Blepharospasm

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Blepharospasm
Other namesEye dystonia, Eye twitching, Eye spasm
Pronunciation
SpecialtyNeurology, ophthalmology

Blepharospasm is involuntary contraction of the orbicularis oculi (eyelid) muscle.[1][2] The word is derived from the Greek: βλέφαρον / blepharon, eyelid, and σπασμός / spasmos, spasm, an uncontrolled muscle contraction. The condition should be distinguished from the more common, and milder, involuntary quivering of an eyelid, known as myokymia or fasciculation.

Blepharospasm is a neurological disorder characterized by intermittent, involuntary spasms and contractions of the muscles around both eyes (bilateral). These result in abnormal twitching or blinking, and in the extreme, closure of the eyes.

Blepharospasm is one form of a group of movement disorders called dystonia.[2] The condition occurs in middle age and is more frequent among women than men.

Blepharospasm may be a primary or secondary disorder. The primary disorder is benign essential blepharospasm, in which term the qualifier essential indicates that the cause is unknown. Blepharospasm as a secondary disorder is caused by a disorder of the orofacial muscles, Meige's Syndrome, or specific ocular disease or condition, such as keratopy and irritation.

The most common first line treatments for blepharospasm are medication and injections of botulinum toxin into the eyelid protractor muscles.

Epidemiology

Blepharospasm is a fairly rare disease. Estimates of incidence and prevalence vary, tending to be higher in population studies than service studies,[3] likely because of delays in diagnosis.[2] In the United States, approximately 2,000 new cases of blepharospasm are diagnosed each year.[4] Estimates of incidence per million persons-years range from 14.5 in Northern California[5] to 100 in Taiwan.[6] Estimates of prevalence per million range from 12 in Olmsted County, Minnesota[7] to 133 in Puglia, Southern Italy.[8]

The onset of blepharospasm tends to be during the ages 40-60.[6][8] The condition is twice or more frequent among females than males,[5][6] which may be related to menopause.[9] In Taiwan, the condition was found to be more frequent among white-collar workers.[6]

Signs and symptoms

Blepharospasm usually begins with occasional twitches of both eyelids, which progress over time to forceful and frequent spasms and contractions of the eyelids. In severe episodes, the patient cannot open their eyelids (apraxia), which severely limits their daily activities. Prolonged closure of the eyelids may result in functional blindness.[2]

Typically, the symptoms---spasms and contractions of the eyelids---tend to worsen when the patient relaxes but abate during sleep.[10] The symptoms may be temporarily alleviated by sensory tricks (geste antagoniste) including stretching or rubbing the eyebrows, eyelids, or forehead,[11] and singing, talking, or humming.[12]

Patients suffering from blepharospasm report sensory symptoms including sensitivity to light,[13][14] dry eyes,[15] and burning sensation and grittiness in the eyes.[2] Although the symptoms tend to precede the onset of the blepharospasm, the may both be due to a common third factor.[16] Blepharospasm is aggravated by fatigue, stress, and environmental factors such as wind or air pollution.[17]

Although blepharospasm is defined as a bilaterally symmetric disorder that affects both eyes, some research has reported unilateral onset.[18][19]

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, light sensitivity, and even fatigue. Others report no eye problems before onset of symptoms.

Some drugs can induce blepharospasm, such as those used to treat Parkinson's disease, 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 can induce blepharospasm and is a known risk factor for the development of blepharospasm.[20]

Blepharospasm may also come from abnormal functioning of the brain's basal ganglia.[21] 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.[22]

Multiple sclerosis can cause blepharospasm.[23]

Blepharospasm is often associated with dry eyes, but the causal mechanism is still not clear.[15][6] Research in New York and Italy suggest that increased blinking (which may be triggered by dry eyes) leads to blepharospasm.[24][25] A case control study in China shows that blepharospasm aggravates dry eyes.[26]

In many cases, blepharospasm is associated with dystonia in other parts of the body.[27][2]

Diagnosis

Historically, blepharospasm was frequently misdiagnosed,[10] often as a psychiatric condition.[4]

