Blepharospasm

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Blepharospasm
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Left orbicularis oculi, seen from behind.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ibtisam Ashraf, M.B.B.S.[2]

Synonyms and Keywords: Eyelid twitch, benign essential blepharospasm, eyelid spasm

Overview

A blepharospasm ('eye twitching') is any abnormal tic or twitch of the eyelid (from blepharo, eyelid, and spasm, an uncontrolled muscle contraction). However, it is normally distinguished from less serious disorders and refers to Benign Essential Blepharospasm, a focal dystonia (a neurological movement disorder involving involuntary and sustained muscle contractions) of the muscles around the eyes. The cause is often undetermined, but fatigue or a an irritant are possible contributing factors. Symptoms usually last for a few days then disappear without treatment, but in some cases the twitching is chronic and persistent. Occasionally, the symptoms are severe enough to result in effective blindness.

Historical Perspective

  • Blepharospasm was first found in a painting called De Gaper in the 16th century.
  • Later, at the turn of the 20th century, Henry Meige, a French neurologist, described a patient with eyelid and midface spasm, a condition today known as Meige's Syndrome.
  • C. D. Marsden suggested that blepharospasm should be regarded as a focal form of dystonia along with other disorders such as oromandibular dystonia, cervical dystonia, and writer's cramp.[1]
  • Marsden also identified a variety of clinical characteristics, including female preference, peak age at onset between the 5th and 7th decade, a propensity to extend to neighboring areas of the body, and potential correlation with tremor in the head or upper limbs.[2]

Classification

Primary Blepharospasm

  • It is also referred to as benign essential blepharospasm.
  • Mostly sporadic, however, autosomal dominant patterns of inheritance have been observed in a few families.
  • Coffee consumption could possibly be protective.

Secondary Blepharospasm

Pathophysiology

  • There are many pathways that trigger the essential blepharospasm, but the precise cause is unclear. However, several studies suggest that the facilitation of the blink reflex is a result of a failure in inhibitory processes. There is increased excitability of the blink and corneal reflex and deficiency in the cycle of recovery of the R2 portion of the blink reflex.[4]
  • The interneurons that mediate the R2 response of the blink reflex in the basal ganglia are hyper excited and thus dysfunctional in blepharospasm.
  • Another study divided patients into 3 subclasses on the basis of EMG pattern of the Orbicularis Oris and levator palpebrae muscles. The first group had patients with blepharospasm alone, could have an abnormal R2 recovery index. The second group included patients with blepharospasm with spontaneous levator palpebrae inhibition. The last group had patients with involuntary levator palpebrae inhibition but no blepharospasm had a normal R2 recovery index. This suggested that cranial dystonias are not homogenous pathophysiologically.[5]
  • Transcranial Magnetic Stimulation (TMS) has shown that the excitability of primary motor cortex activity is impaired in blepharospasm, as shown by decreased short-term intracortical inhibition in the hand muscles and shortened duration of the cortical silent period in the cranial muscles of these patients.[6]
  • Patients with blepharospasm exhibit an increased somatosensory temporal discrimination threshold (STDT) and is more sensitive than blink reflex recovery for identifying underlying pathophysiology.
  • SYNE1 and CIZ1 mutations also contribute to the etiology of Benign Essential Blepharospasm (BEB).

Causes

  • In most cases, blepharospasm seems to develop spontaneously.
  • Many blepharospasm patients have a previous history of dry eyes and/or light sensitivity.
  • Blepharospasm can also come from abnormal functioning of the brain's basal ganglia.
  • Concomitance with dry eye, as well as other dystonias such as Meige's syndrome, has been observed.
  • Some drugs can induce blepharospasm, such as Dimercaprol, those used to treat Parkinson's disease, as well as sensitivity to hormone treatments, including estrogen replacement therapy for women going through menopause.
  • Blepharospasms can be caused by concussions in some rare cases, when a blow to the back of the head damages the basal ganglia.

Differentiating blepharospasm from other Diseases

Epidemiology and Demographics

  • The mean annual incidence is 0.10%.[7]
  • The prevalence of blepharospasm in the general population is approximately 5 in 100,000.[8]
  • Females are more commonly affected by blepharospasm than males.
  • The peak incidence is in the 50 to 59-year-old age group.
  • People living in developed areas are at greater risk of developing blepharospasm compared to those living in less populated areas, similarly, white-collar workers have a higher chance of having blepharospasm.

Risk Factors

Screening

  • There is insufficient evidence to recommend routine screening for BEB.

Natural History, Complications and Prognosis

  • The symptoms of blepharospasm usually develop after a fifth-sixth decade of life, and starts with the increased frequency of blinking, particularly in response to various common stimuli, including wind, air pollution, sunlight, noise, movements of the head or eyes, and in response to stress or the environment..[10]
  • Patients may complain about photophobia and ocular surface irritation, particularly dry eye symptoms.
  • These signs develop over a complex duration to include spontaneous unilateral spasms that eventually become bilateral.
  • Complications such as corneal abrasions and dermatochalasis can also arise.
  • On the one hand, it is a simple increased blinking rate and occasional eyelid spasms, and on the other hand, it results in functional blindness and eye pain.

