Meige Syndrome

What Meige syndrome is

Meige syndrome is named after the French neurologist Henri Meige, who described it in 1910. It is a segmental dystonia: involuntary muscle contractions affecting two or more neighboring body regions, in this case the eyes and the lower face. It is caused by abnormal signaling in brain circuits that control movement, including the basal ganglia, rather than by a problem in the muscles or nerves of the face.

Many people first develop blepharospasm, with spasms spreading to the lower face over months to years. Some start with jaw or mouth symptoms.

Symptoms

Eye symptoms (blepharospasm)

• excessive blinking
• involuntary squeezing shut of the eyelids
• light sensitivity and eye irritation
• in severe cases, being unable to see because the eyes will not stay open

Lower face and jaw symptoms (oromandibular dystonia)

• jaw clenching or teeth grinding
• jaw forced open or pulled sideways
• lip pursing, pulling or grimacing
• tongue thrusting or curling
• difficulty chewing, speaking clearly or swallowing
• jaw pain, dental wear and problems with dentures

Other features

• neck involvement (cervical dystonia) or voice involvement (spasmodic dysphonia) in some
• symptoms worse with stress, fatigue, bright light and talking
• symptoms often improved by sensory tricks: touching the chin, lips or eyebrow, chewing gum, humming, holding a pencil in the mouth
• symptoms usually absent during sleep

Causes

• Primary (idiopathic): most cases; no cause found. Genetic factors may contribute in some families.
• Drug-induced: tardive dyskinesia and tardive dystonia after long-term use of antipsychotics or metoclopramide; sometimes levodopa in Parkinson's disease.
• Secondary to brain conditions: stroke, multiple sclerosis, head injury, Parkinson's disease and related disorders.

Diagnosis

Diagnosis is clinical, made by a neurologist or movement disorder specialist based on the pattern of involuntary movements. The doctor will:

• review medicines, especially antipsychotics and anti-nausea drugs
• look for other signs of neurological disease
• check the eyes for dry eye or other irritation that can trigger blinking
• assess chewing, swallowing and speech

Brain MRI or blood tests are used when an underlying cause is suspected, such as onset at a young age, one-sided symptoms or other neurological signs.

Conditions with similar features

Treatment

Botulinum toxin injections

The mainstay of treatment. Small doses are injected into the overactive muscles around the eyes and, for oromandibular dystonia, into jaw muscles such as the masseter, temporalis or the muscles that open the jaw. Effects begin within about a week and last roughly 3 months, so injections are repeated. Eye injections work very well; jaw injections help many people but need careful muscle selection to avoid chewing or swallowing weakness.

Oral medicines

These help some people but often cause side effects:

• anticholinergics such as trihexyphenidyl
• benzodiazepines such as clonazepam
• baclofen
• tetrabenazine and related drugs in some cases

Other approaches

• treat dry eye and use tinted glasses for light sensitivity
• dental appliances or bite guards
• speech and swallowing therapy
• stress management
• if medicine-induced, review or gradually change the triggering medicine under medical supervision

Deep brain stimulation

For severe, disabling Meige syndrome not controlled with injections and medicines, deep brain stimulation of the globus pallidus can significantly improve symptoms in carefully selected patients.

Outlook

Meige syndrome is usually chronic. Symptoms often progress over the first few years and then stabilize. Spontaneous remission is uncommon. With regular botulinum toxin treatment, most people can maintain reasonable function, though jaw symptoms can be harder to control than eye symptoms.

Frequently asked questions

What is Meige syndrome?

Meige syndrome is a movement disorder that combines blepharospasm (involuntary eye closure) with oromandibular dystonia (involuntary jaw, mouth and tongue movements). It is a form of focal or segmental dystonia caused by abnormal brain signaling.

What causes Meige syndrome?

Most cases have no known cause. Some are caused by long-term use of antipsychotic or anti-nausea medicines, and some follow brain conditions such as stroke or Parkinson's disease. Genetics may play a role in some families.

How is Meige syndrome treated?

The main treatment is botulinum toxin injections into the affected eyelid and jaw muscles every few months. Oral medicines, dental appliances, speech and swallowing therapy, and in severe cases deep brain stimulation can also help.

Is Meige syndrome the same as blepharospasm?

No. Blepharospasm affects only the muscles around the eyes. Meige syndrome is blepharospasm plus involuntary movements of the jaw, mouth and tongue. Blepharospasm sometimes spreads over time to become Meige syndrome.

Is Meige syndrome progressive?

It often worsens over the first few years and may spread to the neck or voice, then usually stabilizes. It does not shorten life expectancy, and treatment can control symptoms for most people.