Diagnosis has been enhanced by the proposal of objective criteria that start from "stereotyped, bilateral and synchronous orbicularis oculi spasms" and proceed to the identification of a "sensory trick" or "increased blinking".[28] The criteria have been validated diagnostic across multiple ethnicities in multiple centers.[29]

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 can be a safe and affordable therapeutic option for blepharospasm.[30]

Botulinum toxin injections have been used to induce localized, partial paralysis. Among most sufferers, botulinum toxin injection is the preferred treatment method.[31] 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.[32]

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.[33]

Although there is no cure, botulinum toxin injections may help temporarily.[34][35] A surgical procedure known as myectomy may also be useful.[34]

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, making it possible for patients to recover substantial function that was lost due to blepharospasm.[36][37][38]

  • See also

Multimedia

Neuroplasticity training

Blepharospasm Research Foundation

References

  1. ^ Defazio, Giovanni; Hallett, Mark; Jinnah, Hyder A.; Conte, Antonella; Berardelli, Alfredo (10 February 2017). "Blepharospasm 40 years later". Movement Disorders. 32 (4): 498–509. doi:10.1002/mds.26934. ISSN 0885-3185. PMC 5941939. PMID 28186662.
  2. ^ a b c d e f Scorr, Laura M.; Cho, Hyun Joo; Kilic-Berkmen, Gamze; McKay, J. Lucas; Hallett, Mark; Klein, Christine; Baumer, Tobias; Berman, Brian D.; Feuerstein, Jeanne S.; Perlmutter, Joel S.; Berardelli, Alfredo; Ferrazzano, Gina; Wagle-Shukla, Aparna; Malaty, Irene A.; Jankovic, Joseph (16 May 2022). "Clinical Features and Evolution of Blepharospasm: A Multicenter International Cohort and Systematic Literature Review". Dystonia. 1. doi:10.3389/dyst.2022.10359. ISSN 2813-2106. PMID 36248010.
  3. ^ Steeves, Thomas D.; Day, Lundy; Dykeman, Jonathan; Jette, Nathalie; Pringsheim, Tamara (31 October 2012). "The prevalence of primary dystonia: A systematic review and meta-analysis". Movement Disorders. 27 (14): 1789–1796. doi:10.1002/mds.25244. ISSN 0885-3185. PMID 23114997.
  4. ^ a b Simon, Guy J. Ben; McCann, John D. (Summer 2005). "Benign Essential Blepharospasm". International Ophthalmology Clinics. 45 (3): 49–75. doi:10.1097/01.iio.0000167238.26526.a8. ISSN 0020-8167. PMID 15970766.
  5. ^ a b Byrd, Erica; Albers, Kathleen; Goldman, Samuel; Klingman, Jeffrey; Lo, Raymond; Marras, Connie; Leimpeter, Amethyst; Fross, Robin; Comyns, Kathleen; Gu, Zhuqin; Katz, Maya; Ozelius, Laurie; Bressman, Susan; Saunders-Pullman, Rachel; Comella, Cynthia (5 April 2016). "Blepharospasm in a Multiethnic Population (P3.348)". Neurology. 86 (16_supplement). doi:10.1212/wnl.86.16_supplement.p3.348. ISSN 0028-3878.
  6. ^ a b c d e Sun, Yng; Tsai, Pei-Jhen; Chu, Chin-Liang; Huang, Wei-Chun; Bee, Youn-Shen (26 December 2018). "Epidemiology of benign essential blepharospasm: A nationwide population-based retrospective study in Taiwan". PLOS ONE. 13 (12): e0209558. Bibcode:2018PLoSO..1309558S. doi:10.1371/journal.pone.0209558. ISSN 1932-6203. PMC 6306223. PMID 30586395.
