Table of ContentsView AllTable of ContentsSymptomsCausesDiagnosisTreatment
Table of ContentsView All
View All
Table of Contents
Symptoms
Causes
Diagnosis
Treatment
Benign essentialblepharospasmis a progressive neurological disorder and a type ofdystonia, 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.
Verywell / JR Bee
While the condition is not life-threatening (benign), the symptoms of benign essential blepharospasm can greatly affect a person’s quality of life. Theearly symptomsof 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 asMeige syndrome.
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 is not a common condition, occurring in approximately20,000 to 50,000 peoplein the U.S. each year. Most people develop the condition when they are over the age of 50; theaverage age for symptoms to first appearis 56. Women seem to develop benign essential blepharospasm twice as often as men, though the reason for this is not clear.
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 asmultiple sclerosisor Parkinson’s. In some cases, the medications used to treatParkinson’smay cause a person to develop symptoms of blepharospasm.
Benign essential blepharospasm is sometimes confused withtardive 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 theeyelid, though researchers have not made a specific, causative link between injury or disease of the eye and benign essential blepharospasm.
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.
Types of Eye Doctor Specialists
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.
If medications do not work to treat the condition, a doctor may recommend a person with benign essential blepharospasm begin receivingBotulinum toxininjections (Botox), which isFDA-approvedto 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, butcomplications were frequentand could include facial paralysis that lasted for months or years. Due to these risks, the surgery is rarely used today.
Deep brain stimulationis also being investigated as a possible treatment for benign essential blepharospasm.
Managing Symptoms
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.
5 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American Academy of Ophthalmology.Blepharospasm.National Institutes of Health.Benign essential blepharospasm.Hammer M, Abravanel A, Peckham E, et al.Blepharospasm: A genetic screening study in 132 patients.Parkinsonism & Related Disorders. 2019;64:315-318. doi:10.1016/j.parkreldis.2019.04.003Kilic A, Erten E, Ozdemir A.Tardive blepharospasm and meige syndrome during treatment with quetiapine and olanzapine.Arch Neuropsychiatr. 2015;52(2):207-209. doi:10.5152/npa.2015.7411Hassell TJW, Charles D.Treatment of blepharospasm and oromandibular dystonia with botulinum toxins.Toxins. 2020;12(4):269. doi:10.3390/toxins12040269Additional ReadingAmerican Association of Neuromuscular & Electrodiagnostic Medicine.Blepharospasm.Genetics Home Reference. NIH. U.S. National Library of Medicine.Benign essential blepharospasm.NORD (National Organization for Rare Disorders).Benign essential blepharospasm.
5 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.American Academy of Ophthalmology.Blepharospasm.National Institutes of Health.Benign essential blepharospasm.Hammer M, Abravanel A, Peckham E, et al.Blepharospasm: A genetic screening study in 132 patients.Parkinsonism & Related Disorders. 2019;64:315-318. doi:10.1016/j.parkreldis.2019.04.003Kilic A, Erten E, Ozdemir A.Tardive blepharospasm and meige syndrome during treatment with quetiapine and olanzapine.Arch Neuropsychiatr. 2015;52(2):207-209. doi:10.5152/npa.2015.7411Hassell TJW, Charles D.Treatment of blepharospasm and oromandibular dystonia with botulinum toxins.Toxins. 2020;12(4):269. doi:10.3390/toxins12040269Additional ReadingAmerican Association of Neuromuscular & Electrodiagnostic Medicine.Blepharospasm.Genetics Home Reference. NIH. U.S. National Library of Medicine.Benign essential blepharospasm.NORD (National Organization for Rare Disorders).Benign essential blepharospasm.
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
American Academy of Ophthalmology.Blepharospasm.National Institutes of Health.Benign essential blepharospasm.Hammer M, Abravanel A, Peckham E, et al.Blepharospasm: A genetic screening study in 132 patients.Parkinsonism & Related Disorders. 2019;64:315-318. doi:10.1016/j.parkreldis.2019.04.003Kilic A, Erten E, Ozdemir A.Tardive blepharospasm and meige syndrome during treatment with quetiapine and olanzapine.Arch Neuropsychiatr. 2015;52(2):207-209. doi:10.5152/npa.2015.7411Hassell TJW, Charles D.Treatment of blepharospasm and oromandibular dystonia with botulinum toxins.Toxins. 2020;12(4):269. doi:10.3390/toxins12040269
American Academy of Ophthalmology.Blepharospasm.
National Institutes of Health.Benign essential blepharospasm.
Hammer M, Abravanel A, Peckham E, et al.Blepharospasm: A genetic screening study in 132 patients.Parkinsonism & Related Disorders. 2019;64:315-318. doi:10.1016/j.parkreldis.2019.04.003
Kilic A, Erten E, Ozdemir A.Tardive blepharospasm and meige syndrome during treatment with quetiapine and olanzapine.Arch Neuropsychiatr. 2015;52(2):207-209. doi:10.5152/npa.2015.7411
Hassell TJW, Charles D.Treatment of blepharospasm and oromandibular dystonia with botulinum toxins.Toxins. 2020;12(4):269. doi:10.3390/toxins12040269
American Association of Neuromuscular & Electrodiagnostic Medicine.Blepharospasm.Genetics Home Reference. NIH. U.S. National Library of Medicine.Benign essential blepharospasm.NORD (National Organization for Rare Disorders).Benign essential blepharospasm.
American Association of Neuromuscular & Electrodiagnostic Medicine.Blepharospasm.
Genetics Home Reference. NIH. U.S. National Library of Medicine.Benign essential blepharospasm.
NORD (National Organization for Rare Disorders).Benign essential blepharospasm.
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