Meige Syndrome - Diagnosis and Treatment

Introduction:

Meige syndrome is a rare disorder that causes involuntary movements of muscles of the jaw, tongue, face, and around the eyes. It is otherwise called Brueghel syndrome or segmental cranial dystonia. A French neurologist, Henry Meige, first investigated facial muscle contractions (spasms). Later, Dr. George Paulson found abnormal muscle spasms in the jaw, face, tongue, and around the eyes, and he proposed the name ‘Meige syndrome’ in 1972.

Meige syndrome is a nervous system disorder involving blepharospasm (uncontrolled spasm of eyelid muscles) and oromandibular dystonia (forceful muscle contractions in the face, tongue, and jaw).

Meige syndrome occurs in individuals between 30 and 70 years of age. Estrogen receptors in women make them more susceptible to Meige syndrome than men.

How Does One Get Meige Syndrome?

Depending on these causative factors, it is classified as follows:

• Primary Meige Syndrome occurs due to alterations in specific genes (p.Gly213Ser).

• Secondary Meige Syndrome happens due to factors such as:

1. Medications like antiemetics and antidepressants increase dopamine activity, thus leading to improper muscle control.

2. Disorders like Parkinson's disease and Wilson disease (an inherited disorder that causes the accumulation of too much copper in the body) are linked with the occurrence of Meige syndrome.

3. Head injury, brain infection, kernicterus (brain damage), and other brain lesions predispose to Meige syndrome.

What Are the Symptoms of Meige Syndrome?

The clinical features differ from one to another. The muscle spasms can be moderate to severe. It includes:

• Forceful blinking of eyes. It is triggered by bright light, stress, depression, or environmental pollution.

• Repeated muscle spasm decreases the space between the eyelids or closes the eye. Individuals may find it challenging to keep their eyes open.

• Dry eyes.

• Difficulty in opening and closing the mouth.

• Repeated actions of chewing, grinding teeth or bruxism, lip pursing, and chin thrusting.

• Eating and swallowing problems.

• The defective muscle movements are initially confined to the face, jaw, tongue, and eyes and later progress to the muscles of the neck, respiratory tract, arms, and legs; it depends on age, any head injury, and the female gender.

• In addition, the syndrome causes spasms of the throat, tongue, and respiratory system. Eating and swallowing become difficult with tongue spasms and breathing difficulties due to the respiratory tract.

What Can Be Done to Alleviate the Symptoms?

Certain patients do the following to reduce the severity of symptoms.

• Walking.

• Pulling the upper eyelid.

• Relaxing or talking.

• Blowing the cheeks and yawning.

• Coldwater exposure.

How Is Meige Syndrome Diagnosed?

There is no particular diagnostic method to evaluate Meige syndrome. However, the following test is done to rule out other disorders.

• Magnetic resonance imaging (MRI) test of the brain detects any lesions, injury, or block of arteries.

• Serum copper level is measured to confirm the diagnosis of Wilson's disease (abnormal copper accumulation in the body). It occurs in association with Meige syndrome.

• Drug screening involves testing blood or urine to evaluate the presence of certain drugs that increase the risk of Meige syndrome.

• Facial electromyography measures muscle activity during contraction. The involuntary movements of various muscles at one time are detected and are used to differentiate Meige syndrome from other facial spasms.

What Is the Treatment of Meige Syndrome?

• Pharmacological Therapy:

1. Drugs like dopamine antagonists (Tiapride, Tetrabenazine), Benzodiazepine, and Baclofen. Decrease dopamine levels and control muscular activity.

2. Eszopiclone and Nitrazepam suppress eyelid spasms. However, the drugs control the spasm for a certain period only.

3. Botulinum toxin (BOTOX) injections are suggested for patients who do not respond well to medications. It restricts (paralyzes) the movements of eyelid muscles for three to four months. Then, the injection is repeated, but long-term use has many side effects. The body produces antibodies against botulinum and becomes resistant to it. It also worsens the repetitive muscular contractions and weakens the surrounding muscles.

• Deep Brain Stimulation: It is the procedure of sending signals to the electrodes placed in specific areas of the brain that control muscular activity. Globus pallidus is a structure in the basal ganglia of the brain. It functions to provide smooth and controlled muscular movements. Its function deteriorates in patients with Meige syndrome. Involuntary actions are controlled by sending signals to the electrodes placed in the globus pallidus. The specialist provides therapies that improve speech and swallowing to control muscle spasms.

• Lifestyle Modifications:

1. Use sunglasses to avoid extreme sun and wind that trigger involuntary movements.

2. The use of chewing gums relieves specific symptoms.

3. Avoid triggering factors.

4. Try specific actions like blowing cheeks and exposure to cold water that alleviate the symptoms.

Which Disorders Are Similar to Meige Syndrome?

• Wilson's Disease: It is characterized by excessive accumulation of copper, especially in the brain, liver, and eyes. It is an inherited disorder, and the symptoms develop between six years and 45 years of age. The clinical features include jaundice, abdominal swelling, fatigue, tremors, walking and speech difficulties, anxiety, and thinking impairment.

• Temporomandibular Joint Disorder: It involves the dysfunction of the jaw joint. It is caused by trauma, infections, or excessive straining of muscles involved in chewing and speech. The characteristic features are pain in the joint around the ears that radiates to the neck and back and difficulty in chewing, opening, and closing the mouth. A clicking sound is also heard in jaw movements.

• Tardive Dyskinesia: It causes stiff and uncontrollable movements of the face and other body parts. It occurs due to prolonged use of neuroleptic drugs suggested for managing psychological issues. It causes repetitive movements like blinking eyes, sucking, and waving of arms.

Are Meige Syndrome and Meige Disease the Same?

Meige disease involves abnormal fluid build-up in the lower legs due to improper lymphatic system functioning. It is an inherited disorder that produces swelling of the feet and ankles that progress to the legs and knees. On the other hand, Meige syndrome is a rare neurological disorder that causes forceful and involuntary contraction of the jaw muscles and also the muscles around the eyes.

Conclusion:

The rare neurological disorders that cause various involuntary and repetitive muscle movements lead to muscle weakness and interfere with the individual's regular activity. The condition often gets misdiagnosed for other similar conditions. Also, the syndrome itself is a differential diagnosis for conversion disorders, such as Myasthenia gravis and multiple sclerosis. Therefore, a multi-specialty approach is critical in diagnosing such disorders at the earliest and providing medication and therapy to improve the standard of living.