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What is it?
Blepharospasm is a focal dystonia characterised by increased blinking and involuntary closing of the eyes. People with blepharospasm have normal vision. Visual disturbance is due solely to the forced closure of the eyelids.
Blepharospasm affects the eye muscles and 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. It is almost always present in both eyes.
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, spasms do not appear for several hours after waking.
In a few cases, spasms may intensify so that the eyelids remain forcefully closed for several hours at a time.
Blepharospasm can occur with dystonia affecting the mouth and/or jaw (oromandibular dystonia, Meige’s Syndrome). In such cases, spasms of the eyelids are accompanied by jaw clenching or mouth opening, grimacing, and tongue protrusion.
If blepharospasm causes any type of impairment, it is because muscle contractions interfere with normal function. Features such as cognition, strength, and the senses, including vision and hearing, are normal. While dystonia is not fatal, it is a chronic disorder and prognosis is difficult to predict.
Blepharospasm is believed to be due to abnormal functioning of the basal ganglia, which are deep brain structures involved with the control of movement. The basal ganglia assist in initiating and regulating movement. What goes wrong in the basal ganglia is still unknown. An imbalance of dopamine, a neurotransmitter in the basal ganglia, may underlie several different forms of dystonia, but much more research needs to be done for a better understanding of the brain mechanisms involved with dystonia.
Though a history of eye trauma may be obtained in some persons, the relationship between trauma and blepharospasm has not been established. In most people it develops spontaneously with no known precipitating factor. Cases of inherited blepharospasm have been reported, usually in conjunction with early-onset generalised dystonia, which is associated with the DYT1 gene.
Blepharospasm may be rarely secondary or symptomatic, occurring in association with other disorders such as tardive dystonia, parkinsonian syndromes, and Wilson’s disease.
Diagnosis of blepharospasm is based on information from the affected individual and the physical and neurological examination. At this time, there is no test to confirm diagnosis of blepharospasm, and, in most cases, laboratory tests are normal.
Blepharospasm should not be confused with:
• Ptosis – drooping of the eyelids caused by weakness or paralysis of a levator muscle of the upper eyelid.
• Blepharitis – an inflammatory condition of the lids due to infection or allergies.
• Hemifacial spasm – a non-dystonic condition involving various muscles on one side of the face, often including the eyelid, and caused by irritation of the facial nerve. The muscle contractions are more rapid and transient than those of blepharospasm, and the condition is always confined to one side.
Currently there is no cure for dystonia, but treatments are available to help to ease the symptoms related to the disorder. Working with your doctor, an individualized strategy for treatment can be developed.
The approach for treatment of dystonia is usually three-tiered: oral medications, botulinum toxin injections, and surgery. These therapies may be used alone or in combination. Complementary care may also have a role in the treatment management depending on the form of dystonia, and supportive therapy may provide an important adjunct to medical treatment.
A multitude of drugs has been studied to determine benefit for people with blepharospasm, but none appears to be uniformly effective.
About one-third of people’s symptoms improved when treated with oral medications such as Rivotril (clonazepam), Artane (benzhexol), and Lioresal (baclofen), but the degree of improvement is usually unsatisfactory and at the expense of side effects.
Botulinum toxin injections are the primary and most effective form of treatment for blepharospasm. Botulinum toxin is a therapeutic muscle-relaxing agent that helps reduce the uncontrollable muscular contractions associated with dystonia. It is injected into specific muscles where it acts to weaken muscle activity sufficiently to reduce a spasm but not enough to cause paralysis.
Minute doses of the toxin are injected intramuscularly into several sites above and below the eyes. The sites of the injection vary slightly from person to person and according to physician preference. The most common places are the corners of each eyelid, under the lids, to the side of the eye, and in the brow.
Benefits can begin in one to fourteen 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 are infrequent and transient and include drooping of the eyelid, blurred vision, tearing, and double vision. All recover spontaneously.
The myectomy surgery can provide functional surgical improvement for persons with blepharospasm. Surgery is usually recommended after nonsurgical therapies such as medication or botulinum toxin injections are tried and yield little or no benefit. The myectomy removes nearly all of the squeezing muscles of the upper eyelids. These muscles are removed through a brow incision and an eyelid crease incision. The post-operative use of botulinum toxin may be needed as an adjunct to this treatment.
Dark glasses are a common aid for people with blepharospasm as they reduce the intensity of sunlight which bothers many people with blepharospasm.
The use of sensory tricks may also be effective in dealing with blepharospasm. Some of the most common “tricks” are pulling on the upper eyelid, pinching the neck, talking, humming, yawning, singing, sleeping, reading, or looking down. Different sensory tricks work for different people, and if a person finds a sensory trick that works, it usually continues to work.
Dystonia and its emotional offshoots affect every aspect of a person’s life - how we think, the way we act, and how we cope. By educating yourself with information, you have taken the first step in dealing with dystonia.
Stress is an inevitable part of life, and although it clearly does not cause dystonia, it can aggravate dystonia symptoms. Stress-reduction programmes such as relaxation techniques, meditation, and journal writing may be beneficial.
Sometimes depression can be a byproduct of dystonia. Depression may aggravate symptoms and make them worse, but, often, treating depression can result in an improvement of dystonia. It is important to remember that depression is a disorder; it is treatable and not a reflection of one’s self.
Many people are experiencing similar symptoms. Reassurance from family, friends, and others who have dystonia is beneficial. Dystonia Ireland has a support group in Dublin and it is our intention eventually to set up similar groups throughout Ireland. Sharing experiences at support group meetings offers encouragement, camaraderie, and the latest information about new treatments and medical advances.
With written permission, this information is reproduced from materials published and copyrighted by the Dystonia Medical Research Foundation, Chicago, IL, USA www.dystonia-foundation.org