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Department of Neurology

Houston, Texas

BCM neurologists see patients through the Baylor Clinic and some of the world's leading specialty clinics.
Department of Neurology
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Blepharospasm

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 a 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 “dry eyes”, but eye drops or lubricants rarely provide any benefit. It is important for 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% of patients, some patients with blepharospasm obtain 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 to 5 days and the average duration is 3 to 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% 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 2 weeks. There is no apparent decline in benefit and the frequency of complications actually decreases after repeat BTX treatments.

Selected References

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  • 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.
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  • 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, 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).
  • 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. Cranial-cervical dystonia. Adelman G, Smith BH, eds, Elsevier’s Encyclopedia of Neuroscience, 3rd edition, Elsevier Science, Amsterdam, The Netherlands, 2004. CD-ROM.
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  • 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.
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  • Kenney C, Jankovic J. Botulinum toxin in the treatment of blepharospasm and hemifacial spasm. J Neural Transm 2007 (in press).
  • Klein C, Ozelius LJ. Dystonia: clinical features, genetics, and treatment. Curr Opin Neurol 2002;15:491-7.
  • Nemeth AH. The genetics of primary dystonias and related disorders. Brain 2002;125:695-721.
  • 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

Appendix

Benign Essential Blepharospasm Research Foundation, Inc
BEBRF, Inc.
P.O. Box 12468
Beaumont, TX 77726-2468
Tel: 409-832-0788
Fax: 409-832-0890
Email: bebrf@ih2000.net
Web: http://www.blepharospasm.org

For additional information visit http://www.bcm.edu/neurology/pdcmdc/