Blepharospasm is any abnormal contraction of the

orbicularis oculi muscle The orbicularis oculi is a muscle in the face that closes the eyelids. It arises from the nasal part of the frontal bone, from the frontal process of the maxilla in front of the lacrimal groove, and from the anterior surface and borders of a short ...

. The condition should be distinguished from the more common, and milder, involuntary quivering of an eyelid, known as

myokymia Myokymia is an involuntary, spontaneous, localized quivering of a few muscles, or bundles within a muscle, but which are insufficient to move a joint. One type is superior oblique myokymia. Myokymia is commonly used to describe an involuntary eye ...

, or

fasciculation A fasciculation, or muscle twitch, is a spontaneous, involuntary muscle contraction and relaxation, involving fine muscle fibers. They are common, with as many as 70% of people experiencing them. They can be benign, or associated with more seri ...

. In most cases, blepharospasm symptoms last for a few days and then disappear without treatment, but in some cases the twitching is chronic and persistent, causing life-long challenges. In these cases, 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. People have normal eyes, but for periods of time are effectively blind due to their inability to open their eyelids. In contrast, the reflex blepharospasm is due to any pain in and around the eye. It is of two types: essential and reflex blepharospasm. The benign essential blepharospasm (BEB) is a

focal dystonia Focal dystonia, or focal task specific dystonia, is a neurological condition, a type of '' dystonia'', that affects a muscle or group of muscles in a specific part of the body during specific activities, causing involuntary muscular contractions an ...

—a neurological movement disorder involving involuntary and sustained contractions of the muscles around the eyes. The term ''essential'' indicates that the cause is unknown, but fatigue, stress, or an irritant are possible contributing factors. Blepharospasm is sometimes part of

benign fasciculation syndrome Benign fasciculation syndrome (BFS) is characterized by fasciculation (twitching) of voluntary muscles in the body. The twitching can occur in any voluntary muscle group but is most common in the eyelids, arms, hands, fingers, legs, and feet. The ...

. Although there is no cure,

botulinum toxin Botulinum toxin, or botulinum neurotoxin (BoNT), is a neurotoxic protein produced by the bacterium ''Clostridium botulinum'' and related species. It prevents the release of the neurotransmitter acetylcholine from axon endings at the neurom ...

injections may help temporarily. A surgical procedure known as myectomy may also be useful. BEB is a fairly rare disease, affecting only one in every 20,000 people in the United States. The word is from Greek: βλέφαρον / blepharon, eyelid, and σπασμός / spasmos, ''

spasm A spasm is a sudden involuntary contraction of a muscle, a group of muscles, or a hollow organ such as the bladder. A spasmodic muscle contraction may be caused by many medical conditions, including dystonia. Most commonly, it is a muscle c ...

,'' an uncontrolled muscle contraction.

Signs and symptoms

* Excessive blinking and spasming of one or both eyes – characterized by uncontrollable eyelid closure of durations longer than the typical blink reflex. The spells of spasming may last for 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, cheeks, 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. Some people with blepharospasm have a history of

dry eyes Dry eye syndrome (DES), also known as keratoconjunctivitis sicca (KCS), is the condition of having dry eyes. Other associated symptoms include irritation, redness, discharge, and easily fatigued eyes. Blurred vision may also occur. Symptoms rang ...

, light sensitivity, and even

fatigue Fatigue describes a state of tiredness that does not resolve with rest or sleep. In general usage, fatigue is synonymous with extreme tiredness or exhaustion that normally follows prolonged physical or mental activity. When it does not resolve ...

. Others report no eye problems before onset of symptoms. Some drugs can induce blepharospasm, such as those used to treat

Parkinson's disease Parkinson's disease (PD), or simply Parkinson's, is a long-term degenerative disorder of the central nervous system that mainly affects the motor system. The symptoms usually emerge slowly, and as the disease worsens, non-motor symptoms beco ...

