Medical Botox (botulinum toxin A) is a toxin produced by the bacteria Clostridium botulinum. The toxin weakens muscles by blocking nerve impulses transmitted from the nerve endings of the muscles. Botulinum toxin is an approved treatment for blepharospasm and hemifacial spasm in the United States and Canada.
The brain normally sends electrical messages to muscles so they can contract and move. A substance called acetylcholine transmits the electrical messages to the muscles. Botulinum works to block the release of acetylcholine so the muscles don’t receive a message to contract. That means that the muscle spasms stop or are greatly reduced, relieving the patient of symptoms.
Patients normally see benefits of Botulinum treatment in 1 to 14 days after treatment. The effects last for an average of three to four months. Repeated treatments remain effective over a long period of time, as long as the doses are kept small and the injections are given at a minimum of three-month intervals.
Side effects of Botox treatment may include ptosis (droopy eyelid), blurred vision, double vision, lagophthalmos (an inability to close eyelids), ectropion (eyelid turns out), sagging of the mouth, brow droop, or epiphora (watery eyes).
Treatment of blepharospasm depends on the patient’s psychological state and how much the condition interferes with the patient’s life (occupation, home life, pursuing hobbies or other activities). Some oral medications might be used to treat blepharospasm, but few have undergone rigorous clinical trials. Botulinum toxin A (Botox) is the most effective and best-tolerated treatment for BEB and related disorders.
When treating blepharospasm, the physician uses a fine needle to inject tiny doses of botulinum toxin into muscles at several sites above and below the eyes. The sites of the injection vary slightly from one patient to another and according to physician preference. Injection sites usually include the eyelid, the brow, and the muscles under the lower lid. Long-term follow-up studies have shown Botox to be a very safe and effective treatment for BEB, with up to 90 % of patients obtaining almost complete relief of their blepharospasm.
Sometimes Botox treatments don’t provide adequate relief of blepharospasm, or functional or cosmetic deformities of the eyelids prevent the patient from achieving an optimal result. In those situations, surgery might be considered.
Myectomy (surgery to remove all or some of the muscles responsible for eyelid closure) currently is the most effective surgical treatment of blepharospasm. Myectomy has improved visual disability in 75 to 80 percent of patients with blepharospasm.
Newer techniques in myectomy improve function, promote faster healing, and have better cosmetic results and fewer complications than earlier surgeries. The procedure can be performed on an outpatient basis. Chronic lymphdema (swelling) that used to last for months or even years is now markedly reduced to a period of several weeks to a few months.
Myokymia is a condition characterized by spontaneous, fine muscle twitches without muscular atrophy or weakness. Eyelid myokymia typically affects the muscle of one of the lower eyelids. The upper eyelids might also be affected occasionally.
Eyelid myokymia is normally benign, self-limited, and not associated with any disease. The symptoms might sometimes be so irritating to the patient that they must be treated. Botox injections are an excellent method of reducing or eliminating the twitches.
Botox injections will not cure BEB or its associated conditions. Instead, the goal of Botox therapy is to achieve improvement, seen by the condition having a smaller impact on the patient’s normal activities. Therapy must be individualized, because what works for one patient might cause intolerable side effects in another. Treatment often follows a trial-and-error approach to find the right combination of medication. Patients and their physicians must watch for and monitor benefits of treatment and possible side effects.
Blepharospasm is a name that can be applied to any abnormal blinking or eyelid tic or twitch resulting from any cause, ranging from dry eyes to Tourette’s syndrome. The blepharospasm referred to here is officially called Benign Essential Blepharospasm (BEB) to distinguish it from the less serious secondary blinking disorders. “Benign” indicates the condition is not life threatening and “essential” is a medical term meaning “of unknown cause”. It is both a cranial and a focal dystonia. Cranial refers to the head and focal indicates confinement to one part. The word dystonia describes abnormal involuntary sustained muscle contractions and spasms.
