Blepharospasm

What is blepharospasm?

Blepharospasm is a rare neurological condition which causes the involuntary blinking or spasming of the eyelids. Benign essential blepharospasm can present itself as rapid and frequent blinking, forced eyelid closure, or an inability to open the eyes (apraxia of eyelid opening). Often, it can be a combination of these.

Blepharospasm can be a disabling condition, which can go onto effect other facial muscles. In fact, 30% of patients also experience involuntary tongue, mouth and neck movements. This can give the appearance of lip and mouth movements or tongue and jaw spasm. Benign essential blepharospasm can have a significant impact on an individual’s vision and, occasionally, the muscles of the larynx (voice-box) can become affected, producing a hoarse or whispering voice.

There is, unfortunately, no cure for the condition, but there are many treatment options available. With the right combination of medical and surgical treatments, most patients go on to regain normal functioning of the eye and face muscles.

What causes blepharospasm?

The causes of blepharospasm are complex. There is certainly a genetic predisposition and many patients will report a family history, including chronic irritation of the eyes. This is usually in the form of blepharitis or dry eyes. It is thought that chronic eye irritation can induce a maladaptive reflex response, allowing the normal blinking reflex to assume a life of its own and overwhelm the normal functioning of the blink reflex.

Our recent research at Moorfields Eye Hospital in London has also shown that blepharospasm patients have an underlying abnormality in the nerve layer of the corneal epithelium (the outer layer of the cornea of the eye) and associated decreased sensitivity of the ocular surface. Similar sensory abnormalities have been identified in other dystonias and neurological movement disorders, in which sustained or repetitive muscle contractions result in twisting or abnormal fixed postures. This suggests that decreased sensory stimulation to the brain can cause an input/output mismatch, which can cause blepharospasm.

What are the symptoms of blepharospasm?

In the early stages of the condition, symptoms of blepharospasm are typically the frequent and involuntary blinking of the eye muscles, and eye irritation. Patients may also find themselves experiencing fatigue, emotional tension and sensitivity to bright lights or other environmental factors, like the wind or air pollution.

As the condition develops, symptoms may become more common and it may become increasingly difficult for patients to keep their eyes open, often for several minutes. In severe cases, this can affect vision and can cause a person to become functionally blind.

Over time, spasms in other facial muscles, such as the tongue, mouth and neck, may also occur. Benign essential blepharospasm may therefore be associated with the clenching of the jaw, grimacing or protrusion of the tongue.

In most cases, spasms occur during the daytime, which often means that the problem ceases when a person is asleep. Other activities, such as laughing, singing and yawning, may also temporarily relieve symptoms. On the other hand, activities such as reading, watching TV and driving can cause symptoms to worsen.

Confocal microscopy of the corneal nerve plexus demonstrating abnormalities in patients with blepharopsasm compared with controls.

How is blepharospasm diagnosed?

Blepharospasm is diagnosed by a clinical eye examination by a doctor, usually a neurologist or an ophthalmologist, as well as a discussion of symptoms and patient history.

In some cases, the condition may be difficult to diagnose.

How is blepharospasm treated?

There is currently no cure for blepharospasm, but several treatment options exist to help manage the condition and reduce the severity of symptoms.

Simple treatment measures start with keeping the eyes comfortable by using eye drops, using specialist sunglasses, and occasionally covering one eye, which can help prevent spasms for a short while.

The most effective blepharospasm treatment method is the use of Botulinum toxin type A (BoNT injections). Some neurologists may also use tablets to help control the spasms, but these have a variety of side effects. They are normally only recommended when BoNT therapy has been unsuccessful.

Above all, it is essential that patients are treated in a clinic which has specific expertise in the management of this condition. Daniel Ezra practices at the internationally renowned Moorfields Eye Hospital. This is considered a centre of excellence in the treatment of Blepharospasm, with several hundred patients being treated per year.

The first step in treating blepharospasm is to identify and eliminate any cause for ocular surface irritation. This will be carried out using a full slit lamp biomicroscope examination. This is important because conditions causing irritation of the eye are usually either dry-eye or blepharitis, but they can also include Sjorgren’s syndrome, thyroid eye disease and herpetic keratopathy.

Once identified, the preferred method is the careful injection of Botulinum toxin type A into the overacting facial muscle groups, using a very fine needle. This works to block nerve signals and temporarily relax/weaken the muscles, preventing the spasms.

All patients will have different patterns of spasm and so the injection programme is tailored to each individual. Most patients will get significant relief from undergoing BoNT therapy, reducing the involuntary spasms and helping patients to keep their eyes open.

The BoNT treatment will take effect between 3-5 days after treatment, with the full effect being seen by two weeks. After this, there should be a significant reduction in eyelid spasms.

In addition, if you are found to have brow ptosis (droop), excess upper eyelid skin, or a droopy eyelid, surgery can also be used to correct them.

Is blepharospasm treatment painful?

The BoNT injections may be slightly painful, but most patients are able to handle the discomfort without the need for anaesthetic cream. That being said, if a patient is particularly uneasy about the injections, a topical anaesthetic numbing cream (EMLA) can be applied to the area beforehand.

Ice packs or cold compresses can be applied to the area afterwards and can help with any discomfort.

Will I need repeat blepharospasm treatment?

Yes, if you wish to maintain the effect of treatment, you will need to undergo routine follow-up injections, as the results wear off over time. Most patients tend to undergo repeat treatments around every 3-4 months. As time progresses, some patients find that they are able to extend the amount of time between their next BoNT treatment.

