Patient may provide a history of blinking that is exacerbated by noise, pollution, smog, wind and sudden movement of the head or neck. Other symptoms include eye discomfort, tearing, eye irritation and vague eye pain or photophobia. Initially the symptoms may be one sided but often progress and affect both eyes. A common complaint is that the symptoms of blinking are often worsened in bright light.

Patients may report not being able to read, watch TV, walk or drive. A positive family history may provide a clue to the diagnosis. Most patients will indicate that the symptoms subside during sleep, talking, while looking downwards, when using artificial eye drops and relaxing. Initially the eyelid contractions are mild but with time, the contractions appear forceful. The disorder is progressive over a few years and then become stable. Remission without treatment is rare. The spasms often last several seconds and primarily involve the orbicularis oculi muscle.

• Movement Disorder

  We present a patient with a facial movement disorder that has characteristics of both blepharospasm and bilateral asynchronous hemifacial spasm. [ncbi.nlm.nih.gov]

  Bressman, Milestones in dystonia, Movement Disorders, 26, 6, (1106-1126), (2011). Matthew J. [dx.doi.org]

  It is not life-threatening It is a form of dystonia, a movement disorder in which sustained muscle contractions cause twitching and repetitive movements. BEB begins gradually with occasional eye blinking and/or irritation. [brainfoundation.org.au]

• Pain

  Pain and focal dystonias have been associated with chronic pain conditions such as complex regional pain syndrome. Corneal pain, frequently known as "dry eye", may be a neuropathic pain condition with abnormalities of the nerve plexus. [ncbi.nlm.nih.gov]

  Side effects of Botox Anxiety; back pain; dizziness; drowsiness; dry mouth; dry or irritated eyes; facial pain; flu-like symptoms; headache; inability to focus eyes; increased cough; indigestion; nausea; neck pain; pain, redness, swelling, or tenderness [healingtheeye.com]

  Eye exposure produces intense tearing, pain, conjunctivitis, and blepharospasm. From Wikipedia This example is from Wikipedia and may be reused under a CC BY-SA license. [dictionary.cambridge.org]

  Various stimuli such as corneal or eyelid irritation, pain, emotion, stress and other nerve stimulants trigger the sensory limb of the circuit. [eyeplastics.com]

• Weakness

  […] mouth; dry or irritated eyes; facial pain; flu-like symptoms; headache; inability to focus eyes; increased cough; indigestion; nausea; neck pain; pain, redness, swelling, or tenderness at the injection site; runny nose; sensitivity to light; stiff or weak [healingtheeye.com]

  The weakness is worst at the end of the day, due to the fatigue of the myasthenic muscles. [infodystonia.com]

  We suggest that aiding closure of the weak eyelid in this case caused a reduction in blink system excitability via the same compensatory mechanisms that initially produced the eyelid spasm. [ncbi.nlm.nih.gov]

• Short Stature

  Bloom syndrome is a rare autosomal recessive disorder characterized by short stature, brachydactyly, malar hypoplasia and facial telangiectasia, erythema and cafe au lait spots. Affected individuals have an increased risk of developing malignancies. [radiopaedia.org]

  Most clinicians engaged in studies of Bloom syndrome consider the volatility of the chromosomes to be a major contributor to both short stature and a predisposition to cancer. [rarediseases.org]

  Treatment of short stature with growth hormone should be avoided, as it is thought that it increases the chance of cancer. Radiotherapy and chemotherapy may be unsuitable due to risk of severe adverse effects due to the genetic defect. [dermnetnz.org]

  Bloom syndrome is an inherited disorder characterized by short stature, a skin rash that develops after exposure to the sun, and a greatly increased risk of cancer. [ghr.nlm.nih.gov]

• Falling

  She said: "When I was working with students one-to-one my eyes would close, they thought I was falling asleep or angry with them. "My face wouldn’t respond so it was uncomfortable having those face-to-face conversations. [ouh.nhs.uk]

  The range of ailments falling under the umbrella-term of "abnormal involuntary movement disorders" is diverse and includes conditions as different from each other as Parkinson's disease, restless legs syndrome and blepharospasm. [cordingleyneurology.com]

