WHAT IS IT?

Also known as hemifacial spasm and blepharospasm

The word blepharo means “eyelid”, and spasm refers to “uncontrolled muscle contraction”. The term blepharospasm can be applied to any abnormal blinking or eyelid tic or twitch resulting from any cause, ranging from common dry eyes to neurologic conditions such as Tourette’s syndrome and tardive dyskinesia. The blepharospasm that is often encountered is sometimes called benign essential blepharospasm (BEB) to distinguish it from other blinking disorders. “Benign” indicates that the condition is not life threatening, and “essential” is a medical term meaning “of unknown cause”. Patients with blepharospasm have normal eyes. The visual disturbance is due solely to the forced closure of the eyelids, where patients are functionally blind when the spasm is occurring.

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.  An MRI of the brain should be done in cases of hemifacial spasm to rule out treatable causes of the spasm.

In rare cases, heredity may play a role in the development of blepharospasm.

WHO GETS IT?

Essential blepharospasm affects women about twice as commonly as men.  It is more common in older age and typically develops in the sixth decade.  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 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.

TREATMENTS AVAILABLE

General Measures

  • Blepharospasm can be a reflex reaction to an underlying disease (most commonly, ocular surface disease like dry eye) and this needs to be ruled out/managed before drugs are considered for the blepharospasm.
  • Wearing dark glasses can reduce bright light triggers and prevent embarrassment due to the stares of onlookers.
  • Voluntary manoeuvres, such as pulling the eyelid, pinching the neck, talking, yawning, humming and singing, help some sufferers.
  • Patients with severe blepharospasm must not drive. Those with mild or well-controlled blepharospasm may drive subject to a doctor’s approval.

Drugs

  • Blepharospasm does not respond well to medication, and usually produce unpredictable results. Any symptom relief is usually short term and tends to be helpful in only 15 percent of the cases.
  • Preferred treatment is injection of botulinum toxin type A into eyelid muscles. This treatment has been found to be highly effective, helping up to 90% of patients.  Botulinum toxin, produced by the bacterium Clostridium botulinum, paralyzes the muscles of the eyelids. This effect is, however, temporary, and twitching will inevitably return after 3 to a maximum of 6 months.  Not repeating the injections does not make the condition worse than before, nor do repeat injections make a patient dependent on it.  Side effects are minimal and patients usually report good results after Botox injections.

Surgery

Where vision is severely impaired by prolonged, severe eye closure, unresponsive to pharmacological techniques, protractor myomectomy may be used (removal of some muscles of eye closure). Its use will only be considered as a last resort.

Alternative treatments may include biofeedback, acupuncture, hypnosis, chiropractic, and nutritional therapy. The benefits of these alternative therapies have not been proven.

Most cases of benign essential blepharospasm cannot be cured although symptomatic relief is good with botulinum toxin injections. The long-term safety and efficacy of this drug appears to be excellent.

EYELID TWITCHING / MYOKYMIA

Eyelid twitching or eyelid myokymia refers to involuntary fine contractions of the eyelid muscle without muscular wasting or weakness. Eyelid myokymia is typically one-sided and involves the orbicularis oculi muscle of one of the lower eyelids; occasionally, one of the upper eyelids can be affected. In most cases, eyelid myokymia is benign, self-limited, and not associated with any disease. Intervention is usually unnecessary.

Possible precipitating factors include stress, fatigue, and excessive caffeine or alcohol intake.