blepharospasm

{{short description|Abnormal contraction or twitch of the eyelid}}

{{Redirect-several|for=other eye twitches|Fasciculation|Myokymia|dab=off}}

{{Infobox medical condition (new)

| name = Blepharospasm

| synonyms = Eye dystonia, Eye twitching, Eye spasm

| pronounce = {{IPAc-en|ˌ|b|l|ɛ|f|ə|r|oʊ-|ˈ|s|p|æ|z|əm}}

| field = Neurology, ophthalmology

| treatment =

| medication =

| prognosis =

| frequency =

| deaths =

}}

Blepharospasm is a neurological disorder characterized by intermittent, involuntary spasms and contractions of the orbicularis oculi (eyelid) muscles around both eyes.{{Cite journal |last=Hallett |first=Mark |date=2002-11-12 |title=Blepharospasm: Recent advances |url=https://www.neurology.org/doi/10.1212/01.WNL.0000027361.73814.0E |journal=Neurology |language=en |volume=59 |issue=9 |pages=1306–1312 |doi=10.1212/01.WNL.0000027361.73814.0E |pmid=12434791 |issn=0028-3878|url-access=subscription }}{{Cite journal |last1=Hallett |first1=Mark |last2=Evinger |first2=Craig |last3=Jankovic |first3=Joseph |last4=Stacy |first4=Mark |date=2008-10-14 |title=Update on blepharospasm |url=http://dx.doi.org/10.1212/01.wnl.0000327601.46315.85 |journal=Neurology |volume=71 |issue=16 |pages=1275–1282 |doi=10.1212/01.wnl.0000327601.46315.85 |pmid=18852443 |issn=0028-3878|pmc=2676990 }}{{Cite journal |last1=Defazio |first1=Giovanni |last2=Hallett |first2=Mark |last3=Jinnah |first3=Hyder A. |last4=Conte |first4=Antonella |last5=Berardelli |first5=Alfredo |date=2017-02-10 |title=Blepharospasm 40 years later |url=http://dx.doi.org/10.1002/mds.26934 |journal=Movement Disorders |volume=32 |issue=4 |pages=498–509 |doi=10.1002/mds.26934 |pmid=28186662 |pmc=5941939 |issn=0885-3185}}{{Cite journal |last1=Scorr |first1=Laura M. |last2=Cho |first2=Hyun Joo |last3=Kilic-Berkmen |first3=Gamze |last4=McKay |first4=J. Lucas |last5=Hallett |first5=Mark |last6=Klein |first6=Christine |last7=Baumer |first7=Tobias |last8=Berman |first8=Brian D. |last9=Feuerstein |first9=Jeanne S. |last10=Perlmutter |first10=Joel S. |last11=Berardelli |first11=Alfredo |last12=Ferrazzano |first12=Gina |last13=Wagle-Shukla |first13=Aparna |last14=Malaty |first14=Irene A. |last15=Jankovic |first15=Joseph |date=2022-05-16 |title=Clinical Features and Evolution of Blepharospasm: A Multicenter International Cohort and Systematic Literature Review |journal=Dystonia |volume=1 |doi=10.3389/dyst.2022.10359 |doi-access=free |pmid=36248010 |issn=2813-2106|pmc=9557246 }} These result in abnormal twitching or blinking, and in the extreme, sustained eyelid closure resulting in functional blindness.

The word blepharospasm 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 one form of a group of movement disorders called dystonia. It 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 may occur as secondary to conditions including dry eyes and other specific ocular disease or conditions, Meige's syndrome and other forms of dystonia, and Parkinson's disease and other movement disorders.

Blepharospasm occurs in middle age and is more frequent among women than men. The most common treatments are medication and periodic injections of botulinum toxin into the eyelid muscles.

