Abnormal, involuntary eyelid and facial movements are caused by a variety of neurologic lesions and represent part of the spectrum of dystonic movement disorders or dystonia. Essential blepharospasm, hemifacial spasm, aberrant facial nerve regeneration following facial nerve palsy, and Meige’s Syndrome are debilitating, chronic disorders involving the eyelids. Other forms of dystonia involving the head and neck include oromandibular dystonia, torticollis, and spastic dysphonia. It is common for patients to initially have involuntary contractions involving one area and later progress to involvement of adjacent muscle groups. Slow, sustained, twisting, pulling, or squeezing movements characterize dystonic movement disorders. Frequently the cause cannot be determined and the diagnosis is based on clinical findings.
Essential blepharospasm is a form of craniocervical dystonia that typically begins in the 6th decade of life and involves females more than males. Patients often initially present with an increased blinking frequency, photophobia (light sensitivity), and dry eyes. These symptoms progress to sustained, then forceful eyelid closure and eventually uncontrollable eyelid spasms. Essential blepharospasm is a bilateral condition; however, some patients may have asymmetric involvement. Symptoms often fluctuate for 5 years or so before reaching a plateau or stable phase. Blepharitis, bright lights, watching TV, and interpersonal interaction may “trigger” a worsening of the eyelid spasm. The spasms typically diminish during sleep.
Blepharospasm may have an alternative presentation called apraxic blepharospasm. Patients with eyelid apraxia are unable to open their eyelids. Elimination of eyelid spasms with Botox treatment may help with eyelid opening; however, even with good treatment of blepharospasm many patients with apraxia will continue to have difficulty with eyelid opening. This is a very challenging condition to treat. Severe cases of eyelid apraxia may improve with eyelid surgery and/or frontalis sling procedures.
The cause of Essential Blepharospasm and Eyelid Apraxia are not fully understood and is an area of intense medical research.
Meige’s syndrome (Henri Meige, 1911) is a dystonic condition involving the oromandibular region and lower face. Frequently, the lower facial spasms are accompanied by blepharospasm. The lower facial spasm of Meige’s syndrome can be quite disfiguring, but the associated eyelid spasms often cause the greatest disability and distress in most patients.
Hemifacial spasm is characterized by involuntary contractions of the muscles of one side of the face. The condition typically begins with an intermittent twitching of one eyelid that progresses over months to years to involve other areas of the involved side of the face. Hemifacial spasm is believed to be caused by compression of the facial nerve. While the source of compression is not frequently identified, a magnetic resonance imaging study (MRI and MRA) is typically recommended to identify an anomalous brainstem vessel or brainstem mass lesion. Patients with a known area of blood vessel compression may benefit from microvascular decompression surgery performed by an experienced neurosurgeon.
Treatment of Blepharospasm
A variety of medications used for treating other systemic disorders such as Parkinson’s disease, hypertension, and anxiety occasionally have a beneficial effect on blepharospasm. Artane (anti-cholinergic), Klonopin (gabanergic muscle relaxant), and Keppra (anti-seizure) have been shown to help some essential blepharospasm and/or Meige patient. Some of these medications have signficant side effects and interaction with other medications, so the patient’s primary care physician should closely monitor their use. Unfortunately, moderate to severe blepharospasm does not usually respond to medical therapy.
Dry Eye Treatment
- Tinted eyeglasses (FL-41, Bangerter foil)
- Acupuncture (for painful blepharospasms)
- Reverse sensory tricks – patient gains relief from relieving a stimulus (for example - taking off spectacles)
- Behavioral Modification
1. Avoid fighting the spasm. Allow the spasm to occur and naturally relax.
2. Increase blinking. During periods of non-spasm, frequent blinking may be helpful.
Botox™ (botulinum toxin type A) injections directly over the involved muscles are the mainstay of treatment for Essential Blepharospasm, Meige’s Syndrome, and Hemifacial Spasm. When injected locally, by an experienced physician this treatment is very safe and very effective. Most patients experience dramatic improvement in 7-10 days after injection. The effects of the injection last 3-6 months and periodic injections are necessary to achieve continued control of the eyelid spasms. There can be some variability in the effectiveness of Botox treatments from patient to patient and from treatment to treatment in the same patient. Botox™ was approved by the U.S.F.D.A. in 1989 for the treatment of blepharospasm and hemifacial spasm.
Other Botulinum Toxin Preparations
Other botulinum toxin products have been introduced in Europe and Canada in the last several years. More recently, alternative botulinum toxin therapies have entered the U.S. market. Each product has a different therapeutic profile. Clinical experience with these second generation products is limited relative to the extensive experience with Botox™ over the past two decades.
Dysport™ (type A also know as Reloxin) and Xeomin™ (a pure botulinum neurotoxin) were both recently approved by the U.S.F.D.A. and may have similar therapeutic efficacy as Botox. Dr. Klapper has experience with both of these medications and several investigators are looking at these newer products to determine their role in the treatment of blepharospasm and for cosmetic use. Myobloc™ (botulinum toxin type B) injections have been reported to be more painful than Botox™ or Dysport™. Due to its larger molecule size, Myobloc™ is believed to migrate further from the site of injection with perhaps a greater risk of unwanted effects. Dr. Klapper does not provide Myobloc™ injections.
Millions of dollars are spent each year investigating available preparations and looking for improved treatments that may prolong the duration of action of Botox and related products.
For patients with severe blepharospasm and unable to continue with botulinum toxin injections, myectomy surgery may be considered. This surgery involves removal of orbicularis oculi muscle (the sphincter muscle responsible for eyelid closure). Myectomy surgeries may be performed in a limited or extensive fashion. A limited myectomy is often performed at the time of blepharoplasty or ptosis repair and may lessen dependence on Botox following eyelid surgery. Extensive myectomy surgery, fortunately, is rarely indicated. It is much more complex and may be disfiguring. Extensive myectomy is generally reserved for severe cases of blepharospasm that are not responsive to periorbital Botox injections.
Upper Eyelid Ptosis Repair and/or Blepharoplasty Surgery
Excessive dermatochalasis (loose, redundant, over-hanging upper eyelid skin) may activate or “trigger” worsening of blepharospasm. Redundant upper eyelid skin can also interfere with the superior field of vision and limit patients’ normal activities of daily living. Upper eyelid blepharoplasty surgery corrects excessive and dermatochalasis and provides both functional and cosmetic improvement.
Many patients with blepharospasm have or develop droopy upper eyelids or ptosis. As with dermatochalasis, upper eyelid ptosis may limit a patient’s already restricted peripheral field of vision. Ptosis surgery corrects the abnormal eyelid position. A conservative, limited myectomy may be recommended at the time of ptosis or blepharoplasty surgery in order to better control blepharospasm and reduce dependence on botulinum toxin injections.