What Is Benign Essential Blepharospasm?

Overview

The intricate dance of our eyelids, with their quick and coordinated movements, is a mystery of human biology. These typical simple movements, however, become unpredictable in certain people, resulting in a condition known as benign blepharospasms. Benign Essential Blepharospasm (BEB) involves twitching or spasms of the eyelids. It is a variety of dystonia; a condition of movement where muscular spasms result in twitching or habitual movements. These muscle spasms and contractions take place uncontrollably.¹

Despite being largely benign, this mysterious condition significantly impacts a person’s quality of life. Recognising the symptoms of benign blepharospasm, getting the proper medical care, and developing efficient coping mechanisms are essential to understanding the nuances of this condition. This article aims to bring awareness to this frequently misunderstood condition with the information to face its difficulties and reclaim control over their eye health and general well-being.

Causes of benign essential blepharospasm

The exact cause of BEB is not always clear. However, several factors may contribute to its development. Functional neuroimaging has provided some evidence that the basal ganglia, a collection of brain cells in charge of controlling voluntary movements, are dysfunctional. Though the exact neurological processes that cause blepharospasm are not fully known, it is thought to be caused by a disruption in the complex interplay of neurotransmitters and neuronal circuits that control eyelid movement.²

According to research, benign blepharospasm may have a genetic component (GNAL and CIZ1). Some people may be more likely to develop this illness genetically than others, especially if there is a family history of blepharospasm or other movement disorders.³

There are occasions where benign blepharospasm appears to be impacted by environmental factors, although the specific environmental triggers are still unknown. In some people, blepharospasm symptoms are aggravated by stress, exhaustion, bright lights, and specific medications. The frequency and severity of spasms can be reduced while improving overall illness control by recognising and controlling these triggers.

Signs and symptoms of benign essential blepharospasm

Early intervention of BEB require an understanding of the symptoms. People with BEB frequently experience frequent eye closure, sometimes leading to prolonged closure and significant vision impairment or functional blindness. Moreover, symptoms might include excessive and involuntary blinking and an “eye-twitching” feeling, ranging from mild to severe. The spasms may occasionally be accompanied by discomfort, ocular irritation, and heightened length sensitivity (photophobia). Over time, the symptoms affecting both eyes tend to get worse.¹

Types of BEB

 Depending on the severity, BEB is classified into three different classes:⁵

1. Tics and twitches: The twitch is frequently only felt rather than seen because the muscle spasms are mild.
2. Blepharospasm: It is the more severe kind of eyelid twitch, where the spasms of the eyelid muscles are unconscious and more persistent.
3. Meige’s syndrome: It is also known as Oromandibular dystonia, where all eyes, mouth, and tongue move repetitively and involuntarily.

Diagnosis

BEB diagnosis relies on physical examination, medical history and ruling out other possible causes. Unfortunately, as of now, there is no specific test for BEB. Since BEB shares some features with a variety of other conditions, such as ptosis (droopiness of the eyelids), it can be misdiagnosed or not be recognised at all.^1,2

Neurological tests and evaluations of the frequency and seriousness of eye spasms must support the diagnosis. Electromyography (EMG) or imaging tests may, in some circumstances, be utilised to assess the underlying muscle activity further and rule out other disorders.^4,5

Management and treatment options for BEB

 Several treatment and management options for BEB include:^4,5,7

1. Medical interventions: The effectiveness of several drugs in treating benign blepharospasm symptoms has been investigated. To help lessen muscular spasms and enhance general eyelid control, doctors may prescribe sedatives, muscle relaxants, and anticholinergic medications. However, these drugs may have possible negative effects, and their efficacy can differ from person to person.
2. Botox therapy: Injections of Botox are one of the most popular and efficient therapies for BEB. These injections reduce the frequency and severity of spasms by momentarily weakening the muscles that move the eyelids. To retain their efficacy, Botox treatments often need to be repeated every few months.⁸
3. Surgical Options: Surgical techniques may be considered in extreme situations or when other therapies have not sufficiently relieved the symptoms. Specific muscles or nerves near the eyelids may be selectively removed or repositioned during surgical operations. When less intrusive treatments have failed, and the ailment severely impacts the patient’s quality of life, these procedures are typically the only option.
4. Lifestyle adjustments: Lifestyle changes can help control BEB more effectively. Spasm frequency and intensity can be decreased by getting enough rest and limiting exposure to known triggers (e.g. bright lights). Working with healthcare specialists is crucial to pinpoint the best lifestyle changes for each person.
5. Stress management: Prioritising emotional health and, if necessary, obtaining professional assistance is crucial. Individuals who need assistance navigating the emotional aspects of living with a visible and perhaps socially challenging disability can benefit from mental health treatment, such as therapy or counselling.