  7. ^ Bradley, Elizabeth A.; Hodge, David O.; Bartley, George B. (May 2003). "Benign Essential Blepharospasm Among Residents of Olmsted County, Minnesota, 1976 to 1995: An Epidemiologic Study". Ophthalmic Plastic & Reconstructive Surgery. 19 (3): 177–181. doi:10.1097/01.IOP.0000065203.88182.CF. ISSN 0740-9303. PMID 12918550.
  8. ^ a b Defazio, G.; Livrea, P.; De Salvia, R.; Manobianca, G.; Coviello, V.; Anaclerio, D.; Guerra, V.; Martino, D.; Valluzzi, F.; Liguori, R.; Logroscino, G. (12 June 2001). "Prevalence of primary blepharospasm in a community of Puglia region, Southern Italy". Neurology. 56 (11): 1579–1581. doi:10.1212/wnl.56.11.1579. ISSN 0028-3878. PMID 11402121.
  9. ^ Martino, Davide; Livrea, Paolo; Giorelli, Maurizio; Masi, Gianluca; Aniello, Maria Stella; Defazio, Giovanni (2002). "Menopause and Menarche in Patients with Primary Blepharospasm: An Exploratory Case-Control Study". European Neurology. 47 (3): 161–164. doi:10.1159/000047975. ISSN 0014-3022. PMID 11914554.
  10. ^ a b Defazio, Giovanni; Livrea, Paolo (2004). "Primary Blepharospasm". Drugs. 64 (3): 237–244. doi:10.2165/00003495-200464030-00002. ISSN 0012-6667.
  11. ^ Martino, Davide; Liuzzi, Daniele; Macerollo, Antonella; Aniello, Maria Stella; Livrea, Paolo; Defazio, Giovanni (15 March 2010). "The phenomenology of the geste antagoniste in primary blepharospasm and cervical dystonia". Movement Disorders. 25 (4): 407–412. doi:10.1002/mds.23011. ISSN 0885-3185. PMID 20108367.
  12. ^ Peckham, E. L.; Lopez, G.; Shamim, E. A.; Richardson, S. Pirio; Sanku, S.; Malkani, R.; Stacy, M.; Mahant, P.; Crawley, A.; Singleton, A.; Hallett, M. (17 February 2011). "Clinical features of patients with blepharospasm: a report of 240 patients". European Journal of Neurology. 18 (3): 382–386. doi:10.1111/j.1468-1331.2010.03161.x. ISSN 1351-5101. PMC 3934127. PMID 20649903.
  13. ^ Adams WH, Digre KB, Patel BC, Anderson RL, Warner JE, Katz BJ (July 2006). "The evaluation of light sensitivity in benign essential blepharospasm". American Journal of Ophthalmology. 142 (1): 82–87. doi:10.1016/j.ajo.2006.02.020. PMID 16815254.
  14. ^ Molloy, Anna; Williams, Laura; Kimmich, Okka; Butler, John S; Beiser, Ines; McGovern, Eavan; O'Riordan, Sean; Reilly, Richard B; Walsh, Cathal; Hutchinson, Michael (22 April 2015). "Sun exposure is an environmental factor for the development of blepharospasm". Journal of Neurology, Neurosurgery & Psychiatry. 87 (4): 420–424. doi:10.1136/jnnp-2014-310266. ISSN 0022-3050. PMID 25904812.
  15. ^ a b Elsron, J S; Marsden, C D; Grandas, F; Quinn, N P (July 1988). "The significance of ophthalmological symptoms in idiopathic blepharospasm". Eye. 2 (4): 435–439. doi:10.1038/eye.1988.79. ISSN 0950-222X. PMID 3253136.
  16. ^ Defazio, Giovanni; Abbruzzese, Giovanni; Stella Aniello, Maria; Di Fede, Roberta; Esposito, Marcello; Fabbrini, Giovanni; Girlanda, Paolo; Liguori, Rocco; Marinelli, Lucio; Martino, Davide; Morgante, Francesca; Santoro, Lucio; Tinazzi, Michele; Berardelli, Alfredo (15 December 2011). "Eye symptoms in relatives of patients with primary adult‐onset dystonia". Movement Disorders. 27 (2): 305–307. doi:10.1002/mds.24026. ISSN 0885-3185.
