Blepharospasm

What It Is, Causes, Signs and Symptoms, Treatment, and More

Author: Emily Miao, MD, PharmD

Editor: Alyssa Haag, MD

Editor: Lily Guo, MD

Editor: Kelsey LaFayette, DNP, ARNP, FNP-C

Illustrator: Abbey Richard, MSc

Modified: Jan 06, 2025

What is blepharospasm?

Blepharospasm, also known as benign essential blepharospasm (BEB), is a neurologic disorder characterized by repetitive and involuntary contractions of the eyelid muscles, including the orbicularis oculi muscle, which is responsible for eyelid closure, blinking, and squinting. It is considered a movement disorder called dystonia, which causes abnormal movements and muscle contractions of the body. Blepharospasm is estimated to occur in 20 to 100 out of every 1,000,000 individuals and is twice as common in genetic females than in genetic males.

An infographic detailing the background, causes, signs and symptoms, diagnosis, and treatment of blepharospasm.

What causes blepharospasm?

The exact underlying causes of blepharospasm are poorly understood, but existing theories suggest that it is multifactorial, including genetic, environmental, and functional components. Blepharospasm may be caused by certain inherited gene mutations (e.g., GNAL, CIZ1) which are primarily expressed in the basal ganglia, a brain structure responsible for coordinating and initiating voluntary movement. Generally, more than one of these factors (e.g., genetic, environmental, functional) must be present for the disease to develop. This is also known as the two-hit hypothesis which suggests that a predisposing factor and an environmental trigger must be present to cause disease. For example, an individual can have a genetic predisposition (e.g., family history) to blepharospasm and then experience an environmental trigger (e.g., stress, bright lights, sensory stimuli), which ultimately contributes to the development of the disease.

Other secondary causes of blepharospasm include issues with the facial nerve (e.g., compression) which normally controls eye and face movement and neurologic disorders (e.g., tardive dyskinesia, hemifacial spasm) which may cause eyelid spasms. Tardive dyskinesia is an often irreversible neurologic disorder characterized by involuntary movements of the face (e.g., facial grimacing, lip smacking), eyes (i.e., blepharospasm), tongue (e.g., protrusion), and body parts, which is often due to long-term exposure to dopamine-blocking medications (e.g., haloperidol, metoclopramide) used to treat neuropsychiatric disorders. Additionally, hemifacial spasm is characterized by involuntary muscle contractions on one side of the face, which can also present as blepharospasm if the eyelid muscles are involved.

What are the signs and symptoms of blepharospasm?

Signs and symptoms of blepharospasm include increased blinking or involuntary eye closure, eye irritation, sensitivity to bright light, and increased tearing, resulting in dry eyes. Both eyes are typically affected. These symptoms may increase in frequency with environmental triggers (e.g., fatigue, stress, caffeine consumption). In severe cases, individuals may experience functional blindness due to the persistent contraction of eyelid muscles, which can interfere with vision and activities of daily living. Rarely, the spasms seen in blepharospasm may extend to other muscle groups within the lower face and neck, causing involuntary contractions of these muscle groups (i.e., facial grimacing), also known as Meige syndrome.

How is blepharospasm diagnosed?

The diagnosis of blepharospasm is mainly clinical and it begins with a thorough review of symptoms and medical history. A neuro-ophthalmologic evaluation can help observe the involuntary contractions around the eye and face, and exclude secondary causes of blepharospasm. For example, global involuntary movements may be more suggestive of blepharospasm secondary to tardive dyskinesia; whereas localized facial tonic movements may suggest hemifacial spasm.

The clinical diagnosis is confirmed when the following proposed criteria are met:

(a) Presence of characteristic eye spasms that lead to eyelid narrowing and/or closure and at least one of the following:

(i) Presence of a maneuver (e.g., lightly touching the affected body part) which reduces the dystonic symptoms
(ii) Increased rate of blinking

How is blepharospasm treated?

Treatment of blepharospasm consists of non-pharmacologic, pharmacologic, and surgical options aimed at reducing symptom severity and frequency of eyelid spasms. Non-pharmacologic options include using artificial tears to lubricate the eyes and avoidance of environmental triggers (e.g., bright, flashing lights, caffeine). Injection of botulinum toxin into the affected muscles is highly effective. Botulinum toxin is a neurotoxin that is often used to temporarily weaken muscles by blocking nerve transmission, thereby relaxing the eyelid muscles and reducing excessive twitching. Pharmacotherapy can also be used, including muscle relaxants (e.g., baclofen, tizanidine) however, they are typically not as effective as botulinum toxin. In severe, refractory cases, myectomy, a surgery to remove the portion of the affected eyelid muscle can provide symptom relief. A referral to an ophthalmologist may also ensure comprehensive care.

What are the most important facts to know about blepharospasm?

Blepharospasm, also known as benign essential blepharospasm (BEB), is a neurologic disorder characterized by repetitive and involuntary contractions of the eyelid muscles. Existing theories of the underlying cause suggest that blepharospasm is multifactorial and includes genetic, environmental, and functional components. Secondary causes may include facial nerve disorders, neurologic disorders, and medications. Symptoms include increased blinking or involuntary eye closure, eye irritation, sensitivity to bright light, and tearing, resulting in dry eyes. The diagnosis is clinical and is confirmed when reported symptoms fulfill the proposed criteria. There is currently no cure and treatment is largely supportive care.

References

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Hallett M. Blepharospasm: Recent advances. Neurology. 2002;59(9):1306-1312. doi:10.1212/01.wnl.0000027361.73814.0e

Snir M, Weinberger D, Bourla D, Kristal-Shalit O, Dotan G, Axer-Siegel R. Quantitative changes in botulinum toxin a treatment over time in patients with essential blepharospasm and idiopathic hemifacial spasm. Am J Ophthalmol. 2003;136(1):99-105. doi:10.1016/s0002-9394(03)00075-8

Steeves TD, Day L, Dykeman J, Jette N, Pringsheim T. The prevalence of primary dystonia: A systematic review and meta-analysis. Mov Disord. 2012;27(14):1789-1796. doi:10.1002/mds.25244