Meige's syndrome II
The term Meige's syndrome II/Brueghel's syndrome is used for blepharospasm and oromandibular dystonia occurring together. It is a disabling spasm of the facial musculature consisting of primary blepharospasm followed by abnormal facial movement. Squinting may begin unilaterally but soon becomes bilateral. In time, the lower facial muscles becomes involved with yawning, jaw opening, and abnormal tongue movements. The voice is often affected as well. The involuntary movements cease during sleep. The condition may be aggravated by eating or talking and is sometimes lessened by humming, singing, yawning or voluntary opening of the mouth.
Henry Meige in 1910 described a condition characterized by blepharospasm and facial, mandibular, oral, lingual, and laryngeal spasms and called it "spasm facial median." There is a distinct overlap between benign essential blepharospasm (BEB) and Meige although either can exist without the other.
One of the earliest suspected documentations of blepharospasm is a painting - De Gaper - by the Flemish artist Brueghel (1525-1569), who painted a woman with apparent blepharospasm with facial and neck involvement. The term "Brueghel syndrome" is used when extensive mandibular involvement is a major component of the disease.
Talkow in 1870 described a patient with similar findings, as did Horatio C. Wood in presentations at the University of Pennsylvania in the 1870s.
- H. Meige and E. Feindel.
Les tics et leur traitement. Paris, 1902. Translated into English.
- H. Meige:
Tics. Paris, 1905.
- H. Meige:
Les convulsions de la face, une forme clinique de convulsion faciale, bilaterale et mediane.
Revue neurologique, Paris, 1910, 20: 437-443.
- C. D. Marsden:
Blepharospasm-oromandibular dystonia syndrome (Brueghel’s syndrome). A variant of adult-onset torsion dystonia?
Journal of Neurology, Neurosurgery and Psychiatry, London, 1976, 39: 1204-1209.