Patients with blepharospasm have normal eyes. The visual disturbance is due solely to the forced closure of the eyelids. Blepharospasm usually begins gradually with excessive blinking and/or eye irritation. In the early stages it may only occur with specific precipitating stressors, such as bright lights, fatigue, and emotional tension. As the condition progresses, it occurs frequently during the day. The spasms disappear in sleep, and some people find that after a good night’s sleep, the spasms don’t appear for several hours after waking. Concentrating on a specific task may reduce the frequency of the spasms. As the condition progresses, the spasms may intensify so that when they occur, the patient is functionally blind; and the eyelids may remain forcefully closed for several hours at a time.
Blepharospasm is thought to be due to abnormal functioning of the basal ganglia which are situated at the base of the brain. The basal ganglia play a role in all coordinated movements. We still do not know what goes wrong in the basal ganglia. It may be there is a disturbance of various “messenger” chemicals involved in transmitting information from one nerve cell to another. In most people blepharospasm develops spontaneously with no known precipitating factor. However, it has been observed that the signs and symptoms of dry eye syndrome frequently precede and/or occur concomitantly with blepharospasm. It has been suggested that dry eye may trigger the onset of blepharospasm in susceptible persons. Infrequently, it may be a familial disease with more than one family member affected. Blepharospasm can occur with dystonia affecting the mouth and/or jaw (oromandibular dystonia, Meige syndrome). In such cases, spasms of the eyelids are accompanied by jaw clenching or mouth opening, grimacing, and tongue protrusion.
Myokymia is the spontaneous, fine fascicular contractions (twitches) of muscle without muscular atrophy or weakness. Eyelid myokymia typically involves the muscle of one of the lower eyelids; occasionally, the upper eyelids also can be affected. In most cases, eyelid myokymia is benign, self-limited, and not associated with any disease. Occasionally, the spasms are so irritating to the patient that they must be treated, and Botox is an excellent method of reducing or eliminating the twitches.
What is the best treatment for BEB and the various other forms? It is determined by how much BEB is interfering with your life, and this includes your occupation, your home life, pursuing hobbies and other pleasurable activities, or your psychological state. The most effective and best-tolerated treatment for BEB and related disorders is botulinum toxin type A (BOTOX).
Botulinum toxin is an approved treatment for blepharospasm and hemifacial spasm in the United States and Canada. This is a toxin produced by the bacteria Clostridium botulinum. It weakens the muscles by blocking nerve impulses transmitted from the nerve endings of the muscles. When it is used to treat blepharospasm, very tiny doses of botulinum toxin are injected into muscles at several sites above and below the eyes. The sites of the injection will vary slightly from patient to patient and according to physician preference. They are usually given on the eyelid, the brow, and the muscles under the lower lid. The injections are carried out with a very fine needle. Benefits begin in 1 – 14 days after the treatment and last for an average of three to four months. Long-term follow-up studies have shown it to be a very safe and effective treatment, with up to 90 % of patients obtaining almost complete relief of their blepharospasm. Side effects include drooping of the eyelid (ptosis), blurred vision, and double vision (diplopia). Tearing may occur. All are transient and recover spontaneously. Providing the dose is kept small and the injections carried out at a minimum of three-month intervals, repeated treatments remain effective over a long period of time.
Dr. Siddens has used Botulinum Type A Toxin (BOTOX) since 1991 in hundreds of patients with BEB and hemifacial spasm. However, a newer type of botox, type B (MYOBLOC), has become available, but Dr. Siddens’ experience with Myobloc is very limited. Furthermore, he has not used Myobloc for hemifacial spasm. Type B Toxin may be slightly more convenient to use, as it comes already in solution and has a very long “shelf life.” Nevertheless, Type A Toxin remains the first injection choice for patients with BEB, since Type B Toxin seems to last only about 2/3 as long and is reported by patients to hurt more than Botox during injection. Two newer forms of Botulinum Type A have been developed (DYSPORT and XEOMIN). Dysport is only indicated for to treat the abnormal head position and neck pain that happens with cervical dystonia (CD) in adults, and to improve the look of moderate to severe frown lines between the eyebrows (glabellar lines) in adults younger than 65 years of age for a short period of time (temporary). Xeomin is indicated treat the abnormal head position and neck pain that happens with cervical dystonia (CD) in adults and to treat abnormal spasm of the eyelids (blepharospasm) in adults who have had prior treatment with onabotulinumtoxinA (Botox). Xeomin is considered a “naked” drug, i.e., no additive protein molecules. The difference is that Botox has an accompanying protein, but Xeomin is ‘naked’. In the skin, all of the protein detaches from Botox within 1 minute of injection so there is no reason why the effect of Xeomin should differ from Botox. Xeomin is new to the United States, and Dr. Siddens has yet to try this medication. It may be best suited to those who have no responded well to Botox, but time will tell.