  Depending on patient symptoms, institute catheterization if PVR urine volume exceeds 200 mL and continue until PVR falls below 200 mL. [botoxmedical.com]

  Double vision can very disturbing, a source of headache and falls as the edge of the pavement, for instance, will be difficult to appreciate. [infodystonia.com]

• Muscle Rigidity

  […] nuchal rigidity, muscle rigidity, parkinsonian gait, and glabellar reflex abnormal, parkinsonian rest tremor), akathisia (includes akathisia, restlessness, hyperkinesia, and restless leg syndrome), dyskinesia (dyskinesia, muscle twitching, choreoathetosis [mymemory.translated.net]

• Sialorrhea

  […] pharmacist Visit www.xeomin.com to obtain the FDA-approved product labeling Call 1-844-4MYMERZ Uses XEOMIN is a prescription medicine used in adults: that is injected into glands that make saliva and is used to treat long-lasting (chronic) drooling (sialorrhea [xeomin.com]

  […] following pathological states of high interdisciplinary relevance are focused in this article: Table 1 Diseases treated with botulinum toxin type A in head and face medicine with high interdisciplinary relevance Facial nerve paralysis Hypersalivation, Sialorrhea [dx.doi.org]

• Hypersalivation

  Autonomous Nerve System Hypersalivation Hypersalivation is of high relevance for patients suffering from different diseases (see Fig 6 ) [ 21, 22 ]. [dx.doi.org]

• Sweating

  Gustatory sweating, sweating of the face Gustatory sweating is a common sequelae following parotid gland surgery [ 23 – 28 ]. [dx.doi.org]

  […] such as blepharospasm (spasm of the eyelids), cervical dystonia Botox is approved for treatment of conditions such as blepharospasm (spasm of the eyelids), cervical dystonia (severe neck muscle spasms), and severe primary axillary hyperhydrosis (excess sweating [englishtohindi.in]

  […] flu-like symptoms; headache; inability to focus eyes; increased cough; indigestion; nausea; neck pain; pain, redness, swelling, or tenderness at the injection site; runny nose; sensitivity to light; stiff or weak muscles at or near the injection site; sweating [healingtheeye.com]

  Use a tight baseball cap, hair band or sweat band around the forehead. Put your head back – close your eyes, or look down. Go in a dark place and try to relax. [dystonia.org.uk]

• Hyperhidrosis

  Primary Axillary Hyperhidrosis The most frequently reported adverse events (3%-10% of adult patients) following injection of BOTOX® for severe primary axillary hyperhidrosis in double-blind studies include injection-site pain and hemorrhage, nonaxillary [botoxmedical.com]

  Neuroendocrine tumors Renal Cell Carcinoma Endocrinology Acromegaly Precocious puberty Severe primary IGF-1 deficiency Growth hormone deficiency Turner syndrome Neurosciences Blepharospasm Cervical dystonia Cerebral palsy Equinus deformity Hemifacial spasm Hyperhidrosis [ipsennordic.com]

  Treatment of focal hyperhidrosis with botulinum toxin type A: long-term follow up in 61 patients. Br J Dermatology 2001;145:289-293. [ Links ] 14. D'Onofrio V, Miletto P, Leandro G, Iaquinto G. [scielo.br]

• Muscle Spasm

  In up to a third of cases there are synchronous mid and / or lower facial spasms and the spectrum of disorder extends to cranial dystonia involving jaw & neck muscle spasms (e.g. antecollis or retrocollis). [clinicaltrials.gov]

  Blepharospasm or benign essential blepharospasm (BEB) is a muscle disorder that causes abnormal, involuntary spasms of the muscles around the eye(s). [faeye.com]

  Botox® can be injected directly into the muscles surrounding the eye, helping to reduce their activity. This will reduce muscle spasms and help promote muscle relaxation, both alleviating your pain and reducing muscle spasms and tension. [laser15.com]

• Torticollis

  The phenomenon can be regarded as the equivalent of the sensory trick (ST or geste antagonistique) seen in some cases of spasmodic torticollis whereby finger tip pressure on the chin prevents or reduces the muscle spasms in the neck and allows the head [clinicaltrials.gov]