Diagnosis

Diagnostic Study of Choice

  • Presence of stereotyped, bilateral, and synchronous orbicularis oculi spasms including narrowing/closure of the eyelids, increased blinking, inability to voluntarily suppress the spasms and presence of effective sensory trick confirm the diagnosis of blepharospasm.[11]

History and Symptoms

  • Uncontrollable tics or twitches of the eye muscles and surrounding facial area
  • Excessive blinking of the eyes, or forced closure of durations longer than the typical blink reflex
  • Dryness of the eyes
  • Sensitivity to the sun and bright light
  • Blepharitis, iritis, conjunctivitis or corneal disease

Physical Examination

  • Benign essential blepharospasm is a clinical diagnosis and a diagnosis of exclusion.
  • Physical examination demonstrates bilateral spontaneous spasms, leading to eye closing, typically decrease during sleep.

Laboratory Findings

Electrocardiogram

  • There are no ECG findings associated with blepharospasm.

X-ray

  • There are no x-ray findings associated with blepharospasm.

Echocardiography or Ultrasound

CT Scan

  • There are no CT scan findings associated with blepharospasm.

MRI

  • There are no MRI findings associated with blepharospasm.

Other Imaging Findings

  • There are no other imaging findings associated with blepharospasm.

Other Diagnostic Studies

  • There are no other diagnostic studies associated with blepharospasm.

Treatment

Medical 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 chloride has been found effective.
  • Botulin toxin (BoNT) injections into the eyelids and eyebrows are now generally considered as the treatment of choice.[12]

Non-Pharmacotherapy

  • 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.
  • Using tweezers to remove excess eyelashes from the outer corner of the eyelid may sometimes resolve this condition.

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.
  • Deep brain stimulation (DBS) has been found to be effective in patients who have become refractory to other forms of therapy.[13]

Primary Prevention

Secondary Prevention

References

  1. Marsden CD (1976). "Blepharospasm-oromandibular dystonia syndrome (Brueghel's syndrome). A variant of adult-onset torsion dystonia?". J Neurol Neurosurg Psychiatry. 39 (12): 1204–9. doi:10.1136/jnnp.39.12.1204. PMC 492566. PMID 1011031.
  2. Valls-Sole J, Defazio G (2016). "Blepharospasm: Update on Epidemiology, Clinical Aspects, and Pathophysiology". Front Neurol. 7: 45. doi:10.3389/fneur.2016.00045. PMC 4814756. PMID 27064462.
  3. Defazio G, Hallett M, Jinnah HA, Conte A, Berardelli A (2017). "Blepharospasm 40 years later". Mov Disord. 32 (4): 498–509. doi:10.1002/mds.26934. PMC 5941939. PMID 28186662.
  4. Berardelli A, Rothwell JC, Day BL, Marsden CD (1985). "Pathophysiology of blepharospasm and oromandibular dystonia". Brain. 108 ( Pt 3): 593–608. doi:10.1093/brain/108.3.593. PMID 4041776.
  5. Hallett M, Evinger C, Jankovic J, Stacy M, BEBRF International Workshop (2008). "Update on blepharospasm: report from the BEBRF International Workshop". Neurology. 71 (16): 1275–82. doi:10.1212/01.wnl.0000327601.46315.85. PMC 2676990. PMID 18852443.
  6. Hallett M, Evinger C, Jankovic J, Stacy M, BEBRF International Workshop (2008). "Update on blepharospasm: report from the BEBRF International Workshop". Neurology. 71 (16): 1275–82. doi:10.1212/01.wnl.0000327601.46315.85. PMC 2676990. PMID 18852443.
  7. Sun Y, Tsai PJ, Chu CL, Huang WC, Bee YS (2018). "Epidemiology of benign essential blepharospasm: A nationwide population-based retrospective study in Taiwan". PLoS One. 13 (12): e0209558. doi:10.1371/journal.pone.0209558. PMC 6306223. PMID 30586395.
  8. "Benign Essential Blepharospasm - NORD (National Organization for Rare Disorders)".
  9. Sun Y, Tsai PJ, Chu CL, Huang WC, Bee YS (2018). "Epidemiology of benign essential blepharospasm: A nationwide population-based retrospective study in Taiwan". PLoS One. 13 (12): e0209558. doi:10.1371/journal.pone.0209558. PMC 6306223. PMID 30586395.
  10. Bentivoglio AR, Daniele A, Albanese A, Tonali PA, Fasano A (2006). "Analysis of blink rate in patients with blepharospasm". Mov Disord. 21 (8): 1225–9. doi:10.1002/mds.20889. PMID 16622858.
  11. Defazio G, Hallett M, Jinnah HA, Berardelli A (2013). "Development and validation of a clinical guideline for diagnosing blepharospasm". Neurology. 81 (3): 236–40. doi:10.1212/WNL.0b013e31829bfdf6. PMC 3770163. PMID 23771487.
  12. Simpson DM, Blitzer A, Brashear A, Comella C, Dubinsky R, Hallett M; et al. (2008). "Assessment: Botulinum neurotoxin for the treatment of movement disorders (an evidence-based review): report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology". Neurology. 70 (19): 1699–706. doi:10.1212/01.wnl.0000311389.26145.95. PMC 5565261. PMID 18458230.
  13. Hallett M, Evinger C, Jankovic J, Stacy M, BEBRF International Workshop (2008). "Update on blepharospasm: report from the BEBRF International Workshop". Neurology. 71 (16): 1275–82. doi:10.1212/01.wnl.0000327601.46315.85. PMC 2676990. PMID 18852443.

External Links

  • Blepharospasm Resource Guide from the National Eye Institute (NEI).


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