If you have had what you consider to be an inadequate response to botulinum toxin then before going straight to medical therapy, it is essential to first determine why the response was inadequate, as the majority of people with BEB respond well. One of the most common reasons for “failure” of botulinum toxin is inexperience of the practitioner. Although injecting botulinum toxin is relatively straightforward, experience with Botox is everything, and if you have not had a good response, make sure you are seeing an experienced “injector.” Other reasons for an inadequate response include atypical blepharospasm (such as eyelid opening apraxia), unrealistic expectations (it’s not a cure) or the development of resistance, which is relatively uncommon. Once these factors have been considered, then it is reasonable to combine botulinum toxin with medication(s).
The most effective and best-tolerated treatment for BEB and related disorders remains botulinum toxin. However, there are a variety of oral medications used for blepharospasm, but few have been subjected to rigorous clinical trials, and therefore treatment is mostly a matter of trial and error. Dr. Siddens does not prescribe these oral medications, but works with your primary care physician to find which oral medication may be bet.
Therapy must be individualized – what works for one patient may cause intolerable side effects in another. And, just like botulinum toxin, medications are also not a cure.
Instead, the goal is to achieve improvement, evidenced by blepharospasm taking less of a toll on your normal personal and occupational activities. Unless a patient can tell me a specific way in which they are functioning better, then it’s unlikely a medication is helping. Not uncommonly though, as a seemingly unhelpful medication is being tapered off, you may notice worsening of blepharospasm, proving that the medication was helping more than originally appreciated and therefore worth continuing. In addition to monitoring closely for beneficial effects, it is equally important to watch for side effects. A discussion about common and uncommon side effects should always proceed starting a medication. But, you cannot expect your physician to go over every single potential side effect, so you need to be responsible for learning about the medications yourself. By taking such an active role in your treatment, the relationship with your physician becomes a partnership rather than a paternalistic one in which you play a passive role.
Surgery has very limited value in the treatment of BEB, but surgical treatment of BEB is possible. Before surgery is recommended, patients are advised to try safe, potentially efficacious, nonsurgical therapy such as botulinum toxin injections or oral medications, or a combination of both. Functionally impaired patients (vision loss, etc.) with blepharospasm who have not tolerated or responded well to medication or botulinum toxin are candidates for surgical therapy.
At present, the myectomy surgery (removal of some or all of the muscles responsible for eyelid closure) has proven to be the most effective surgical treatment for blepharospasm. Current experience has found that myectomy has improved visual disability in 75-80% of cases of blepharospasm.
The myectomy provides the best functional surgical improvement to patients suffering from blepharospasm. By combining the techniques we have learned for cosmetic surgery of the brow and midfacial region , there is excellent functional improvement after myectomy surgery as well as much better cosmesis. The treatment also provides faster healing, is performed as outpatient surgery, and much less morbidity and fewer complications than the original myectomy description. The sharp transition zone between areas of muscle removal and areas in which muscle is not removed is more gradual and acceptable with the newer techniques available. One of the biggest complications of the original myectomy surgery was the chronic lymphedema, or swelling, which lasted for months to sometimes years. The edema and lymphedema have been markedly shortened to several weeks or a few months by these new techniques.
