blepharospasm

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Spasmodic winking caused by the involuntary contraction of an eyelid muscle.

Definition

Blepharospasm is an involuntary closure of the eyelids.

Description

"Blepharo" refers to the eyelids, and "spasm" to involuntary muscle contraction. In blepharospasm, the eyelids close involuntarily due to an unknown cause within the brain. Blepharospasm is a form of dystonia, a disorder characterized by sustained muscle contraction. The most common form of blepharospasm is called "benign essential blepharospasm," meaning it is not life threatening and is not due to some other identifiable disorder. A condition called hemifacial spasm causes similar symptoms, but affects only one side of the face, and is caused by an irritation of the facial nerve outside of the brain.

Demographics

Blepharospasm is estimated to affect approximately 15,000 people in the United States. Onset is most commonly between the ages of 40 and 60, but can begin in childhood or old age. Women are affected approximately twice as often as men.

Causes and symptoms

The cause of benign essential blepharospasm is unknown. Evidence suggests it may be genetic in some cases, although genes have not been identified. A person with blepharospasm often has dystonia in another region of the body such as the mouth or the hands (i.e., writer's cramp). Other forms of dystonia or tremor may affect other family members. Blepharospasm is not caused by a problem with the eyes themselves, but rather with the brain regions controlling the muscles of the eyelids.

Secondary blepharospasm occurs due to some identifiable cause. The most-common cause of secondary blepharospasm is a reaction to antipsychotic medications, and is called tardive dystonia. Damage to the brain, either through stroke, multiple sclerosis, or trauma, may also cause blepharospasm.

Blepharospasm often begins with increased frequency of blinking, which may be accompanied by a feeling of irritation in the eyes or "dry eye." It progresses to intermittent, and then sustained, forceful closure of the eyelids. Symptoms are usually worse when the patient is tired, under stress, or exposed to bright light. Symptoms may become severe enough to interfere with activities of daily living, and can render the patient functionally blind.

Diagnosis

Blepharospasm is diagnosed by a careful clinical exam. A detailed medical history is taken to determine exposure to drugs or other possible causative agents, and a family history is used to determine if other family members are affected by other forms of dystonia or tremor.

Treatment team

The treatment team consists of a neurologist and possibly a neurosurgeon.

Treatment

The most effective treatment for blepharospasm is injection of botulinum toxin (BTX) into the muscles controlling the eyelids. BTX temporarily prevents the muscles from contracting, allowing patient to keep their eyes open. BTX is a safe and effective treatment for this condition. Usually the effects are seen within several days of injection, have their maximum effect for 6–8 weeks, and last between 12 and 16 weeks, at which time reinjection is performed. Side effects of BTX injection include mild discomfort at the injection site(s), and occasional double vision or inability to lift the eyelids due to local spread of the toxin to other muscles. Dry eyes or excessive tearing may also occur. Development of resistance to BTX injections is possible if the patient's immune system creates antibodies against the toxin. While this has not been reported in blepharospasm as the injected dose is very low, it has occurred in other conditions in which the doses are higher.

Oral medications are rarely effective for blepharospasm. Among the most widely used are anti-cholinergics (trihexyphenidyl, benztropine), baclofen, and benzodiazepines (diazepam, clonazepam). Surgery is an option for patients who do not respond to BTX injections. The surgical procedures are performed to remove part of the overactive muscles, or to sever the nerve leading to them, or both. Unfortunately, surgery is rarely completely successful, and there is a high rate of recurrence of blepharospasm.

Clinical trials

There are no current clinical trials for blepharospasm since effective treatment is available.

Prognosis

Blepharospasm is a chronic condition, which tends to worsen over time. Many patients with blepharospasm develop other dystonias in other body regions.

Resources

WEBSITES

Benign Essential Blepharospasm Research Foundation. (April 19, 2004.) http://www.blepharospasm.org/.

WE MOVE. (April 19, 2004.) http://www.wemove.org.

Richard Robinson

Spasm of the orbicularis oculi muscle of the eyelid.

This article needs additional citations for verification. Please help improve this article by adding reliable references. Unsourced material may be challenged and removed. (February 2009)

Blepharospasm
Classification and external resources
Left orbicularis oculi, seen from behind.
ICD-10	G24.5
ICD-9	333.81
DiseasesDB	15748
eMedicine	oph/202
MeSH	D001764

A blepharospasm (from Greek: blepharo, eyelid, and spasm, an uncontrolled muscle contraction), is any abnormal tic or twitch of the eyelid.

It normally refers to benign essential blepharospasm, a focal dystonia—a neurological movement disorder involving involuntary and sustained muscle contractions of the muscles around the eyes. Benign means the condition is not life threatening. Essential indicates that the cause is unknown, but fatigue, stress, or an irritant are possible contributing factors. Symptoms sometimes last for a few days then disappear without treatment, but in most cases the twitching is chronic and persistent, causing lifelong challenges. The symptoms are often severe enough to result in functional blindness. The person's eyelids feel like they are clamping shut and will not open without great effort. Patients have normal eyes, but for periods of time are effectively blind due to their inability to open their eyelids.

