Involuntary facial movements have been recognized for long time and were depicted by artists who were fascinated how these movements distorted the facial expression. For example, in the 16th century, the Flemish artist Brueghel painted a woman with apparent blepharospasm and involuntary jaw opening. Although the eponym "Meige's syndrome" is still occasionally used to designate the combination of blepharospasm (involuntary eye closure) and oro-mandibular dystonia (involuntary jaw and mouth spasms), a more appropriate term to describe this combination is cranial dystonia. The term dystonia means involuntary sustained, patterned, twisting, pulling or squeezing movements. One of the most common types of adult-onset focal dystonia is blepharospasm.
Before the development of sustained closure of the eyelids, about one-third of blepharospasm patients report increased frequency of blinking, commonly associated with a feeling of irritation in the eyes and light sensitivity (photophobia). The blinking usually progresses to more sustained contractions of the orbicularis oculi (eye lid muscles) leading to forceful closure of eyelids. Although always bilateral, in some cases blepharospasm starts in one eye or may remain asymmetrical. Many patients are initially misdiagnosed as having "dry eyes" but eye drops or lubricants rarely provide any benefit. It is important to note that blepharospasm is a neurological, not an ophthalmological, disorder as there is usually no abnormality with the eyes on careful eye examination.
Blepharospasm is seldom an isolated condition; this form of dystonia is often associated with dystonia in other facial, jaw, or neck muscles leading to "cranial-cervical dystonia." In some patients the dystonia spreads to involve the vocal cords (spasmodic dysphonia), trunk and limbs. Although usually sporadic (without family history), genetic mechanisms many be involved, as they often are in other forms of dystonia.
Blepharospasm may vary in severity from only a slightly annoying or embarrassing condition to a disabling disorder that may render the patient functionally blind and interfere with daily activities such as reading, watching television, and driving. Blepharospasm is usually exacerbated by bright light (photophobia) and, as a result, many patients wear sunglasses both outside and inside. The spasms may be transiently alleviated by pulling on an upper eyelid or an eyebrow, pinching the neck, talking, humming, yawning, singing, sleeping, relaxation, reading, concentration, looking down and other maneuvers or sensory tricks ("geste antagonistique").
Botulinum toxin (BTX) injections into the eyelids and eyebrows is now considered the treatment of choice, providing moderate to marked improvement in over 90 percent of patients. Some patients with blepharospasm have partial relief from medications such as clonazepam, trihexyphenidyl, lorazepam, baclofen and tetrabenazine. The average latency from the time of the BTX injection to the onset of improvement is 2-5 days and the average duration is 3-4 months, after which time the treatment must be repeated in order to sustain the benefit. In addition to the marked functional improvement, there is usually a meaningful amelioration of discomfort and, because of less embarrassment, the patients' self-esteem also frequently improves. Although about 10-15 percent of all treatment sessions are followed by some side effects (ptosis, blurring of vision or diplopia, tearing, and local hematoma), the complications only rarely affect patient's functioning and usually resolve spontaneously in less than two weeks. There is no apparent decline in benefit and the frequency of complications actually decreases after repeat BTX treatments. In very severe cases in patients with significant functional impairment who have not responded well to botulinum toxin, surgery may be another option. A myectomy, which involves removal of some or all of the muscles responsible for eyelid closure, may be considered. Other surgeries such as myo-osseous fixation and deep brain stimulation are currently under investigation.
Benign Essential Blepharospasm Research Foundation
P.O. Box 12468
Beaumont, TX 77726-2468
Phone: (409) 832-0788
Fax: (409) 832-0890
©2011 Joseph Jankovic, M.D.
Blomstedt P, Tisch S, Hariz MI. Pallidal deep brain stimulation in the treatment of Meige syndrome. Acta Neurol Scand. 2008;118:198-202.
Borodic GE. Orbicularis oculi myo-osseous fixation: a new treatment for benign essential blepharospasm and blepharospasm associated with diffuse facial dystonia (meige syndrome). Ophthalmic Surg Lasers Imaging. 2010;41(3):360-9.
Brodsky H, Vuong KD, Thomas M, Jankovic J. Glabellar and palmomental reflexes in parkinsonian disorders. Neurology. 2004;63:1096-8.
Defazio G, Brancati F, Valente EM, et al. Familial blepharospasm is inherited as an autosomal dominant trait and relates to a novel unassigned gene. Mov Disord. 2003;18:207-12.
Defazio G, Livrea P. Primary blepharospasm: diagnosis and management. Drugs. 2004;64(3):237-44.
Defazio G, Martino D, Abbruzzese G, et al. Influence of coffee drinking and cigarette smoking on the risk of primary late onset blepharospasm: evidence from a multicentre case control study. J Neurol Neurosurg Psychiatry. 2007;78:877-9.
