Meige Syndrome

Meige syndrome is a combined form of dystonia – involuntary, irregular muscle contractions – of the lower face, jaw and neck, as well as of the eye.  Described by French neurologist Henri Meige in the early 20th century, it is characterized by forceful blinking and chin jutting. The rare syndrome generally appears later in life, at middle age or beyond (40 – 70 years old). Patients are more likely to be women than men. It is also called idiopathic orofacial dystonia cervical-cranial dystonia, or Brueghel syndrome, after the 16th century Dutch painter believed to have captured a depiction of this syndrome. (1)

Meige syndrome is believed to be caused by a defect in a network of brain cells that control involuntary movement, like blinking, which is orchestrated partially by the basal ganglia. Occasionally similar symptoms may be induced by medical drugs, notably those used to treat Parkinson’s disease. If that is suspected, a reduced dose may alleviate the problem. (1, 2)

There is no cure, although some patients may improve with time. Botulinum toxin injections may be used to suppress mouth spasms. Anti-spasm agents, muscle relaxants, or anti-convulsants have been reported to provide modest relief, at best, to some patients. In addition, patients have found support groups beneficial and can employ some environmental modifications, such as using sunglasses and a hat to avoid bright sunlight or windy environments that may trigger a response. Some activities such as chewing gum, looking down to cook, knit, or garden are also said to be of help. Deep brain stimulation of the globus pallidus internus has been used in other dystonias, and reported effective in a number of patients with Meige syndrome. (3)


References:

  1. Paulson G. Meige’s Syndrome. http://www.blepharospasm.org/meiges-syndrome1.html (accessed March 12, 2012)

  2. The Dystonia Society. Meige Syndrome. http://www.dystonia.org.uk/index.php/about-dystonia/types-of-dystonia/meige-syndrome (accessed Jan. 14, 2013)

  3. Sako W, Morigaki R, Mizobuchi Y, Tsuzuki T, Ima H, Ushio Y, Nagahiro S, Kaji R, Goto S. Bilateral pallidal deep brain stimulation in primary Meige syndrome. Parkinsonism Relat Disord. 2011 Feb;17(2):123-5.

Reviewed July 18, 2013
Stefanos Korfias, MD, PhD
General Secretary, Southeastern Europe Chapter, International Neuromodulation Society
Consultant Neurosurgeon
Senior Lecturer, University of Athens
Department of Neurosurgery, Evangelismos General Hospital
Athens, Greece

Last Updated on Tuesday, April 25, 2017 11:14 AM