  Neurology, 35(2): 283-4 [ CR ] [ PM ] [ EČ ][ GS ] Schroder, J.M., Huffmann, B., Braun, V., Richter, H.P. (1992) Spasmodic torticollis: Severe compression neuropathy in rami dorsales of cervical nerves C1-6. [scindeks.ceon.rs]

  […] basal ganglia, unspecified G24.0 Drug induced dystonia G24.01 Drug induced subacute dyskinesia G24.02 Drug induced acute dystonia G24.09 Other drug induced dystonia G24.1 Genetic torsion dystonia G24.2 Idiopathic nonfamilial dystonia G24.3 Spasmodic torticollis [icd10data.com]

  MalaCards based summary : Blepharospasm, Benign Essential, also known as benign essential blepharospasm, is related to lymphatic malformation 5 and schwartz-jampel syndrome, type 1, and has symptoms including myoclonus, oromandibular dystonia and torticollis [malacards.org]

• Blepharospasm

  Blepharospasm ICD9 333.81 Disease Blepharospasm is involuntary closure of both eyelids. [eyewiki.aao.org]

  Analysis of blink rate in patients with blepharospasm. Mov Disord. 2006 Apr 18. [Medline]. Henderson JW. Essential blepharospasm. Trans Am Ophthalmol Soc. 1956. 54:453-520. Malinovsky V. Benign essential blepharospasm. [emedicine.medscape.com]

  Examples of blepharospasm blepharospasm This process has been fastest in easily defined clinical situations such as focal dystonias - blepharospasm is a good example - and slowest in the more complex world of rehabilitation. [dictionary.cambridge.org]

• Blepharitis

  Eyelid scrubs with baby shampoo or other non-irritating soap can minimize any blepharitis that can also aggravate blepharospasms. [eyewiki.aao.org]

• Visual Impairment

  People with blepharospasm usually have normal eyes, but the forced closure of the eyelids causes visual impairment. [faeye.com]

  Occasionally, it can be so intense it results in severe visual impairment. Aetiology [ 1, 2 ] Blepharospasm is a subtype of focal dystonia. Most cases are idiopathic and termed benign essential blepharospasm or primary blepharospasm. [patient.info]

  Sources of support If eye dystonia is causing visual impairment, the following may be helpful sources of support: The Royal National Institute for Blind People (RNIB) Your local Sensory Support service. [dystonia.org.uk]

• Blepharoptosis

  To our knowledge this is the first reported case of a combination of unilateral blepharospasm and blepharoptosis with bilateral vertical gaze palsy. [ncbi.nlm.nih.gov]

  Ptosis : Ptosis is may also be called Blepharoptosis. It refers to an eyelid which is droopy. This may possibly cause a loss of vision, especially while reading, headaches, and eyebrow strain. [eyeplastics.com]

  Complications Complications associated with botulinum toxin injection include: Bruising, blepharoptosis, ectropion, epiphora, diplopia, lagophthalmos, and corneal exposure. [eyewiki.aao.org]

  Risk of blepharoptosis is reduced by avoiding the central upper eyelid. Also, there is a trend toward avoiding the medial lower eyelid, which carries increased risk of affecting the underlying inferior oblique muscle. [healio.com]

• Foreign Body Sensation

  body sensation in eyes, lacrimation increased, erythema of eyelid, eyelid oedema, eyelid disorder, ocular hyperaemia Last Update: 2017-04-26 Usage Frequency: 1 Quality: Lithuanian EPS apėmė apibendrintą analizę toliau išvardytų terminų: parkinsonizmas [mymemory.translated.net]

• Facial Grimacing

  When blepharospasm is part of Meige's syndrome, it is associated with facial grimacing. Reflex blepharospasm may be accompanied by photophobia and ocular signs of blepharitis and surface disease. [eyewiki.aao.org]

  We report on two cases who presented with involuntary facial grimacing and frowning. Blepharospasm has never been documented in one, and the other case showed difficulty in lid opening only in the early phase of the disease. [ncbi.nlm.nih.gov]

• Dystonia

  […] is cranial dystonia. [bcm.edu]

  […] severity of the cervical dystonia. [infodystonia.com]

