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Meige syndrome


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Other Names:
Meige's syndrome; Oral facial dystonia; Brueghel syndrome; 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 See More
Categories:
Congenital and Genetic Diseases; Nervous System Diseases

Summary Summary


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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.[1][2] 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.[1] Other treatment options, such as deep brain stimulation, are currently being considered.
Last updated: 2/17/2015

Symptoms Symptoms


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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.[1]

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.[1]

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.[1]

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.[1]
Last updated: 2/18/2015
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Prognosis Prognosis


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The long-term outlook (prognosis) for people with Meige syndrome is hard to predict because the specific symptoms, severity and progression of the condition vary from person to person. The prognosis likely depends on several factors, such as age of onset; rate of progression; muscles affected; how early treatment is started; and how a person responds to treatment. Some affected people improve over time.

The first symptoms of Meige syndrome may occur anywhere from about 30 to 70 years of age, but they most often occur in the sixth decade of life.[3] Blepharospasm (dystonia of the eyelids) is the most frequent initial complaint of affected people.[4]

In most cases, dystonia begins focally. Over time, it may spread to other muscles of the body. When dystonia spreads, it most commonly spreads to areas immediately next to affected muscles. For example, blepharospasm typically spreads to the lower face and jaw muscles. Rarely, dystonia can spread to more distant muscles in the arms and legs.[5] The chance for dystonia to spread is thought to be highest within the first 3 years after onset. However, it can spread more than a decade later. Overall, people with initial onset of blepharospasm have at least a 50% lifetime risk of dystonia spreading.[5]

One study found that on average, affected people show gradual worsening of involuntary movements for a little over 2 years, followed by movements slowly improving. The authors of this study suggested that factors that may contribute to a better prognosis include younger age of onset; shorter time between initial onset and progression to the worst symptoms; mild involuntary movements when the symptoms were the worst; and shorter time between onset and starting treatment.[6]
Last updated: 2/18/2015

Research Research


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Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Meige syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations


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Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

  • Benign Essential Blepharospasm Research Foundation
    637 North 7th Street, Suite 102
    PO Box 12468
    Beaumont, TX 77726-2468
    Telephone: 409-832-0788
    Fax: 409-832-0890
    E-mail: bebrf@blepharospasm.org
    Website: http://www.blepharospasm.org
  • Cure Dystonia Now
    PO Box 9040
    Farmingdale, NY 11735
    Telephone: +1-516-584-4156
    E-mail: info@curedystonianow.org
    Website: http://www.curedystonianow.org/index
  • Dystonia Medical Research Foundation
    1 East Wacker Drive
    Suite 1730
    Chicago, IL 60601-1905
    Toll-free: 1-0800-377-3978
    Telephone: +1-312-755-0198
    Fax: +1-312-803-0138
    E-mail: dystonia@dystonia-foundation.org
    Website: https://dystonia-foundation.org/
  • Dystonia Society
    89 Albert Embankment
    London, SE1 7TP United Kingdom
    Telephone: 020 7793 3651 (general enquiries)
    E-mail: https://www.dystonia.org.uk/contact
    Website: https://www.dystonia.org.uk/
Do you know of an organization? We want to hear from you.

Learn More Learn More


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These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Meige syndrome. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers


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Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

Have a question? Contact a GARD Information Specialist.

References References


  1. Meige syndrome. NORD. April 14, 2008; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/235/viewAbstract.
  2. Lyons MK, Birch BD, Hillman RA, Boucher OK, and Evidente VG. Long-term follow-up of deep brain stimulation for Meige syndrome. Neurosurg Focus. August, 2010; 29(2):E5. https://www.ncbi.nlm.nih.gov/pubmed/20672922.
  3. Møller E, Werdelin LM, Bakke M, Dalager T, Prytz S, Regeur L. Treatment of perioral dystonia with botulinum toxin in 4 cases of Meige's syndrome. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. November, 2003; 96(5):544-549.
  4. Reese R, Gruber D, Schoenecker T, Bäzner H, Blahak C, Capelle HH, Falk D, Herzog J, Pinsker MO, Schneider GH, Schrader C, Deuschl G, Mehdorn HM, Kupsch A, Volkmann J, and Krauss JK. Long-term clinical outcome in meige syndrome treated with internal pallidum deep brain stimulation. Mov Disord. March, 2011; 26(4):691-698. https://www.ncbi.nlm.nih.gov/pubmed/21312284.
  5. Mark S. Ledoux. Meige's Syndrome. Benign Essential Blepharospasm Research Foundation (BEBRF). December, 2008; http://www.blepharospasm.org/meiges-syndrome2.html. Accessed 2/18/2015.
  6. Kamitsukasa I, Yamada T, Tokumaru Y, Hirayama K. [Clinical features and factors related to the functional prognosis in Meige's syndrome]. Rinsho Shinkeigaku. March, 1995; 35(3):231-236.
  7. Czyz CN, Burns JA, Petrie TP, Watkins JR, Cahill KV, Foster JA. Long-term botulinum toxin treatment of benign essential blepharospasm, hemifacial spasm, and Meige syndrome. Am J Ophthalmol. July, 2013; 156(1):173-177. https://www.ncbi.nlm.nih.gov/pubmed/23541393.
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