Meige syndrome

Alternate names

Meige's syndrome; Oral facial dystonia; Brueghel syndrome; Idiopathic blepharospasm-oromandibular dystonia syndrome; Segmental cranial dystonia; Blepharospasm-oromandibular dystonia; Meige dystonia; Blepharospasm - oromandibular dystonia; Blepharospasm-oromandibular dystonia syndrome

Definition

Meige syndrome is a rare, neurological condition characterized by blepharospasm (abnormal movement of the eyelids); oromandibular dystonia (spasms in the jaw and tongue); and sometimes, cervical dystonia.

Cause

The exact cause of Meige syndrome is unknown, but researchers suspect that it is due to a combination of genetic and environmental factors.

Signs and symptoms

• The specific symptoms, severity and progression of Meige syndrome can vary from person to person.
• It most often affects middle-aged people and usually is characterized by a combination of blepharospasm and oromandibular dystonia.
• Blepharospasm is characterized by abnormal, forced blinking and spasms of the eyelids.
• Eye irritation often occurs in response to various stimuli such as bright lights, wind or fatigue.
• As the frequency of muscle spasms and contractions increases, it may become progressively harder for affected people to keep their eyes open.
• Oromandibular dystonia is characterized by involuntary and often forceful contractions of the jaw and tongue, which can make it hard to open or close the mouth.
• Some people may have clenching of the teeth, displacement of the jaw, grimacing, chin thrusting, or repeated pursing of the lips.
• Eyelid and facial muscle tone may gradually decline.
• People with Meige syndrome may also have spasms of the tongue and throat, which can result in the tongue protruding from the mouth and difficulty swallowing. In some cases, muscles in the neck, arms, legs or other areas of the body may become affected.

Diagnosis

Diagnosis should include:[1].

• Facial electromyography
• MRI/CT brain to rule out the stroke or any other brain lesion
• Serum SSA/SSB level
• Serum Cu and ceruloplasmin level
• Beck's depression inventory
• Serum drug screen

Treatment

• Anticholinergics (e.g., trihexyphenidyl), dopamine antagonists (e.g., tiapride, tetrabenazine), GABA receptor agonists (e.g., benzodiazepines, baclofen) are effective for this condition.
• Other medications include antiepileptics (e.g., valproic acid), and a number of psychoactive drugs.
• Eszopiclone and nitrazepam react at those specific subunits (omega-1 and omega-2) of the GABA receptor complex that alleviate the eyelid spasming. According to some case reports, zolpidem is effective in such patients as it is highly specific for a GABA omega-1 receptor.
• Long-term use of psychoactive drugs can cause eyelid spasming that is more often associated with the use of typical antipsychotics, though worsening of blepharospasm has been reported with the use of olanzapine.
• Botulinum A injection is showing promising results.
• Deep brain stimulation of globus pallidus interna (GPi) has proved to be an effective treatment option in whom botulism toxin and other conservative treatment options are not providing the required results.[2].

NIH genetic and rare disease info

• NIH info on Meige syndrome

Meige syndrome is a rare disease.

Meige syndrome Resources
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