  Ozelius, Genetic evaluation in hereditary dystonia, Handbook of Dystonia, 10.3109/9781841848525.003, (22-52), (2012). William G. Ondo and Raja Mehanna, Cranial dystonia, Handbook of Dystonia, 10.3109/9781841848525.009, (127-143), (2012). [dx.doi.org]

  […] by dystonia experts, and empiricism. [web.archive.org]

  Blepharospasm may lead to embarrassment in social situations, and oromandibular dystonia can affect speech, making it difficult to carry on the simplest conversations. [dictionary.cambridge.org]

• Stroke

  Neurological Disorders and Stroke » [brainfacts.org]

  KEYWORDS: movement disorders (other than parkinsons); ophthalmology; stroke [ncbi.nlm.nih.gov]

  Benign Essential Blepharospasm NORD gratefully acknowledges Mark Hallett, MD, Chief, Human Motor Control Section, NIH National Institute of Neurological Disorders and Stroke, for assistance in the preparation of this report. [rarediseases.org]

• Excitement

  PURPOSE: The somatosensory-evoked blink reflex (SBR) is one of the release phenomena of blink reflex, possibly resulting from increased excitability of brainstem reticular formation. [ncbi.nlm.nih.gov]

  Previous studies demonstrated the possibility that the excitability of facial motor neurons is regulated by monoaminergic modulators, such as dopamine1 and serotonin (5‐HT),2, 3 and that disturbance of the regulation might be involved in its pathogenesis [onlinelibrary.wiley.com]

  This cutting-edge approach has created an excitement among his clinical team and superb outcomes for his patients. In Dr. [claruseye.com]

  In people with blepharospasm the motor cortex, the part of the brain which controls voluntary muscle activity such as the closing of the eyes, becomes over excited. [ouh.nhs.uk]

• Seizure

  […] swallowing or breathing; double or blurred vision or other vision changes; drooping of the upper eyelid; eyelid swelling; fainting; fever, chills, or persistent sore throat; irregular heartbeat; loss of bladder control; loss of strength; paralysis; seizures [healingtheeye.com]

  (includes seizure and partial seizure). [botoxmedical.com]

  Focal seizures Aberrent reinnervation/synkinesis Medical or surgical, with oral medications having limited usefulness. In secondary causes of blepharospasm, treat the underlying etiology. [eyewiki.aao.org]

  Artane (anti-cholinergic), Klonopin (gabanergic muscle relaxant), and Keppra (anti-seizure) have been shown to help some essential blepharospasm and/or Meige patient. [klapperplasticsurgery.com]

• Dizziness

  Side effects of Botox Anxiety; back pain; dizziness; drowsiness; dry mouth; dry or irritated eyes; facial pain; flu-like symptoms; headache; inability to focus eyes; increased cough; indigestion; nausea; neck pain; pain, redness, swelling, or tenderness [healingtheeye.com]

  […] transporters in the brain depression, drowsiness, Parkinsonism Anticonvulsant levetiracetam (KEPPRA) binds to the synaptic vesicle protein SV2A irritability, headaches Imidazopyridine zolpidem (AMBIEN) binds to the benzodiazepine receptor 1 drowsiness, dizziness [web.archive.org]

  They include itching, rash, red itchy welts, wheezing, asthma symptoms, or dizziness or feeling faint. Get medical help right away if you experience symptoms; further injection of BOTOX ® should be discontinued. [botoxblepharospasm.com]

  Tell your doctor or get medical help right away if you get wheezing or trouble breathing, or if you get dizzy or faint. [xeomin.com]

In most people with blepharospasm the diagnosis is delayed. The reason is that healthcare workers often do not recognize it as a dystonia or an illness. In addition, it is often felt that the condition is benign or cosmetic and does not warrant any treatment. Studies reveal that many patients see several doctors before a diagnosis of blepharospasm is made.

The diagnosis is primarily made on a physical exam and requires excluding other disorders. The key feature of blepharospasm is bilateral eyelid involvement which differentiates it from facial myokymia and hemifacial spasms. Another disorder that can be confused with blepharospasm is Parkinson disease. No blood work is required. In some rare cases, an MRI or CT scan of the head will be ordered to look for a mass.

