Blepharospasm
“Blepharo” means eyelid and “spasm” means uncontrolled muscle contraction. So blepharospasm refers to any uncontrolled and involuntary movement of the eyelid. It can range from abnormally frequent blinking to eyelid twitching to severe contraction of the eyelids such that the patient cannot keep their eyes open.
When is there no cause for it, then it is known as primary blepharospasm. This is also called Benign Essential Blepharospasm (BEB). BEB is a neurological disorder that often starts in middle age. It usually begins with frequent blinking that then progresses to more forced blinking spasms. In many patients, it continues to worsen until treated. In advanced cases, the eyes spasm shut, leading to a form of functional blindness in about 10% of patients.
Some blepharospasm is secondary to other causes that irritate the eyes or eyelids, such as dry eye. This is known as secondary blepharospasm and is a much less serious disorder than BEB.
There is no known cause for BEB.
Whilst it is likely that genetics play a role, it does not generally run in families.
Whilst BEB is commonly associated with anxiety and depression, and often triggered by extreme emotional distress, these are not considered a cause of BEB.
BEB is much more common in women than men, and typically starts in the 40s-60s.
Whilst 20% of patients will only ever have blepharospasm, many others will develop spasms elsewhere in the body, such as the face and neck.
BEB can present in different ways in different patients:
- Increased frequency of blinking
- Eyelid spasms
- Irritation and light sensitivity of the eyes
- Spasm of the mid or lower face
BEB usually affects both eyelids, but can also be unilateral (only affect one eyelid). BEB is often triggered by:
- Stress or fatigue
- Bright lights
- Visual activities such as driving, watching TV or reading
There are no tests to diagnose BEB. The diagnosis is usually made on examination by an experienced doctor trained to recognize the symptoms and signs.
As BEB is a poorly understood disease, it is often misdiagnosed or misinterpreted. Patients have often seen several health professionals and doctors before BEB is correctly diagnosed. Some patients may have had their symptoms passed off as “malingering” (“pretending”) or just that they were tired or stressed.
BEB is a diagnosis of exclusion. This means that other causes for the blepharospasm need to be ruled out first (eg dry eye) before the diagnosis of BEB can be made.
Occasionally tests may be required to rule out other more serious neurological conditions, especially if the blepharospasm is accompanied by other unusual symptoms or signs.
The frequent and prolonged squeezing of the eyelid muscles can lead to other problems such as:
- Drooping of the upper eyelids
- Drooping of the eyebrows
- Excess folds of skin in the upper eyelids
These problems can also block the vision and cause discomfort and may require surgery to fix.
There is no cure for BEB. Botox injections, other oral medications, surgical intervention, patient education and the help of various support groups are all part of a successful program to helping patients manage their blepharospasm.
Botox is by far the most effective treatment for reducing the symptoms of BEB.
Other oral medications (such as anti-Parkinsons, anti-epileptic drugs) have been tried but are not generally as effective as Botox.
Surgery may be indicated in more severe cases where Botox has failed but is much more invasive and rarely required.
Some patients discover certain “tricks” that help them control their own blepharospasm. These include whistling, chewing, humming, mouth-opening, and applying pressure to certain areas of the face.
As bright lights often trigger blepharospasm, wearing dark sunglasses can reduce symptoms.
A healthy lifestyle with plenty of sleep, relaxation and exercise have also been shown to be helpful in managing blepharospasm.
Whilst Botox is best-known as a treatment for wrinkles (“wrinkle-relaxer”), it’s original purpose when first discovered was for the treatment of blepharospasm.
Botox works by turning off the chemicals that pass between nerves and muscles, leading to an inactivation of muscle contraction. Thus, Botox injections will “relax” muscles that are involuntarily contracting or spasming.
In BEB, Botox is injected into the muscles of the eyelids, forehead and cheeks to relax the spasm. Multiple injection sites are required. The dose and site of injections is customized to each patient, as every patient will be different.
The only drawback with Botox therapy is that it is nor permanent. Botox injections need to be repeated at regular intervals, usually every 2-6 months, for the life of the patient.
Botox therapy can be easily and safely applied in the clinic. Side effects and complications are rare, but include:
- Droopy upper eyelid
- Double vision
- Droopy lower eyelid
- Dry eye
As Botox wears off, any side-effects or complications will self-resolve within weeks to months.
Many patients with BEB find support groups very useful in helping them manage this difficult and challenging disease.
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