In summary, while the functional results of a myectomy have improved greatly over the past few years, the cosmetic results have also improved and the complications and morbidity associated with the operation have been markedly decreased. The myectomy operation has always been the best surgical treatment for blepharospasm, and by using newer techniques, there has been elimination of many of the negative side effects makes this surgery a much more desirable option to consider when botulinum A toxin is not providing adequate relief of blepharospasm, or if functional or cosmetic deformities of the eyelids are present which prevent the patient from achieving an optimal result.
To sum up, if BOTOX has not provided enough relief for blepharospasm, it is worth considering adding an oral medication. Although in general these medications provide only modest relief, and the response is variable, some patients find them helpful. It is unfortunately a trial-and-error method to find the right combination, and the patient and physician should be vigilant for side effects. Hopefully, research will eventually reveal the cause of BEB, at which point a rational, effective means of therapy will be developed and used.
Blepharospasm is a term that can apply to any abnormal blinking or eyelid tic or twitch due to any cause (from dry eyes to Tourette’s syndrome). In this case, the blepharospasm referred to is officially known as benign essential blepharospasm (BEB), to distinguish it from the less serious secondary blinking disorders.
“Benign” in BEB indicates that the condition is not life threatening. “Essential” is a medical term meaning “of unknown cause.” BEB is both a cranial and focal dystonia. That means the condition is confined to one area of the head, and that it involves abnormal, involuntary sustained muscle contractions and spasms.
Patients with blepharospasm have normal eyes; their visual problems are because of the forced closure of the eyelids.
Symptoms of blepharospasm usually begin gradually with excessive blinking and/or eye irritation. In the early stages, it may only occur with specific stressors, such as bright lights, fatigue, or emotional tension. As the condition progresses, it occurs frequently during the day. The spasms disappear during sleep, and some patients find that the spasms don’t appear for several hours after waking from a good night’s sleep. Concentrating on a specific task may reduce the frequency of the spasms. As BEB progresses, the spasms may intensify so that the patient is functionally blind when spasms occur. The eyelids also might remain forcefully closed for several hours at a time.
Causes: Scientists believe blepharospasm is caused by abnormal functioning o the basal ganglia. The basal ganglia are situated at the base of the brain and play a role in all coordinated movements. Physicians aren’t sure what goes wrong with the basal ganglia to lead to blepharospasm, but it might be related to a disturbance in chemicals that transmit information from one nerve cell to another.
BEB usually develops spontaneously, with no known precipitating factor. The signs and symptoms of dry eye syndrome often precede blepharospasm and might occur in conjunction with BEB. Dry eye might trigger the onset of BEB in susceptible persons, or BEB might occur with dystonia affecting the mouth and/or jaw (known as oromandibular dystonia or Meige syndrome). In these cases, eyelid spasms occur with jaw clenching or mouth opening, grimacing, and tongue protrusion.
Hemifacial spasm (HFS) is a form of BEB that involves spastic muscle contractions of half the patient’s face. HFS often progresses to include some weakness of facial muscles on the same side. Spasms are often so severe that the patient completely closes one eye.
HFS often is caused by irritation to a facial nerve near the brainstem. The irritation most often comes from vascular (blood vessel) compression of the nerve. Patients sometimes experience decreased hearing on the affected side.
In rare cases, HFS can occur bilaterally, meaning it affects both sides of the face. When a patient has bilateral HFS, the two sides do not spasm together.
The hallmark of HFS is the development of spasms before any weakness is apparent.
If Botox injections don’t resolve HFS and the condition worsens, physicians might discuss the possibility of surgery with the patient. Surgical treatment of HFS usually consists of microvascular decompression. The goal of surgery is to move the blood vessel (artery or vein) away from the vulnerable site on the nerve and provide a pad to prevent future compression. Patients usually stay in the hospital for 2 or 3 days following the procedure.
Complications can include infection, brain fluid leak, facial weakness, hearing loss, and stroke. Recovery from microvascular decompression takes approximately six weeks. Up to 95 percent of patients experience spasm relief after microvascular decompression. 90 percent of these patients are spasm free in the first three months after surgery. For others it can take about 18 months to see complete resolution.