The decision to treat blepharospasm depends on the degree of disability. The condition is not life threatening. Mild cases of blepharospasm with no disability may be managed with conservative care and observation. Individuals with severe symptoms should be referred to an ophthalmologist or neurologist [6[ [7] [8] [9] [10]. Patients who have mild to moderate symptoms can be managed in the following ways:

Conservative care

• Irrespective of the degree of symptoms conservative methods should be offered to all individuals.
• It is well known that rose tinted sunglasses can provide protection from ultraviolet light and decrease photophobia.
• Lid hygiene is recommended to diminish blepharitis and lid irritation.
• Use of artificial tears and ocular lubricants may help resolve the dye eye symptoms in some patients.

Botulinum toxin (Botox)

Local injection with botox is main treatment. Botulinum toxin works by inhibiting presynaptic release of acetylcholine at the neuromuscular junction, leading to muscle paralysis. Once eyelid muscles are paralyzed, the dystonia is temporarily relieved. The effects of botox are not immediate and are also dose related. Relief of symptoms occurs after 2-3 days and last 2-3 months.

Botulinum toxin treatments are given over several weeks to determine an effective dose. Repeat injections are then given every 2-3 months. About 90% see a response with botulinum toxin. Treatment is usually well tolerated, but adverse effects may include:

• Corneal exposure/lagophthalmos
• Diplopia
• Ectropion
• Ecchymosis
• Entropion
• Epiphora
• Lower facial weakness
• Photophobia
• Ptosis
• Symptomatic dry eye

Medications

Several medications may be used as second line treatment but their benefits are limited and short lived. In addition medications also have systemic adverse effects. Drugs used include benzodiazepines, anticholinergics, serotonin antagonists, antipsychotics, lithium, tetrabenazine, meprobramate, amphetamine, phenobarbital, parasympathomimetics, antimuscarinics, antihistamines and GABA agonists. A few patients see some benefit from sedatives like lorazepam and clonazepam. Overall the effectiveness of drugs to treat blepharospasm is mild and only seen in a few patients. Chemomyectomy (neurectomy) by injection of doxorubicin is relatively effective, but can cause skin ulceration. 

Surgery

Patients who have shown no or partial response to botox may be considered for a surgical procedure. Myectomy is widely used to treat chronic blepharospasm. Most ocular surgeons perform am upper eyelid procedure first and wait for a few months to assess the response. Only if the response is unsatisfactory is surgery on the lower eye lid undertaken. The major reason to stage the surgery is to reduce the risk of lymph edema, which can be significant and prolonged.

Other complications of surgery include the following:

• Corneal exposure with dryness
• Supra orbital anesthesia
• Hematoma
• Infection
• Skin necrosis
• Brow hair loss
• Ptosis
• Trichiasis
• Upper and lower lid retraction
• Canthal deformity

Complete myectomies do benefit a significant number of patients in terms of symptoms improvement. The results are comparable to botox treatment. However, before undertaking surgery it is important to warn the patient that the surgery is not curative. It is only used in cases that fail to respond to botulinum toxin.

At one end of the spectrum, blepharospasm may just be a cosmetic problem but at the other extreme it can present with a high rate of blinking that interferes with reading, watching TV or working. Some people may have great difficulty with functional vision. At this stage, the individual may not be able to drive or even read the newspaper. Even though botox improves symptoms, the benefits are temporary. With myectomy the results are permanent but the risk of complication is high. Because of the poor quality of life, many people also report being anxious and depressed.

If blepharospasm is untreated it can lead to the following:

• Brow ptosis
• Canthal tendon laxity
• Cosmetic deformity
• Dermatochalasis
• Eyelid malposition
• Functional loss of vision
• Lid ptosis

The definitive cause of blepharospasm remains unknown. There is a familial inheritance but often the penetrance is not complete. So far the role of the environment, existing eye disease or drinking coffee has not been shown to be the case. Risk factors for blepharospasm include the following:

• Prior face or head trauma
• Prior ocular disease
• Family history
• History of dystonia or tremor

Blepharospasm is not rare at all. It has been reported in all races and cultures. Because the disorder is often asymptomatic, its exact prevalence remains unknown. Blepharospasm is three times more likely to affect women than men. The condition often comes to medical attention in the 4th-5th decade of life but it can occur at any age [5].

