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Blepharospasm Research
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Blepharo means "eyelid". Spasm means "uncontrolled muscle contraction". The term blepharospasm ['blef-a-ro-spaz-m] can be applied to any abnormal blinking or eyelid tic or twitch resulting from any cause, ranging from dry eyes to Tourette's syndrome to tardive dyskinesia. 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 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
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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.
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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 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. Blepharospasm can be induced by drugs, such as those used to treat Parkinson's disease. When it is due to antiparkinsonian drugs, reducing the
dose alleviates the problem.
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What are the Current Forms of Therapy?
Drug therapy for blepharospasm is difficult. Medications have different mechanisms of action and generally produce unpredictable and short-lasting benefits. One drug may work for some patients and not for others. When the effects of one drug wear off, sometimes the replacement with another drug helps. There is, therefore, no fixed or best regimen. Establishing a satisfactory treatment scheme requires patience on the part of both the physician and the patient.
The following drugs may be tried: Artane (trihexyphenidyl), Cogentin (benztropine), Valium (diazepam), Klonapin (clonazepam), Lioresal (baclofen), Tegretol (carbamazepine), Sinemet or Modopar (levodopa), Parlodel (bromocriptime), and Symmetrel (amantadine). This list is by no means complete, and there are many more new drugs being
developed. The use of these medications requires close supervision from a neurologist, and it is important that the patient does not change the dosage or stop the medications without consulting his/her neurologist.
Additional information about drug treatment
Before surgery is recommended, patients are advised to try safe, potentially efficacious, nonsurgical therapy such as botulinum toxin injections. Functionally impaired patients with blepharospasm who have not tolerated or responded well to medication or botulinum toxin are candidates for surgical therapy. At present, protractor myectomy (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.
Stress makes all movement disorders, including blepharospasm, worse. Some patients may benefit from a course of stress management from an occupational therapist. Patients learn their own coping techniques which they share with others at support group meetings. Dark glasses are the commonest aid. They fulfill two functions. They reduce the intensity of sunlight which bothers many people with blepharospasm, and they hide the eyes from curious onlookers. Unless the patient is receiving treatment that is effective, he/she is at risk for becoming socially isolated. This fear of sustained spasms occurring
while driving, crossing the road, socializing, walking, or taking part in some sporting activity, etc. becomes overwhelming, and the patient stays at home in familiar, safe surroundings. Support from family and friends is important. Thousands of persons are
experiencing the same symptoms. The Benign Essential Blepharospasm Research Foundation has support groups throughout the U.S. and one in Canada. Sharing experiences at support group
meetings will reassure a patient and his/her family. Up-to-date treatments and medical advances are also presented at support group meetings and an annual conference. The Dystonia Medical Research Foundation has
support groups throughout the U.S and Canada for persons with all types of dystonia - not just blepharospasm.
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