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Blepharospasm: the condition treatment options
Using Botox with the proper technique can alleviate a debilitating condition.
BY DEBORAH D. SHERMAN, M.D.

As an ophthalmic plastic surgeon, my practice focuses on patients with different conditions affecting the eyelids; including tumors, trauma, acquired malpositions and medical conditions such as thyroid eye disease. One of the most rewarding and yet challenging conditions that I encounter in my practice is blepharospasm, a focal dystonia estimated to affect 400,000 people worldwide.1

What is Blepharospasm?

Blepharospasm is a focal dystonia characterized by chronic intermittent or persistent involuntary eyelid closure due to spasmodic contractions of the orbicularis oculi muscles. Other facial and neck muscles can also be involved, as well as so-called apraxia of the eyelid opening (inability to initiate eyelid elevation even after cessation of orbicularis spasms). Most cases are idiopathic and blepharospasm is generally a life-long disorder. Its severity ranges from repeated frequent blinking to persistent forceful closure of the eyelids, causing functional blindness.2 Episodes of blepharospasm usually become more frequent as the condition progresses.

The causes of blepharospasm are not fully known; however, it is believed that heredity may play a role in the development of the condition.3 While anyone can display symptoms of blepharospasm, approximately 75% of patients are female, with the average onset of disease at 56 years of age.4

Available Treatment Options

Although the etiology of primary blepharospasm is unknown, it has been postulated that blepharospasm is neurobiologically based on dysfunction of the basal ganglia and an impairment of the dopamine neurotransmitter system. Therefore, therapy for blepharospasm includes administration of anticholinergic and tranquilizing drugs, as well as neuromuscular blocking agents such as the botulinum toxins. Botulinum toxin type A (Botox, Allergan) was approved by the FDA for the treatment of blepharospasm in 1989 with studies showing a high success rate and marked patient improvement, making it the first-line treatment of choice. Botulinum toxin type B (Myobloc, Solstice Neurosciences, Inc.) was also approved for the treatment of the condition in 2000, but patients often complain of significant stinging and burning during the injection, due to its acidic pH level, so it is more often used when patients fail to respond to Botox. Prior to the FDA approval of Botox, surgical therapy was performed more frequently and is still used today in more severe, refractory cases.

Systemic medications present challenges, as patient outcomes vary greatly and are often difficult to predict. The medications available are also associated with side effects and usually require close supervision by a treating physician.

Surgery, in the form of myectomy, involving partial or complete removal of the muscles responsible for eyelid closure, may be the best treatment for severe blepharospasm patients, but is also associated with side effects. This surgical procedure can leave patients unable to properly open or close their eyes, leading to increased rates of dry eye and greater susceptibility to ocular surface diseases. Surgical procedures may not entirely alleviate spasms and can lead to permanent facial disfigurement. Therefore, an experienced surgeon well trained in the technique of myectomy is crucial to an optimal outcome. When considering surgical treatment, full myectomy is reserved for Botox failures and limited myectomy is an excellent adjunct to Botox. Surgical procedures including partial or complete removal of the seventh nerve have also been shown to be effective in relieving symptoms in this patient population.5

First-Line Therapy

Botox is a first-line therapy preferred for the majority of my patients as it produces the best results with very few associated risk factors and side effects. In a study published in the European Journal of Ophthalmology reviewing the product's safety and efficacy, it was found that 93% of test subjects reported improvement after Botox treatment.6

This treatment involves injecting the medication directly into the affected muscles, relieving spasms in approximately 48 hours and lasting for 3 to 4 months depending upon the patient and the amount of medication used. This duration of efficacy has been demonstrated to be longer than clostridium botulinum type A toxin-haemagglutinin complex (Dysport, Ipsen) or Myobloc for this condition.7 Patients can be reinjected as the effects of Botox wear off over time and treatment can be customized by adjusting dosing and injection location. This customized treatment regimen is advantageous as it results in additional benefits to patients by addressing and treating each case individually.

It is important to stress that physicians use only authentic Botox. This product has an established, 15-year history of safety and efficacy for treating this condition. Unfortunately, cases have occurred when other veterinary, unregulated, non-FDA approved toxins were given to patients who suffered severe complications, such as paralysis requiring intubations and artificial respirations.

Consultation and Follow-up

When seeing patients with a blepharospasm for the first time, I speak with them at length about their condition and other ophthalmic concerns, such as pre-existing dry-eye symptoms or contact lens wear and other coexistent neurologic conditions. During this consultation, I also find it helpful to take pictures of the patient's face to assess the proper treatment. Based upon the patient's spasm patterns, I am able to chart where injections will be needed using a facial muscle map such as the one pictured.

The patient's location and severity of spasms will dictate the dosage, number and locations of injections. The initial dosage and pattern of injection sites can be modified after re-evaluating the patient's response 2 weeks following the initial treatment. Injections are then given just under the skin as needed — on average 6 to 8 injection sites in the periorbital area. The patient may notice improvement of spasms on average 2 to 10 days after the treatment has been given.

During follow-up appointments, discussions with the patient and a review of their current and previous response to treatments allows for customization of the dosage, pattern, and location of the Botox. Furthermore, if there is evidence of non-response to the injections, then other treatment modalities can be explored to provide the patient with the greatest benefit in treating their blepharospasm.

Those ophthalmologists interested in learning the injection technique for Botox treatment for blepharospasm, have several options. There are often excellent review articles published in the scientific journal Ophthalmic Plastic and Reconstructive Surgery. There are also 35 approved 2-year fellowships in Ophthalmic Plastic Surgery, as well as training symposia offered at the Fall American Society of Ophthalmic Plastic and Reconstructive Surgery (ASOPRS) meeting during the Annual American Academy of Ophthalmology meeting.

Conclusion

Botox is the treatment option that offers blepharospasm patients superior efficacy and safety. Proper treatment of this condition will allow patients to complete everyday tasks, such as driving or reading. Relief of the debilitating symptoms will allow a newfound quality of life, increased functionality and self-confidence.

For additional information on ASOPRS or their fellowship programs, visit their Web site at www.asoprs.org. For additional information on blepharospasm, visit the Benign Essential Blepharospasm Research Foundation Web site at www.blepharospasm.org.

Deborah Sherman, M.D., is one of the nation's leading ophthalmic plastic surgeons. She practices cosmetic and reconstructive eyelid surgery at the Sherman Aesthetic Center in Nashville, Tenn. and is a clinical professor at Vanderbilt University.

References

1. University of Oxford - Isis Innovation: http://www.isis-innovation.com/licensing/1195.html

2. Costa J et al. Botulinum toxin type A therapy for blepharospasm. Cochrane Database Syst Rev. 2005:CD004900.

3. National Eye Institute Web Site: http://www.nei.nih.gov/health/blepha/index.asp#2

4. Henderson JW. Essential blepharospasm. Trans Am Ophthalmol Soc. 1956;54:453-520.

5. Fante RG. Differential section of the seventh nerve as a tertiary procedure for the treatment of benign essential blepharospasm. Ophthal Plast Reconstr Surg. 2001;17:276-80.

6. Calace P, et al. Treatment of blepharospasm with botulinum neurotoxin type a: long-term results. Eur J Ophthalmol. 2003;13:331-336.

7. Bihari, K. Safety, effectiveness and duration of effect of Botox after switching from Dysport for blepharospasm, cervical dystonia, and hemifacial spasm dystonia, and hemifacial spasm. Curr Med Res Opin. 2005 (3):433-438.