Blepharospasm
Encyclopedia
A blepharospasm is any abnormal contraction or twitch of the eyelid
.
It normally refers to benign essential blepharospasm, a focal dystonia
—a neurological movement disorder involving involuntary and sustained contractions of the muscles around the eyes. Essential indicates that the cause is unknown, but fatigue, stress, or an irritant are possible contributing factors. Symptoms sometimes last for a few days then disappear without treatment, but in most cases the twitching is chronic and persistent, causing lifelong challenges. The symptoms are often severe enough to result in functional blindness. The person's eyelids feel like they are clamping shut and will not open without great effort. Patients have normal eyes, but for periods of time are effectively blind due to their inability to open their eyelids.
Although strides have recently been made in early diagnosis, blepharospasm is often initially mis-diagnosed as allergies or "dry eye syndrome". It is a fairly rare disease, affecting only one in every 20,000 people in the United States.
Some drugs can induce blepharospasm, such as those used to treat Parkinson's disease
, as well as sensitivity to hormone treatments, including estrogen-replacement therapy for women going through menopause. Blepharospasm can also be a symptom of acute withdrawal from benzodiazepine dependence
. In addition to blepharospasm being a benzodiazepine withdrawal symptom, prolonged use of benzodiazepines can induce blepharospasm and is a known risk factor for the development of blepharospasm.
Blepharospasm may also come from abnormal functioning of the brain basal ganglia
. Simultaneous dry eye and dystonias such as Meige's syndrome
have been observed. Blepharospasms can be caused by concussions in some rare cases, when a blow to the back of the head damages the basal ganglia.
Eyelid
An eyelid is a thin fold of skin that covers and protects an eye. With the exception of the prepuce and the labia minora, it has the thinnest skin of the whole body. The levator palpebrae superioris muscle retracts the eyelid to "open" the eye. This can be either voluntarily or involuntarily...
.
It normally refers to benign essential blepharospasm, a focal dystonia
Dystonia
Dystonia is a neurological movement disorder, in which sustained muscle contractions cause twisting and repetitive movements or abnormal postures. The disorder may be hereditary or caused by other factors such as birth-related or other physical trauma, infection, poisoning or reaction to...
—a neurological movement disorder involving involuntary and sustained contractions of the muscles around the eyes. Essential indicates that the cause is unknown, but fatigue, stress, or an irritant are possible contributing factors. Symptoms sometimes last for a few days then disappear without treatment, but in most cases the twitching is chronic and persistent, causing lifelong challenges. The symptoms are often severe enough to result in functional blindness. The person's eyelids feel like they are clamping shut and will not open without great effort. Patients have normal eyes, but for periods of time are effectively blind due to their inability to open their eyelids.
Although strides have recently been made in early diagnosis, blepharospasm is often initially mis-diagnosed as allergies or "dry eye syndrome". It is a fairly rare disease, affecting only one in every 20,000 people in the United States.
Symptoms
- Excessive blinking and spasming of the eyes, usually characterized by uncontrollable eyelid closure of durations longer than the typical blink reflex, sometimes lasting minutes or even hours.
- Uncontrollable contractions or twitches of the eye muscles and surrounding facial area. Some sufferers have twitching symptoms that radiate into the nose, face and sometimes, the neck area.
- Dryness of the eyes
- Sensitivity to the sun and bright light
Causes
Some causes of blepharospasm have been identified; however, the causes of many cases of blepharospasm remain unknown, although some educated guesses are being made. Some blepharospasm patients have a history of dry eyes and/or light sensitivity, but others report no previous eye problems before onset of initial symptoms. Recent research suggests quite a strong link with cases of syphilis (Hardmann and Jean, 2010), however this is yet to be confirmed.Some drugs can induce blepharospasm, such as those used to treat Parkinson's disease
Parkinson's disease
Parkinson's disease is a degenerative disorder of the central nervous system...
, as well as sensitivity to hormone treatments, including estrogen-replacement therapy for women going through menopause. Blepharospasm can also be a symptom of acute withdrawal from benzodiazepine dependence
Benzodiazepine dependence
Benzodiazepine dependence or benzodiazepine addiction is a condition during which a person is dependent on benzodiazepine drugs. Dependence can be either a psychological dependence, physical dependence, or a combination of the two...
. In addition to blepharospasm being a benzodiazepine withdrawal symptom, prolonged use of benzodiazepines can induce blepharospasm and is a known risk factor for the development of blepharospasm.
Blepharospasm may also come from abnormal functioning of the brain basal ganglia
Basal ganglia
The basal ganglia are a group of nuclei of varied origin in the brains of vertebrates that act as a cohesive functional unit. They are situated at the base of the forebrain and are strongly connected with the cerebral cortex, thalamus and other brain areas...
. Simultaneous dry eye and dystonias such as Meige's syndrome
Meige's syndrome
Meige's syndrome is a type of dystonia. It is also known as Brueghel's syndrome and oral facial dystonia. It is actually a combination of two forms of dystonia, blepharospasm and oromandibular dystonia ....
have been observed. Blepharospasms can be caused by concussions in some rare cases, when a blow to the back of the head damages the basal ganglia.
Treatment
- Drug therapy: Drug therapy for blepharospasm has proved generally unpredictable and short-termed. Finding an effective regimen for any patient usually requires trial and error over time. In some cases a dietary supplement of magnesium chlorideMagnesium chlorideMagnesium chloride is the name for the chemical compounds with the formulas MgCl2 and its various hydrates MgCl2x. These salts are typical ionic halides, being highly soluble in water. The hydrated magnesium chloride can be extracted from brine or sea water...
has been found effective. - Botulinum toxinBotulinum toxinBotulinum toxin is a protein produced by the bacterium Clostridium botulinum, and is considered the most powerful neurotoxin ever discovered. Botulinum toxin causes Botulism poisoning, a serious and life-threatening illness in humans and animals...
injections (Botox is a widely known example) have been used to induce localized, partial paralysis. Among most sufferers, botolinum toxin injection is the preferred treatment method. Injections are generally administered every three months, with variations based on patient response and usually give almost immediate relief (though for some it may take more than a week) of symptoms from the muscle spasms. Most patients can resume a relatively normal life with regular Botulinum toxin treatments. A minority of sufferers develop minimal or no result from Botox injections and have to find other treatments. For some, Botulinum toxin diminishes in its effectiveness after many years of use. An observed side effect in a minority of patients is ptosisPtosisPtosis refers to droopiness of a body part. Specifically, it can refer to:* Ptosis * Ptosis * Enteroptosis * Gastroptosis...
or eyelid droop. Attempts to inject in locations that minimize ptosis can result in diminished ability to control spasms. - Surgery: Patients that do not respond well to medication or botulinum toxin injection are candidates for surgical therapy. The most effective surgical treatment has been protractor myectomy, the removal of muscles responsible for eyelid closure.
- Dark glasses are often worn because of sunlight sensitivity, as well as to hide the eyes from others.
- Stress management and support groups can help sufferers deal with the disease and prevent social isolation.
External links
- Blepharospasm Resource Guide from the National Eye Institute (NEI).
- BEBRF: Benign Essential Blepharospasm Research Foundation Information about blehparospasm