Is BoNT treatment safe?

Yes, BoNT treatment is perfectly safe and there is no risk of developing botulism or food poisoning. Based on the experience of treating thousands of patients (in over three decades at Moorfields Hospital), we are also confident that there is also no risk of developing general health problems after administration.

Moreover, there is no known risk when the treatment is given to women who are pregnant or breastfeeding, although some do prefer to postpone treatment until they have given birth or weaned their children.

Mr Ezra is the clinical lead for the BoNT service at Moorfields Eye Hospital and has extensive experience at treating many conditions, so you will be in very safe hands.

I have tried BoNT, but it is not working, what other blepharospasm treatments are there?

Sometimes BoNT treatment is not successful. This can occur initially or after many years of successful use when the patient develops a resistance to the drug. Fortunately, there are many other approaches to consider:

  • Changing the type of Botulinum toxin - Incorporating different formulations can be used to overcome resistance to a particular strain. There are also new variants that have been proven to be effective.
  • Oral medications - There are several types of treatments which act directly for the brain and you may be asked to try these medications to determine if they are effective.
  • Surgery - There are a variety of surgical treatments that can improve blepharospasm in resistant cases. See below.

Can I undergo surgery for blepharospasm?

Yes, surgery does have a role to play in the management of blepharospasm. Surgery is usually reserved for patients who do not respond adequately to BoNT alone. Our research and audit data indicate that debulking the upper eyelid and normalising the lid and brow positions can improve the condition. Surgery is not an alternative to BoNT. Rather, it should be thought of as a way to improve the response to BoNT.

The following surgical treatments can be used to help manage blepharospasm:

  • Blepharoplasty - To remove excess upper eyelid skin and muscle. It is common to have excess upper lid tissue and debulking this can allow the eye to open better.
  • Orbicularis myectomy - This is a surgical procedure that involves the targeted removal of muscle fibres closest to the eyelid margin, which can have the effect of weakening the eyelid closure muscles.
  • Brow suspension surgery - This is particularly effective for patients with apraxia of eyelid opening and involves internal suspension of the eyelid unit to the brow. This can be a very effective treatment in the appropriate patient group.
  • Direct Brow Lift - Benign Essential Blepharospasm (BEB) patients will often develop brow droop as a result of constant mechanical draw-down of the brow. Lifting the brow tissues to open the eye can have a dramatic impact on disease severity.
  • Ptosis correction - Similarly, the constant blinking of the eyes can weaken the insertion of the eyelid elevator muscles, causing ptosis. This can be addressed by ptosis correction surgery.
 

Hemifacial Spasm

Hemifacial Spasm (HFS) is a condition that is often confused with blepharospasm but has a very different cause and natural history. HFS is an involuntary contraction (or twitch) of the facial muscles, albeit on one side of the face. Hemifacial spasm is usually due to irritation of the facial nerve that controls the movement of muscles around the eyes, eyelids, mouth and lips.

In approximately 90% of cases, eye twitching starts near the eye before gradually progressing down the face. In the remaining 8% to 10% off patients, it begins near the chin and moves up towards the eye area. While the twitching is not painful, it can cause undue embarrassment and self-consciousness as it begins to interfere with normal facial expressions and vision.

Hemifacial spasm is a rare condition in the developed world with as few as 8 in 100,000 people being affected. In the UK alone, it is thought that there are 4,000 who suffer from hemifacial spasm. It typically manifests itself around 45 years of age and is slightly more common in women.

Excessive eye twitching can be caused by an injury to the facial nerve which controls the facial muscles. It may also arise from a tumour or blood vessel which is pressing against the nerve. The compression causes the nerve to misfire and, thereby, causes the facial muscles to contract.

What causes hemifacial spasm?

Excessive eye twitching can be caused by an injury to the facial nerve which controls the facial muscles. It may also arise from a tumour or blood vessel which is pressing against the nerve. The compression causes the nerve to misfire and, thereby, causes the facial muscles to contract.

How is a diagnosis made?

Initially, a neurological examination needs to be carried out. An MRI scan is typically used to rule out other, more serious, conditions such as a brain tumour, aneurysm or brain arteriovenous malformation (AVM). An electromyogram (EMG) study of the face alongside a nerve conduction velocity (NVC) study to measure the nerve and muscle activity.

What Treatments Are Available?

There are a number of effective treatment options available. These include:

1. Medication
Muscle relaxants may be prescribed for mild cases of hemifacial spasm. However, they do cause some side effects including drowsiness, nausea, skin rashes or even dependence. Therefore, patients are carefully monitored and regular blood tests are taken to ensure that excessive levels do not enter into the bloodstream.

2. BoNT Injections
BoNT (otherwise known as BoNT) can be carefully injected into the affected areas in order to paralysis the muscles. It is commonly used around the eyes, mouth and neck with patients reporting improvements within 3 days. The results can also last up to 3 months and the injections can be re-administered on a long-term basis, although its effectiveness can diminish over the years. This is due to the buildup of the antibodies. Side effects include temporary facial weakness, a drooping eyelid and eye sensitivity although many patients feel that the long-term results far outweigh them.

3. Surgery
When the facial spasms and eye twitching persists a surgical procedure called microvascular decompression can be performed. Here a sponge is inserted between the artery and the nerve to relieve nerve compression or pressure and ultimately stop the spasms. Whilat surgery always comes with a risk, the vast majority of patients report significant improvements and are able to return to their regular lifestyle as quickly as 2 months after the procedure.