, as well as hormone treatments, including estrogen-replacement therapy for women going through menopause. Blepharospasm can also be a symptom of acute withdrawal from benzodiazepines. Prolonged use of

benzodiazepines Benzodiazepines (BZD, BDZ, BZs), sometimes called "benzos", are a class of depressant drugs whose core chemical structure is the fusion of a benzene ring and a diazepine ring. They are prescribed to treat conditions such as anxiety disorders, ...

can induce blepharospasm and is a known risk factor for the development of blepharospasm. Blepharospasm may also come from abnormal functioning of the brain's

basal ganglia The basal ganglia (BG), or basal nuclei, are a group of subcortical nuclei, of varied origin, in the brains of vertebrates. In humans, and some primates, there are some differences, mainly in the division of the globus pallidus into an ext ...

. Simultaneous dry eye and

dystonia Dystonia is a neurological hyperkinetic movement disorder in which sustained or repetitive muscle contractions result in twisting and repetitive movements or abnormal fixed postures. The movements may resemble a tremor. Dystonia is often int ...

s such as Meige's syndrome have been observed. Blepharospasms can be caused by

concussion A concussion, also known as a mild traumatic brain injury (mTBI), is a head injury that temporarily affects brain functioning. Symptoms may include loss of consciousness (LOC); memory loss; headaches; difficulty with thinking, concentration ...

s in some rare cases, when a blow to the back of the head damages the basal ganglia.

Multiple sclerosis Multiple (cerebral) sclerosis (MS), also known as encephalomyelitis disseminata or disseminated sclerosis, is the most common demyelinating disease, in which the insulating covers of nerve cells in the brain and spinal cord are damaged. This ...

can cause blepharospasm.

Treatment

Drug therapy for blepharospasm has proved generally unpredictable and short-termed. Anticholinergics, tranquillizing drugs and botulinum toxin are the mostly used therapeutic options. However serious side effects can be observed as well as failure of therapy. It is therefore not surprising that new therapies are constantly being tested. In this backdrop new evidence shows

Mosapride Mosapride is a gastroprokinetic agent that acts as a selective 5HT4 agonist. The major active metabolite of mosapride, known as M1, additionally acts as a 5HT3 antagonist, which accelerates gastric emptying throughout the whole of the gastroin ...

can be a safe and affordable therapeutic option for blepharospasm.

Botulinum toxin Botulinum toxin, or botulinum neurotoxin (BoNT), is a neurotoxic protein produced by the bacterium ''Clostridium botulinum'' and related species. It prevents the release of the neurotransmitter acetylcholine from axon endings at the neurom ...

injections have been used to induce localized, partial paralysis. Among most sufferers, botulinum 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 botulinum toxin 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 ''ptosis'' or eyelid droop. Attempts to inject in locations that minimize ptosis can result in diminished ability to control spasms. A recent Cochrane systematic review showed that a single treatment session (where both eyelids were injected with BtA multiple times) alleviated the symptoms of blepharospasm, disability, and number of involuntary movements. People 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. Since the root of the problem is neurological, doctors have explored sensorimotor retraining activities to enable the brain to "rewire" itself and eliminate dystonic movements. The work of Joaquin Farias has shown that sensorimotor retraining activities and proprioceptive stimulation can induce

neuroplasticity Neuroplasticity, also known as neural plasticity, or brain plasticity, is the ability of neural networks in the brain to change through growth and reorganization. It is when the brain is rewired to function in some way that differs from how it p ...

, making it possible for patients to recover substantial function that was lost due to blepharospasm. Developed by Th
Eye Research Group Oxford
PressOp™ is a new ethically approved spectacle mounted device that arose after studying individuals with Blepharospasm who regularly use “sensory tricks”. By applying pressure to the temple region of their forehead patients found this helped to enable eye opening. The device is particularly helpful controlling an episode of severe spasm when the carrying out of specific tasks is necessary. Blepharospasm sufferers may find continuity in their quality of life by using the device while effectiveness of treatment is not at its peak.

References

External links

Blepharospasm
– Resource Guide from the National Eye Institute (NEI) {{CNS diseases of the nervous system Extrapyramidal and movement disorders Dystonia Disorders of eyelid, lacrimal system and orbit

Signs and symptoms

Causes

Treatment

See also

References

External links