Closure and opening of the eyelid is controlled by two groups of muscles. The protractors of the eyelid include the Procerus, Corrugator superciliaris and orbicularis oculi. The voluntary eyelid muscles include the frontalis and the levator palpebrae muscles. During normal blinking, the retractors and protractors are under nerve control and operate at different time period. In patients with blepharospasm, the inhibitory control between the retractor and protractor muscles is lost and frequent blinking or spasms of the eyelid results.

Because the cause is not known, prevention is not possible. However, once the disorder has been diagnosed, patients should seek assistance from an ophthalmologist to prevent functional loss of vision.

Blepharospasm or benign essential blepharospasm is a medical term used to describe repeated involuntary closure of the eyes. The blepharospasm is bilateral and does not involve any other facial muscles. Previously, blepharospasm was thought to be neuropsychiatric in origin but today it is regarded a neuropathological disorder with an alteration in the blinking reflex control centre. Blepharospasm is classified as a focal dystonia and while in most cases it is the only feature, in other people it may also be associated with dystonias of the neck and face. Blepharospasm has also been reported to occur in families. The eyelid spasms often last a few seconds to several minutes and primarily involve the eyelid muscles (corrugator superciliaris, orbicularis oculi, and procerus muscles). Blepharospasm is an often misdiagnosed yet treatable disorder which is associated with high morbidity. The disorder presents with frequent blinking episodes which can affect reading, working and even watching television. The condition is progressive and is treated with either botulinum toxin or surgery [1] [2] [3] [4].

Blepharospasm is an eye disorder where the eyelids blink at a variable frequency. When the blink rate is high, the individual may have difficult with vision, driving, reading and has a poor quality of life; in addition, the condition is cosmetically unattractive. The cause of blepharospasm is not well understood but it is felt to be due to overcontraction of the muscles of the eyelid. The diagnosis is made clinically. The ideal treatment for blepharospasm is use of botox. When patients fail to respond to botox surgery can be done to remove the eyelid muscles. Overall, most patients have a good result but the condition is not curable.

1. Potgieser AR, van Dijk JM, Elting JW, de Koning-Tijssen M, Facial tics and spasms. Ned Tijdschr Geneeskd. 2014;158:A7615
2. Ryuji K. Dystonia. Handb Clin Neurol. 2013;116:485-9.
3. Jinnah HA, Berardelli A, Comella C, Defazio G, Delong MR, Factor S, Galpern WR, Hallett M, Ludlow CL, Perlmutter JS, Rosen AR; Dystonia Coalition Investigators. The focal dystonias: current views and challenges for future research. Mov Disord. 2013 Jun 15;28(7):926-43. 
4. Khooshnoodi MA, Factor SA, Jinnah HA. Secondary blepharospasm associated with structural lesions of the brain. J Neurol Sci. 2013 Aug 15;331(1-2):98-101
5. Steeves TD, Day L, Dykeman J, Jette N, Pringsheim T. The prevalence of primary dystonia: a systematic review and meta-analysis. Mov Disord. 2012 Dec;27(14):1789-96
6. Broadbent TJ, Wesley RE, Mawn LA A Survey of Current Blepharospasm Treatment Patterns Among Oculoplastic Surgeons. Ophthal Plast Reconstr Surg. 2015 Mar 25
7. Hallett M, Albanese A, Dressler D, Segal KR, Simpson DM, Truong D, Jankovic J. Evidence-based review and assessment of botulinum neurotoxin for the treatment of movement disorders. Toxicon. 2013 Jun 1;67:94-114.
8. Hellman A, Torres-Russotto D. Botulinum toxin in the management of blepharospasm: current evidence and recent developments. Ther Adv Neurol Disord. 2015 Mar;8(2):82-91
9. Pariseau B, Worley MW, Anderson RL. Myectomy for blepharospasm 2013. Curr Opin Ophthalmol. 2013 Sep;24(5):488-93
10. Kent TL, Petris CK, Holds JB. Effect of upper eyelid myectomy on subsequent chemodenervation in the management of benign essential blepharospasm. Ophthal Plast Reconstr Surg. 2015 May-Jun;31(3):222-6