An Overview of Benign Essential Blepharospasm

Benign essential blepharospasm is a progressive neurological disorder and a type of dystonia, or abnormal muscle posture. In benign essential blepharospasm, uncontrolled muscle contractions cause the eyelids to close involuntarily. When severe, the condition can cause a person to become functionally blind. While there is no cure, several treatments are available to help manage symptoms and progression.

Symptoms

While the condition is not life-threatening (benign), the symptoms of benign essential blepharospasm can greatly affect a person's quality of life. The early symptoms of the condition usually come on gradually and can include:

Symptoms may be more noticeable when a person is tired or upset.

At first, the symptoms of benign essential blepharospasm may only be felt in one eye, but the condition almost always involves both eyes as the condition worsens.

As the disorder progresses, a person with benign essential blepharospasm usually begins to notice symptoms are only present when they are awake. Sometimes, a good night's sleep delays the onset of symptoms the next day. A person may also notice symptoms are less pronounced when they are very focused on a task.

Eventually, a person with benign essential blepharospasm experiences such frequent and intense closure of their eyelids that they may become functionally blind. Functional blindness refers to the fact that even though a person's vision is normal, they are unable to see because they are unable to control the closure of their eyes.

At times the eyes may remain closed for several hours. This can seriously interfere with a person's ability to perform many tasks of daily life, such as reading and driving.

More than half of people with benign essential blepharospasm will eventually experience the symptoms of dystonia in other parts of the body, usually in the mouth, face, or neck. When the uncontrolled movements spread to these areas, the condition is sometimes referred to as Meige syndrome.

Causes

The cause of benign essential blepharospasm is unknown. It is not uncommon for people with no family history or known risk factors to develop the condition. Overall, benign essential blepharospasm likely develops due to a combination of many factors.

Benign essential blepharospasm usually occurs sporadically. However, some cases have been seen in families. Therefore, it's possible genetics play a role, though researchers have yet to link benign essential blepharospasm to a specific gene. Researchers also suspect the condition could be related to dysfunction in the part of the brain that controls movement (basal ganglia).

Other factors, such as environmental exposures, could trigger the disorder in people who are predisposed to developing it.

A person may be more likely to develop benign essential blepharospasm if they have another form of dystonia or conditions that cause similar symptoms, such as multiple sclerosis or Parkinson's. In some cases, the medications used to treat Parkinson's may cause a person to develop symptoms of blepharospasm.

Benign essential blepharospasm is sometimes confused with tardive dyskinesia, another movement disorder that can occur when people take certain medications. The two conditions can look similar if a person with benign essential blepharospasm has symptoms in their face and neck rather than just the eyes. However, tardive dyskinesia doesn't usually cause the eyes to completely, involuntarily close, which can help doctors distinguish between the two disorders when trying to make a diagnosis.

It's possible that a person who has experienced a trauma or injury to the eye may be more likely to develop dystonia affecting the eyelid, though researchers have not made a specific, causative link between injury or disease of the eye and benign essential blepharospasm.

Diagnosis

There is no specific marker that can be tested for when diagnosing benign essential blepharospasm. There are currently no imaging or lab tests that can be used to diagnose the condition.

Benign essential blepharospasm is usually diagnosed after a doctor has considered a patient's medical history and performed a thorough physical exam. The diagnosis is usually confirmed by a specialist, such as a neurologist or an ophthalmologist, when other causes for a person's symptoms have been ruled out.

Treatment

The symptoms of benign essential blepharospasm can greatly disrupt a person's life. While there is no cure for the condition, there are several options for treatment.

When a person is first diagnosed with benign essential blepharospasm, their doctor may recommend they start by trying oral medication to treat the condition. The class of medication initially offered is typically anticholinergic drugs like Thorazine, which are also used in the treatment of Parkinson's and other movement disorders. Benzodiazepines, such as Klonopin or Ativan, antihistamines, and anticonvulsants may also be offered.

If medications do not work to treat the condition, a doctor may recommend a person with benign essential blepharospasm begin receiving Botulinum toxin injections (Botox), which is FDA-approved to treat benign essential blepharospasm. Many medical professionals will recommend Botox injections as a first-line treatment for patients with benign essential blepharospasm. People usually need to get Botox injections on a regular schedule, such as every three months, in order to manage the condition.

Not every person with benign essential blepharospasm will respond well to medications or treatment with Botox injections. In some cases, the condition is severe enough that a doctor may suggest surgery. A procedure to remove part or all of the muscles that control the eyelid (protractor myectomy) may be needed if a person is experiencing severe symptoms that cannot be controlled with medication and/or Botox injections.

In the past, benign essential blepharospasm was sometimes treated with a surgical procedure to remove part of the facial nerves (neurectomy) that surround the eye, but complications were frequent and could include facial paralysis that lasted for months or years. Due to these risks, the surgery is rarely used today.

Deep brain stimulation is also being investigated as a possible treatment for benign essential blepharospasm.

A Word From Verywell

Benign essential blepharospasm is a rare progressive neurological disorder that causes uncontrolled, forceful closure of the eye. When severe, the condition can cause a person to become functionally blind. The condition is most commonly seen in middle-aged women. The exact cause of benign essential blepharospasm is not known, but researchers believe it probably happens due to many genetic, environmental, and other factors. Most people who get benign essential blepharospasm don't have a family history or any known risk factors. There is no cure for the disorder but it can be treated with medication, Botox injections, and in severe cases, surgery.