Blepharospasm
What is it?
Blepharo means “eyelid. Spasm means “uncontrolled muscle contraction”. 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. Blepharospasm is a focal dystonia characterised by increased blinking and involuntary closing of the eyes. This form may be primary or secondary. People with blepharospasm have normal vision. Visual disturbance is due solely to the forced closure of the eyelids. Blepharospasm may be referred to as a ‘cranial dystonia’. Cranial dystonia is a broad description of dystonia that affects any part of the head.
Terms used to describe blepharospasm include: eye dystonia, cranial dystonia, adult onset focal dystonia. When blepharospasm occurs with dystonia in the face, the term Meige’s syndrome may be used.
Symptoms
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). When blepharospasm and oromandibular dystonia occur together, the condition may be referred to as Meige’s syndrome. In such cases, spasms of the eyelids are accompanied by jaw clenching or mouth opening, grimacing, and tongue protrusion.
Cause
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.
Blepharospasm may develop spontaneously with no known precipitating factor or be inherited. Some people with blepharospasm have family members with dystonia affecting different body areas.
Blepharospasm may be secondary due to drug exposure or occur with other disorders such as, parkinsonian syndromes, and Wilson’s disease.
Diagnosis
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.
Treatment
Blepharospasm should only be diagnosed and treated by an Ophthalmologist or a Neurologist specialising in movement disorders.
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 with a small fine needle intramuscularly into several sites above and below the eyes. The sites of the injection vary slightly from person to person and according to the Ophthalmologist or Neurologist. 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.
Medications
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 peoples symptoms improved with oral medications such as Rivotril (clonazepam), Artane (benzhexol), and Lioresal (baclofen), but the degree of improvement is usually unsatisfactory at the expense of side effects.
Surgery
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.
Complementary Therapy/ Coping Strategies
Wear sunglasses, type that wrap around to cut wind and glare
Wear a hat with a brim or visor, tight baseball cap , hair band or sweat band around the forehead
Sit facing away from windows
Place warm or cold compresses on the eye
Wear tinted glasses
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 chewing gum, talking, whistling, humming, sucking on a straw, yawning, singing, sleeping, reading aloud, or looking down. Touching temple or forehead. Put you head back – close your eyes or look down Go into a dark place and try to relax.
Different sensory tricks work for different people, and if a person finds a sensory trick that works, it usually continues to work.
For more detailed information please contact Benign Essential Blepharospasm Research Foundation www.blepharospasm.org
Support
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. Sharing experiences at support group meetings offers encouragement, camaraderie, and the latest information about new treatments and medical advances.