is also called segmental cranial dystonia. Dystonia is a body movement disorder that causes excessive muscle contractions.

Dystonia can affect one or more body parts, sometimes it can affect your whole body. This is an umbrella term for all movement malfunctions, but the conditions under this group have different causes, treatments, progression, and symptoms.

Meige syndrome is a condition where muscle contractions can be forceful and painful. Also, the muscles around your eyes may spasm, and close – a condition called blepharospasm.

When you experience this event, the condition is specifically called idiopathic blepharospasm -oromandibular dystonia syndrome.

Symptoms of Meige Syndrome

Symptoms of Meige syndrome, its severity, and progression vary from person to person.

Most commonly, this condition is seen in middle-aged people and usually seen by a combination of oromandibular dystonia and blepharospasm.

Blepharospasm is a condition that includes spasms of the eyelids and forced abnormal blinking.

You may experience eye irritation due to multiple factors like fatigue, bright lights, and wind. As the frequency of muscle contractions and spasms increases, it may be harder for you to keep your eyes open properly.

Oromandibular, on the other hand, is a condition that causes forceful and involuntary contractions of the tongue and jaw, making it difficult for you to open or close your mouth. There are also cases where patients experienced clenching of the teeth, repeated pursing of the lips, chin thrusting, displacement of the jaw, and grimacing. 

Patients with Meige syndrome may also experience spasms of the throat or tongue, resulting in difficulty swallowing and tongue protruding from the mouth. There are cases where the neck, legs, arms, and other parts of a patient’s body are affected.

Causes of Meige Syndrome

The exact cause of Meige syndrome is still unknown. 

However, doctors theorise that the cause of this condition may be multifactorial, for example, interactions of environmental factors and certain genes. 

The basal ganglia or the malfunctioning of a region of the brain may play a key role in the development of Meige syndrome.

The basal ganglia are located at the base of the brain. It is a group of subcortical nuclei, of varied origin, in the brains of vertebrates. The basal ganglia are responsible for your motor and learning functions.

The exact cause for the problems in the basal ganglia in patients with Meige syndrome is still unknown.

Risk Factors of Meige Syndrome

Although the causes for this condition are still known, it is said that women are more likely to suffer from Meige syndrome than men. 

It tends to develop in middle age, approximately around or after the age of 40. But there are cases where patients aged 60 or more had this condition. 

Rarely, this condition can be seen in people younger than 40 years. 

It is said that certain health conditions may also increase the risk of getting this condition:

• Tardive dyskinesia: This is a neurological syndrome caused due to long-term use of neuroleptic drugs. Generally, neuroleptic drugs are prescribed for psychiatric disorders, some gastrointestinal and neurological disorders. 
• Wilson disease: It is a genetic condition that causes copper to build up in the body. Wilson disease may cause liver disease. 
• Parkinson’s disease: It is a neurological movement disorder. The common symptoms that people experience in Parkinson’s disease are difficulty in coordination and balance, tremors, unsteady walk, slowness of movements, and stiff muscles. It is said that Parkinson’s disease has no cure, but with proper medication and treatment, you can improve symptoms.

Diagnosing Meige Syndrome

There are no lab tests that can rule out Meige syndrome. 

A neurologist will conduct a careful examination and look at your medical history. A neurologist may also ask about the symptoms of development and family medical history. 

If the symptoms experienced are similar to Meige syndrome, your doctor may diagnose it and prescribe treatment. 

Ensure you get medical attention when you experience the symptoms of this condition.

Treatment of Meige Syndrome

Looking at your condition and symptoms, your doctor may suggest the following treatment for Meige syndrome:

• Medications: To start the treatment, your doctor will first prescribe a few medicines. Your doctor may prescribe Baclofen, Clonazepam, Diazepam, and Trihexyphenidyl. 
• Injections of botulinum toxin: It is a preferred treatment for this condition. Your doctor will inject botulinum toxin into the muscles around your jaws and eyes to paralyze these muscles temporarily to reduce the involuntary spasms. Your doctor may suggest Botox injections every three months.
• Speech and swallowing therapy: This therapy may reduce the severity of your spasms. Talk to your doctor before using this therapy. Know if speech and swallowing therapy is suitable for you.
• Deep Brain Stimulation (DBS): It is a low-voltage way to sustain the long-lasting improvement of dystonia symptoms in patients with Meige syndrome. Deep Brain Stimulation includes placing a thin metal electrode into a particular area of your brain and attaching it to an implanted computerised pulse generator. DBS works by changing the abnormal patterns of brain activity.
• Stereotactic brain surgery: Your doctor may order stereotactic brain surgery if you have a severe case that does not respond to other treatments. However, more research is needed to determine if this surgery is beneficial and safe for people with the condition.

Lifestyle Changes and Home Remedies

You may try the following tips to ease the symptoms of Meige syndrome:

• Hot and cold compress: You may use a cold pack or a hot water bag on the involved/inflamed muscle to ease the pain. 
• Looking down: It is reported that looking down can help some sufferers while looking up can trigger the symptoms.
• Wear sunglasses: Sunglasses can prevent some spasms triggered by wind and sun. 

However, it is suggested to ask your doctor once before using these tips. Apart from these tips, your doctor may give a few other suggestions that can be suitable for your particular condition.

Learn more about brain and nervous system disorders, here.