FAQs

How common is BEB?

BEB is a relatively rare condition, but it is the most prevalent type of focal dystonia affecting the eyes. The prevalence of BEB is estimated to be around 1 in 20,000 to 1 in 50,000 individuals. However, it is essential to keep in mind that prevalence rates might differ between populations and geographical areas.⁶

Who is most at risk of BEB?

A particular group of people easily affected by the development of BEB includes:^1,6

1. Individuals between 40-70 years old: BEB typically manifests in mid to late adulthood and is rare in younger individuals.
2. Women: BEB is more prevalent in females than men due to hormonal factors and genetic predispositions. 
3. Individuals with a family history of BEB: The possibility of developing BEB can be increased by a history of the condition in the family or other movement disorders.
4. Individuals working in an unhealthy environment: Studies have investigated environmental elements that could influence BEB development. Even though the precise nature of these connections is not well-defined, these factors may include exposure to certain toxins, infections, or other external triggers.

What is the best treatment option for BEB?

The most effective treatment for BEB is an injection of minute doses of botulinum toxin (e.g. Botox or Xeomin) directly into the affected muscles around the eyes responsible for the involuntary spasms. Botox reduces the activity of these muscles and prevents excessive blinking and eyelid closure by momentarily inhibiting the nerve signals that these muscles receive.²

Does Botox injection for BEB need to get retouched?

The treatment is frequently repeated every three to four months or as needed. Other therapies can be considered when botulinum toxin is insufficiently effective. There are various surgical alternatives as well as some medications that can lessen or reduce spasms.²

What to expect from the surgery?

There are several surgical methods for treating BEB, and the specific procedure may vary depending on the surgeon’s experience and the patient’s particular circumstances. Myectomy is the most common surgical procedure that involves the targeted removal or realignment of specific eyelid muscles to lessen muscle spasms. The procedure is typically carried out under local anaesthesia, and it can take some time before the full effects are apparent as the surgical area heals. While some people’s eyelid spasms significantly improved, others might only notice a partial improvement. It is crucial to note that realistic expectations and ongoing open communication with your doctor must be maintained.²

How can I prevent BEB? 

Since BEB's exact origin is unknown, there is no guaranteed technique to prevent it. However, some general considerations could help control the symptoms or lower the risk of BEB.² They include: 

1. Managing a healthy lifestyle.
2. Getting regular eye examinations.
3. Environmental awareness (minimising exposure to bright lights, excessive screen time, etc.). 

When should I see a doctor?

Suppose you are experiencing symptoms such as persistent eye twitching, increased blinking, frequent involuntary eye closure, or any concerns about your eye health; in that case, consult with a healthcare professional such as a neurologist or ophthalmologist (eye specialist) with experience in movement disorder is essential. Note that early diagnosis and intervention are crucial for managing BEB effectively.²

Summary

Benign Essential Blepharospasm (BEB) is a condition characterised by involuntary eyelid spasms, which lowers the quality of life. Although the exact cause is still unknown, it is believed to be affected by abnormalities in the brain cells responsible for voluntary movements, as well as genetic and environmental factors. BEB symptoms include frequent involuntary eye closure, blinking, and discomfort. There are three levels of BEB severity, and to make a correct diagnosis, physical examinations and neurological tests must rule out other causes. Treatment options include medications, Botox injections, surgical approaches in severe cases, lifestyle changes, and stress management. Although there is no guaranteed prevention for BEB, maintaining a healthy lifestyle, getting regular eye exams, and being environmentally conscious can reduce the risk of developing BEB. When experiencing the symptoms of BEB, getting an early consultation with healthcare professionals is essential for effective management.