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My eyes seem to blink all the time—Is something wrong?
Blinking can be caused by many factors. Something may be wrong. When I first went into practice, I saw a number of patients who had consulted with me for this problem. Most had been to a handful of doctors only to be told there was nothing wrong. Many patients went undiagnosed for years. Most of these patients, however, suffered from a condition called benign essential blepharospasm or BEB for short. This prompted me to conduct a study related to all the factors involved in diagnosing this condition. I was surprised to discover how little the medical community knew about BEB. This was the start of an interest of mine that has continued to this day, that is, taking care of patients with benign essential blepharospasm.
What is Benign Essential Blepharospasm (BEB)?
BEB is an involuntary forceful closure of the eyelids in the absence of eyelid, ocular or central nervous system disease (Figure 23). Much research is being done, but a true cause has not been found. The cause, however, appears to be multifactorial. A genetic predisposition with an environmental trigger seems to be supported by animal models.
BEB is usually a chronic disorder. I have not seen a patient have a spontaneous remission. This has been reported in a small percentage of BEB usually within the first five years of diagnosis.
The incidence in women is about two times greater than in men. The usual age of onset is between 40 and 50. Interestingly, dry eye may a trigger in BEB. Dry eye also appears to be common in postmenopausal women, which is a common age for the onset of BEB. In a recent survey, approximately 40% of patients stated that their occupation was doing some sort of clerical work. It may be that the eye strain associated with clerical work can cause an abnormal blink reflex, initiating blepharospasms. Approximately 30% of patients report a positive family history. Complex genetic mechanisms are beginning to emerge through genetic research.
What are the symptoms of BEB?
Blepharospasm often begins with increased frequency of blinking. Remember that the normal blink rate is about 20 times per minute. Patients with BEB have varying degrees of eyelid closure. Minimal spasms to complete closure can be seen. This makes patients functionally blind and interferes with all aspects of their lives. Just imagine driving and your eyelids go into a spasm and cause your eyes to close. This could be a very dangerous situation. Patients with BEB are usually healthy with no other medical problems. Older patients with BEB can have associated problems such as high blood pressure and diabetes. Blepharospasm patients have higher light sensitivity than normal people.
Eyelid spasms usually occur spontaneously, but can be aggravated by bright lights or irritants to the eyes such as wind or smoke. Sometimes BEB can be accompanied by other spasms of the lower face and neck (also called Meige syndrome).
Some patients present with spasms involving only half of their face. This is called hemifacial spasm.
All patients with BEB and the variants described above require a complete medical and neurological work-up prior to initiating any treatments. Newer imaging studies, like the PET scan (positron emission tomography), are beginning to elucidate abnormalities in specific nerve pathways in the brain (more specifically the putamen in the basal ganglion). There are no specific blood tests or scans for diagnosing BEB and its’ related disorders.
What treatments are available for BEB?
A small percentage of patients can go into remission (less than 5%). Once established, however, there is a very small chance of remission. There is no know cure for BEB. Treatments are aimed at relieving symptoms. Botulinum toxin injection is the main treatment option for BEB. It has been used since the early 1980’s for this condition. Botulinum toxin has been shown to be highly effective and safe. Side effects are minimal. (see fig list side effects). The only disadvantage is that the injections only last 3-6 months. There are eight know types (serotypes) of the toxin. These are described as A-G. The most common types used clinically are A and B. Botulinum toxin A is know as BOTOX in the United States and DYSPORT in Europe. The only form of botulinum B at this time is MYOBLOC. Myobloc is approved by the FDA for torticollis (neck spasms) but not yet for blepharospasm or hemifacial spasm. Myobloc can be used as an off label use, however, for blepharospasm.
A number of medications have been tried as treatment for these conditions. Anticholinergic agents block acetylcholine, the transmitter between the nerve and the muscle. Valium-like drugs, antihistamines, antiepileptic drugs have all been used. My experience is that because these drugs have a lot of side effects, their use is somewhat limited.
Another injectable medication, doxorubicin, has been used on a limited basis. Skin side effects have been an issue with this drug. Oral medications in my experience have been disappointing in the treatment of BEB.
Surgical treatments are performed less frequently now with the use of botulinum. Techniques included removal of 90% of the muscles around the eye and eyebrow. This was an effective treatment, however the surgery was complicated, risky, and had a long post-op recovery period. Most surgical procedures that are performed today are used as an adjunct to botulinum. Modified blepharoplasties and ptosis surgery are standard operations at this time.
Patients with hemifacial spasm that are found to have vascular compression may benefit by decompression surgery performed by a neurosurgeon. This involves relieving the pressure caused by a small blood vessel on the seventh nerve as it exits the brain.
Various types of eyeglass tints may improve light sensitivity in these patients. Investigational treatments at this writing include liposome encapsulated chemomyectomy using doxorubicin. Other treatments that have been tried include acupuncture, hypnosis, bio-feedback and nutritional therapy.
What other eye conditions can cause eyelid blinking or spasms?
1.Dry Eyes: particular attention is paid to the tear film. The tear film is composed of layers, just like a sandwich. There is a fatty (lipid) outer layer, a watery (aqueous) middle layer, and an inner mucous layer. Any disruption in the sandwich can produce dry eyes and blepharospasm.
3.Eyelid inflammation: Rosacea/ blepharitis
4.Eyelid abnormalities such as misdirected eyelashes
5.Central Nervous System disease, brain tumors or seizure disorders
6.Uncorrected or incorrect eyeglass prescription
These conditions cause what we describe as secondary blepharospasm.
So as you can see, patients who present with eyelid spasms undergo an extensive work-up. A comprehensive ophthalmic and neurologic evaluation is performed on every patient before the diagnosis of BEB is made.
What is Apraxia of Lid Opening?
Apraxia of eyelid opening is associated with BEB. These patients have difficulty opening their eyelids even after treatment with BOTOX. Although the eyelid spasms have been reduced, these patients appears to be failures or have minimal improvement. The eyelids seem to droop close without obvious spasm. Treatment includes myectomy (removal of orbicularis muscle) and ptosis repair (tightening of the levator muscle). Sometimes the brow muscles need to used to elevate the eyelids in a procedure known as a frontalis suspension. A sling is used to attach the eyelid to the brow. Patients then lift their eyelids with the sling connected to the brow. There are many materials now being used as slings-from synthetic to donor material to autologous (patients’ own tissue).
Does blepharospasm interfere with cataract surgery?
The biggest problem would be if you had any uncontrollable spasms during surgery. I always recommend paralyzing the muscles with local anesthetic just to ensure safety during the operation.
Patients with BEB and its related disorders should contact the Benign Essential Blepharospasm Foundation for further information. This organization has a wealth of information on these
conditions. Newsletter, local support groups, meeting locations and ongoing research are some of the many resources available. Their web site is www.blepharospasm.org