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Blepharospasm

Blepharospasms

Blepharospasm is an involuntary spasmodic contraction of the orbicularis oculi muscle.


Presentation

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.

Internal Bleeding
  • There were 5 incapacitating motorcycle injuries: 1 cervical spine injury with quadriparesis, 1 blunt abdominal injury with internal bleeding, 2 broken limbs, and 1 ligament rupture; 1 incapacitating car injury: broken limb of other person; and 11 non-incapacitating[ncbi.nlm.nih.gov]
Vertical Gaze Palsy
  • 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]
Skin Ulcer
  • 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.[symptoma.com]
Facial Grimacing
  • 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]
  • 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]
  • Secondary blepharospasm: Meige’s syndrome: Meige’s syndrome may be associated with facial grimacing due to facial dystonia. Breughel’s syndrome: Breughel’s syndrome is a dystonia of motor trigeminal nerve which produces widely open mouth.[aimu.us]
Motor Symptoms
  • BACKGROUNDS: Studies have reported that non-motor symptoms are an important component of primary dystonia. However, evidence supporting cognitive impairment in primary dystonia is limited and contradictory.[ncbi.nlm.nih.gov]
  • The pathophysiology of blepharospasm (BSP), which is characterized by motor symptoms and also sensory symptoms, remains unclear.[ncbi.nlm.nih.gov]
Akathisia
  • Orolingual dyskinesia was present in one case and tardive akathisia in two other cases. The symptoms fluctuated in severity with a number of exacerbating and relieving factors.[ncbi.nlm.nih.gov]
  • Hayashi T, Furutani M, Taniyama J, et al: Neuroleptic induced Meige’s syndrome following akathisia: pharmacologic characteristics. Psychiatry Clin Neurosci 1998; 52:445–448 Google Scholar 11.[dx.doi.org]
Hand Tremor
  • In this report, we describe a 53-year-old female who presented initially with hand tremor following a total hip arthroplasty.[ncbi.nlm.nih.gov]
Bradykinesia
  • A 66-year-old woman presented with a 3-year history of predominantly right-sided blepharospasm and a 1-year history of progressive predominantly left-sided hemiparkinsonism manifested by a left upper extremity resting tremor and left-sided bradykinesia[ncbi.nlm.nih.gov]
Mononeuropathy
  • Bell's palsy is the most common acute mononeuropathy, with an incidence of 20 to 30 cases per 100,000 people per year. Blepharospasm associated with Bell's palsy has been rarely reported.[ncbi.nlm.nih.gov]

Workup

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.

Treatment

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:

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:

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.

Prognosis

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:

Etiology

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:

Epidemiology

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].

Sex distribution
Age distribution

Pathophysiology

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.

Prevention

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.

Summary

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].

Patient Information

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.

References

Article

  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

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Last updated: 2019-07-11 22:14