  17. ^ Coscarelli, Jandira Mourão (May 2010). "Essential Blepharospasm". Seminars in Ophthalmology. 25 (3): 104–108. doi:10.3109/08820538.2010.488564. ISSN 0882-0538.
  18. ^ Grandas, F; Elston, J; Quinn, N; Marsden, C D (1 June 1988). "Blepharospasm: a review of 264 patients". Journal of Neurology, Neurosurgery & Psychiatry. 51 (6): 767–772. doi:10.1136/jnnp.51.6.767. ISSN 0022-3050.
  19. ^ Hwang, WJ (2012). "Demographic and Clinical Features of Patients with Blepharospasm in Southern Taiwan: a university Hospital-Based Study". Acta Neurol Taiwan. 21: 108–114 – via PubMed: 23196730.
  20. ^ Wakakura M, Tsubouchi T, Inouye J (March 2004). "Etizolam and benzodiazepine induced blepharospasm". Journal of Neurology, Neurosurgery, and Psychiatry. 75 (3): 506–507. doi:10.1136/jnnp.2003.019869. PMC 1738986. PMID 14966178.
  21. ^ "Benign Essential Blepharospasm". NORD (National Organization for Rare Disorders). Retrieved 29 January 2020.
  22. ^ Martino, Davide; Defazio, Giovanni; Abbruzzese, Giovanni; Girlanda, Paolo; Tinazzi, Michele; Fabbrini, Giovanni; Aniello, Maria Stella; Avanzino, Laura; Colosimo, Carlo; Majorana, Giuseppe; Trompetto, Carlo (March 2007). "Head trauma in primary cranial dystonias: a multicentre case–control study". Journal of Neurology, Neurosurgery, and Psychiatry. 78 (3): 260–263. doi:10.1136/jnnp.2006.103713. ISSN 0022-3050. PMC 2117628. PMID 17056625.
  23. ^ Goldman, Lee (25 July 2011). Goldman's Cecil Medicine (24th ed.). Philadelphia: Elsevier Saunders. p. 2429. ISBN 978-1437727883.
  24. ^ Evinger, Craig; Bao, Jian-Bin; Powers, Alice S.; Kassem, Iris S.; Schicatano, Edward J.; Henriquez, Victor M.; Peshori, Kavita R. (31 January 2002). "Dry eye, blinking, and blepharospasm". Movement Disorders. 17 (S2): S75–S78. doi:10.1002/mds.10065. ISSN 0885-3185. PMC 3327285. PMID 11836761.
  25. ^ Conte, Antonella; Ferrazzano, Gina; Defazio, Giovanni; Fabbrini, Giovanni; Hallett, Mark; Berardelli, Alfredo (2 June 2017). "Increased Blinking May Be a Precursor of Blepharospasm: A Longitudinal Study". Movement Disorders Clinical Practice. 4 (5): 733–736. doi:10.1002/mdc3.12499. ISSN 2330-1619.
  26. ^ Lu, Rong; Huang, Ruisheng; Li, Kang; Zhang, Xinchun; Yang, Hui; Quan, Yadan; Li, Qian (March 2014). "The Influence of Benign Essential Blepharospasm on Dry Eye Disease and Ocular Inflammation". American Journal of Ophthalmology. 157 (3): 591–597.e2. doi:10.1016/j.ajo.2013.11.014. ISSN 0002-9394.
  27. ^ Abbruzzese, G; Berardelli, A; Girlanda, P; Marchese, R; Martino, D; Morgante, F; Avanzino, L; Colosimo, C; Defazio, G (1 April 2008). "Long-term assessment of the risk of spread in primary late-onset focal dystonia". Journal of Neurology, Neurosurgery & Psychiatry. 79 (4): 392–396. doi:10.1136/jnnp.2007.124594. ISSN 0022-3050.
  28. ^ Defazio, Giovanni; Hallett, Mark; Jinnah, Hyder A.; Berardelli, Alfredo (16 July 2013). "Development and validation of a clinical guideline for diagnosing blepharospasm". Neurology. 81 (3): 236–240. doi:10.1212/wnl.0b013e31829bfdf6. ISSN 0028-3878.