Although strides have recently been made in early diagnosis, blepharospasm is often initially mis-diagnosed as allergies or "dry eye syndrome". It is a fairly rare disease, affecting only one in every 20,000 people in the United States.

Contents 1 Symptoms 2 Causes 3 Treatment 4 See also 5 References 6 External links

Symptoms

• Excessive blinking and spasming of the eyes, usually characterized by uncontrollable eyelid closure of durations longer than the typical blink reflex, sometimes lasting minutes or even hours.
• Uncontrollable tics or twitches of the eye muscles and surrounding facial area. Some sufferers have twitching symptoms that radiate into the nose, face and sometimes, the neck area.
• Dryness of the eyes
• Sensitivity to the sun and bright light^[1]

Causes

Some causes of blepharospasm have been identified, however, the causes of many cases of blepharospasm remains unknown, although some educated guesses are being made. Some blepharospasm patients have a history of dry eyes and/or light sensitivity, but others report no previous eye problems before onset of initial symptoms.

Some drugs can induce blepharospasm, such as those used to treat Parkinson's disease, as well as sensitivity to hormone treatments, including Estrogen replacement therapy for women going through Menopause. Blepharospasm can also be a symptom of acute withdrawal from benzodiazepine dependence. In addition to blepharospasm being a benzodiazepine withdrawal symptom, prolonged use of benzodiazepines can induce blepharospasm and is a known risk factor for the development of blepharospasm.^[2]

Blepharospasm may also come from abnormal functioning of the brain's basal ganglia. Concomitance with dry eye, as well as other dystonias such as Meige's syndrome has been observed. Blepharospasms can be caused by concussions in some rare cases, when a blow to the back of the head damages the basal ganglia.

Treatment

• Drug therapy: Drug therapy for blepharospasm has proved generally unpredictable and short-termed. Finding an effective regimen for any patient usually requires trial and error over time. In some cases a dietary supplement of magnesium chloride has been found effective.
• Botulin toxin (Botox) injections have been used, to induce localized, partial paralysis. Among most sufferers, botox is the preferred treatment method.^[3] Injections are generally administered every three months, with variations based on patient response and usually give almost immediate relief (though for some it may take more than a week), from the spasming. Most patients can resume a relatively normal life with regular Botox treatments. A minority of sufferers get minimal or no result from Botox injections and have to find other treatments. For some, Botox diminishes in its effectiveness after many years of use. An observed side effect in a minority of patients is ptosis or eyelid droop. Attempts to inject in locations that minimize ptosis can result in diminished ability to control spasms.
• Surgery: Patients that do not respond well to medication or botulinum toxin injection are candidates for surgical therapy. The most effective surgical treatment has been protractor myectomy, the removal of muscles responsible for eyelid closure^[4].
• Dark glasses are often worn because of sunlight sensitivity, as well as to hide the eyes from others.
• Stress management and support groups can help sufferers deal with the disease and prevent social isolation.

See also

• Levator palpebrae superioris muscle

References

1. ^ Adams WH, Digre KB, Patel BC, Anderson RL, Warner JE, Katz BJ. Center for Facial Appearances, Department of Ophthalmology and Visual Sciences, University of Utah Health Sciences Center, Salt Lake City, Utah 84132, USA. http://www.ncbi.nlm.nih.gov/pubmed/16815254?dopt=AbstractPlus
2. ^ Wakakura M, Tsubouchi T, Inouye J (March 2004). "Etizolam and benzodiazepine induced blepharospasm" (PDF). J. Neurol. Neurosurg. Psychiatr. 75 (3): 506–7. PMID 14966178. PMC: 1738986. http://www.pubmedcentral.nih.gov/picrender.fcgi?artid=1738986&blobtype=pdf. 
3. ^ Schellini SA, Matai O, Igami TZ, Padovani CR, Padovani CP. Faculdade de Medicina de Botucatu, Universidade Estadual Paulista Julio Mesquita Filho, Botucatu, SP, Brazil. http://www.ncbi.nlm.nih.gov/pubmed/16491229?ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_DiscoveryPanel.Pubmed_Discovery_RA&linkpos=5&log$=relatedreviews&logdbfrom=pubmed
4. ^ Ophthal Plast Reconstr Surg. 1998 Sep;14(5):305-17. Research study finding the percent of patients improved was full myectomy 88%, botulinum-A toxin 86%, and drug therapy 43%. The full myectomy is reserved for botulinum-A toxin failures, and the limited myectomy is an excellent adjunct to botulinum-A toxin. Anderson RL, Patel BC, Holds JB, Jordan DR. Center for Facial Appearances, Department of Ophthalmology, John A. Moran Eye Center, University of Utah, Salt Lake City 84102, USA.

External links

• Blepharospasm Resource Guide from the National Eye Institute (NEI).
• BEBRF: Benign Essential Blepharospasm Research Foundation Information about blehparospasm

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