Meige Syndrome

Meige Syndrome

Article
Brain & Nerve
Bone, Muscle, & Joint
+4
Contributed byKrish Tangella MD, MBANov 18, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Blepharospasm-Oromandibular Dystonia Syndrome
  • Brueghel Syndrome
  • Meige Dystonia

What is Meige Syndrome? (Definition/Background Information)

  • 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. Symptoms and severity can vary
  • The exact cause of Meige Syndrome is unknown, but researchers suspect that it is due to a combination of genetic and environmental factors
  • Treatment focuses on each person's symptoms and may include drug therapy and/or botulinum A toxin (Botox) injections
  • Other treatment options for Meige Syndrome, such as deep brain stimulation, are currently being considered

(Source: Meige Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets Meige Syndrome? (Age and Sex Distribution)

  • Meige Syndrome is a rare disorder affecting elderly adults
  • Both males and females may be affected
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Meige Syndrome? (Predisposing Factors)

  • Currently, no risk factors have been clearly identified for Meige Syndrome

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Meige Syndrome? (Etiology)

  • The cause of Meige Syndrome is not known at the present time
  • It has been suggested that a combination of genetic and environmental factors may contribute to the development of this disorder

What are the Signs and Symptoms of Meige Syndrome?

The signs and symptoms of Meige Syndrome may vary in type and severity among affected individuals, and include:

  • Blepharospasm, characterized by abnormal, forced blinking and spasms of the eyelids
  • Eye irritation in response to bright lights, wind or fatigue
  • Oromandibular dystonia, characterized by involuntary and often forceful contractions of the jaw and tongue, which can make it hard to open or close the mouth
  • Clenching of the teeth
  • Displacement of the jaw
  • Grimacing
  • Chin thrusting
  • Repeated pursing of the lips
  • Spasms of the tongue and throat, leading to tongue protruding from the mouth 
  • Involuntary contractions of muscles in the neck, arms, legs or other areas of the body

(Source: Meige Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How is Meige Syndrome Diagnosed?

Meige Syndrome is diagnosed on the basis of the following information:

  • Complete physical examination
  • Thorough medical history evaluation
  • Assessment of signs and symptoms
  • Laboratory tests
  • Imaging studies
  • Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Meige Syndrome?

The complications of Meige Syndrome may include:

  • Decreased muscle tone in eyelids and face
  • Progressive difficulty in keeping eyelids open
  • Difficulty swallowing, due to mouth and throat spasms

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Meige Syndrome Treated?

There is no cure for Meige Syndrome, and treatment is usually given to manage the signs and symptoms and any complication that develops. The treatment may involve any of the following measures:

  • Drug therapy (using clonazepam, trihexyphenidyl, diazepam, and/or baclofen)
  • Botulinum A toxin injections in affected muscles
  • “Sensory tricks” such as biting on a toothpick (known to relieve symptoms)
  • Speech therapy
  • Swallowing therapy

How can Meige Syndrome be Prevented?

  • Currently, Meige Syndrome may not be preventable, since the cause and risk factors for this syndrome are not clearly known
  • Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders
  • Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of Meige Syndrome? (Outcomes/Resolutions)

  • The prognosis of Meige Syndrome is dependent upon the age of an affected person, severity of the signs and symptoms, the affected muscles, response to treatment, complications and rate of progression of disease
  • The prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Meige Syndrome:

Meige Syndrome may be known by the following additional synonyms:

  • Blepharospasm-Oromandibular Dystonia
  • Idiopathic Blepharospasm-Oromandibular Dystonia Syndrome
  • Meige's Syndrome
  • Segmental Cranial Dystonia

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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