Fahn S, Jankovic J. Principles and Practice of Movement Disorders, Churchill Livingstone, Elsevier, Philadelphia, PA, 2007:1-652. (Accompanied by a DVD of movement disorders.)
Forget R, Tozlovanu V, Iancu A, Boghen D. Botulinum toxin improves lid opening in blepharospasm-associated apraxia of lid opening. Neurology. 2002;58:1843-6.
Hallett M, Evinger C, Jankovic J, Stacy M; BEBRF International Workshop. Update on blepharospasm: report from the BEBRF International Workshop. Neurology. 2008;71:1275-82.
Horwath-Winter J, Bergloeff J, Floegel I, Haller-Schober EM, Schmut O. Botulinum toxin A treatment in patients suffering from blepharospasm and dry eye. Br J Ophthalmol. 2003;87:54-6.
Jankovic J. Blepharospasm. In: Gilman S, ed. MedLink Neurology, Arbor Publishing, La Jolla, CA, Annual Updates 1995-2009.
Jankovic J. Clinical efficacy and tolerability of Xeomin® in the treatment of blepharospasm. European J Neurology. 2009;16 Suppl 2:14-8.
Jankovic J. Cranial-cervical dystonia. Adelman G, Smith BH, eds. Elsevier's Encyclopedia of Neuroscience, 3rd edition, Elsevier Science, Amsterdam, The Netherlands, 2004. CD-ROM.
Jankovic J. Disease-oriented approach to botulinum toxin use. Toxicon. 2009;54:614-23.
Jankovic J. Dystonia: Medical therapy and botulinum toxin. In: Fahn S, Hallett M, DeLong DR, eds. Dystonia 4, Lippincott Williams & Wilkins, Adv Neurol. 2004;94:275-863.
Jankovic J. Treatment of dystonia. Lancet Neurol. 2006;5:864-72.
Jankovic J, Comella C, Hanschmann A, Grafe S. Efficacy and safety of incobotulinumtoxinA (NT 201, Xeomin) in the treatment of blepharospasm: a randomized trial. Mov Disord. 2011;26:1521-8.
Jankovic J, Kenney C, Grafe S, Goertelmeyer R, Comes G. Relationship between various clinical outcome assessments in patients with blepharospasm. Mov Disord. 2009;24:407-13.
Jankovic J, Shannon KM. Movement disorders. In: Bradley WG, Daroff RB, Fenichel GM, Jankovic J, eds. Neurology in Clinical Practice, 5th edition, Butterworth-Heinemann (Elsevier), Philadelphia, PA, 2008.
Jankovic J, Tolosa E, eds. Parkinson's Disease and Movement Disorders, 5th edition, Lippincott Williams and Wilkins, Philadelphia, PA, 2007:1-720. (Accompanied by a CD video atlas.)
Jost WH, Kohl A. Botulinum toxin: evidence-based medicine criteria in blepharospasm and hemifacial spasm. J Neurol. 2001;248:21-4.
Kenney C, Jankovic J. Botulinum toxin in the treatment of blepharospasm and hemifacial spasm. J Neural Transm. 2008;115:585-91.
Kim HT, Edwards MJ, Tyson J, Quinn NP, Bitner-Glindzicz M, Bhatia KP. Blepharospasm and limb dystonia caused by Mohr-Tranebjaerg syndrome with a novel splice-site mutation in the deafness/dystonia peptide gene. Mov Disord. 2007;22:1328-31.
Klein C, Ozelius LJ. Dystonia: clinical features, genetics, and treatment. Curr Opin Neurol. 2002;15:491-7.
Mejia NI, Vuong KD, Jankovic J. Long-term botulinum toxin efficacy, safety and immunogenicity. Mov Disord. 2005;20:592-7.
Nemeth AH. The genetics of primary dystonias and related disorders. Brain. 2002;125:695-721.
Peckham EL, Lopez G, Shamim EA, et al. Clinical features of patients with blepharospasm: a report of 240 patients. Eur J Neurol. 2011;18:382-6.
Simpson DM, Blitzer A, Brashear A, Comella C, Dubinsky R, Hallett M, Jankovic J, Karp B, Ludlow CL, Miyasaki JM, Naumann M, So Y. Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Assessment: Botulinum neurotoxin for the treatment of movement disorders (an evidence-based review): report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2008;70:1699-706.
Tan EK, Chan LL, Koh KK. Coexistent blepharospasm and hemifacial spasm: overlapping pathophysiologic mechanism? J Neurol Neurosurg Psychiatry. 2004;75(3):494-6.
Tucha O, Naumann M, Berg D, et al. Quality of life in patients with blepharospasm. Acta Neurol Scand. 2001;103:49-52.