  29. ^ Defazio, Giovanni; Jinnah, Hyder A.; Berardelli, Alfredo; Perlmutter, Joel S.; Berkmen, Gamze Kilic; Berman, Brian D.; Jankovic, Joseph; Bäumer, Tobias; Comella, Cynthia; Cotton, Adam C.; Ercoli, Tommaso; Ferrazzano, Gina; Fox, Susan; Kim, Han-Joon; Moukheiber, Emile Sami (October 2021). "Diagnostic criteria for blepharospasm: A multicenter international study". Parkinsonism & Related Disorders. 91: 109–114. doi:10.1016/j.parkreldis.2021.09.004. ISSN 1353-8020.
  30. ^ Piyasena INAP; Jayasinghe JAC (March 2014). "Mosapride (5HT4 agonist) in the treatment of blepharospasm". Ceylon Med J. 59 (1): 26–27. doi:10.4038/cmj.v59i1.5527. PMID 24682196.
  31. ^ Schellini SA, Matai O, Igami TZ, Padovani CR, Padovani CP (2006). "Blefarospasmo essencial e espasmo hemifacial: características dos pacientes, tratamento com toxina botulínica A e revisão da literatura" [Essential blepharospasm and hemifacial spasm: characteristic of the patient, botulinum toxin A treatment and literature review]. Arquivos Brasileiros de Oftalmologia (in Portuguese). 69 (1): 23–26. doi:10.1590/S0004-27492006000100005. hdl:11449/68714. PMID 16491229.
  32. ^ Duarte, Gonçalo S; Rodrigues, Filipe B; Marques, Raquel E; Castelão, Mafalda; Ferreira, Joaquim; Sampaio, Cristina; Moore, Austen P; Costa, João (19 November 2020). Cochrane Movement Disorders Group (ed.). "Botulinum toxin type A therapy for blepharospasm". Cochrane Database of Systematic Reviews. 2020 (11): CD004900. doi:10.1002/14651858.CD004900.pub3. PMC 8094161. PMID 33211907.
  33. ^ Anderson RL, Patel BC, Holds JB, Jordan DR (September 1998). "Blepharospasm: past, present, and future". Ophthalmic Plastic and Reconstructive Surgery. 14 (5): 305–317. doi:10.1097/00002341-199809000-00002. PMID 9783280. S2CID 13678666.
  34. ^ a b "Facts About Blepharospasm". August 2009. Retrieved 19 March 2015.
  35. ^ Simpson, D. M.; Hallett, M.; Ashman, E. J.; Comella, C. L.; Green, M. W.; Gronseth, G. S.; Armstrong, M. J.; Gloss, D.; Potrebic, S.; Jankovic, J.; Karp, B. P.; Naumann, M.; So, Y. T.; Yablon, S. A. (18 April 2016). "Practice guideline update summary: Botulinum neurotoxin for the treatment of blepharospasm, cervical dystonia, adult spasticity, and headache: Report of the Guideline Development Subcommittee of the American Academy of Neurology". Neurology. 86 (19): 1818–1826. doi:10.1212/WNL.0000000000002560. PMC 4862245. PMID 27164716.
  36. ^ Farias, Joaquin (2016). Limitless. How your movements can heal your brain: An essay on the neurodynamics of dystonia. Mar Garcia Mejias: Galene Editions. ISBN 978-0-9951701-0-0.
  37. ^ Farias, Joaquin (2012). Intertwined. How to Induce Neuroplasticity: A New Approach to Rehabilitating Dystonias. Galene Editions. ISBN 978-8-4615512-4-8.
  38. ^ Farias, J., Sarti-Martínez, MA. Title: "Elite musicians treated by specific fingers motion program to stimulate propiceptive sense", Congreso Nacional De La Sociedad Anatómica Española, Alicante (España), European Journal of Anatomy, p. 110

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