Epidemiology

Blepharospasm is a fairly rare disease. Estimates of incidence and prevalence vary, tending to be higher in population studies than service studies,{{Cite journal |last1=Steeves |first1=Thomas D. |last2=Day |first2=Lundy |last3=Dykeman |first3=Jonathan |last4=Jette |first4=Nathalie |last5=Pringsheim |first5=Tamara |date=2012-10-31 |title=The prevalence of primary dystonia: A systematic review and meta-analysis |url=http://dx.doi.org/10.1002/mds.25244 |journal=Movement Disorders |volume=27 |issue=14 |pages=1789–1796 |doi=10.1002/mds.25244 |pmid=23114997 |issn=0885-3185}} likely because of delays in diagnosis. In the United States, approximately 2,000 new cases of blepharospasm are diagnosed each year.{{Cite journal |last1=Simon |first1=Guy J. Ben |last2=McCann |first2=John D. |date=Summer 2005 |title=Benign Essential Blepharospasm |url=https://journals.lww.com/internat-ophthalmology/citation/2005/04530/benign_essential_blepharospasm.7.aspx |journal=International Ophthalmology Clinics |language=en-US |volume=45 |issue=3 |pages=49–75 |doi=10.1097/01.iio.0000167238.26526.a8 |pmid=15970766 |issn=0020-8167|url-access=subscription }} Estimates of incidence per million persons-years range from 14.5 in Northern California{{Cite journal |last1=Byrd |first1=Erica |last2=Albers |first2=Kathleen |last3=Goldman |first3=Samuel |last4=Klingman |first4=Jeffrey |last5=Lo |first5=Raymond |last6=Marras |first6=Connie |last7=Leimpeter |first7=Amethyst |last8=Fross |first8=Robin |last9=Comyns |first9=Kathleen |last10=Gu |first10=Zhuqin |last11=Katz |first11=Maya |last12=Ozelius |first12=Laurie |last13=Bressman |first13=Susan |last14=Saunders-Pullman |first14=Rachel |last15=Comella |first15=Cynthia |date=2016-04-05 |title=Blepharospasm in a Multiethnic Population (P3.348) |url=http://dx.doi.org/10.1212/wnl.86.16_supplement.p3.348 |journal=Neurology |volume=86 |issue=16_supplement |doi=10.1212/wnl.86.16_supplement.p3.348 |issn=0028-3878|url-access=subscription }} to 100 in Taiwan.{{Cite journal |last1=Sun |first1=Yng |last2=Tsai |first2=Pei-Jhen |last3=Chu |first3=Chin-Liang |last4=Huang |first4=Wei-Chun |last5=Bee |first5=Youn-Shen |date=2018-12-26 |title=Epidemiology of benign essential blepharospasm: A nationwide population-based retrospective study in Taiwan |journal=PLOS ONE |language=en |volume=13 |issue=12 |pages=e0209558 |doi=10.1371/journal.pone.0209558 |doi-access=free |issn=1932-6203 |pmc=6306223 |pmid=30586395|bibcode=2018PLoSO..1309558S }} Estimates of prevalence per million range from 12 in Olmsted County, Minnesota{{Cite journal |last1=Bradley |first1=Elizabeth A. |last2=Hodge |first2=David O. |last3=Bartley |first3=George B. |date=May 2003 |title=Benign Essential Blepharospasm Among Residents of Olmsted County, Minnesota, 1976 to 1995: An Epidemiologic Study |url=https://journals.lww.com/op-rs/abstract/2003/05000/benign_essential_blepharospasm_among_residents_of.2.aspx |journal=Ophthalmic Plastic & Reconstructive Surgery |language=en-US |volume=19 |issue=3 |pages=177–181 |doi=10.1097/01.IOP.0000065203.88182.CF |pmid=12918550 |issn=0740-9303|url-access=subscription }} to 133 in Puglia, Southern Italy.{{Cite journal |last1=Defazio |first1=G. |last2=Livrea |first2=P. |last3=De Salvia |first3=R. |last4=Manobianca |first4=G. |last5=Coviello |first5=V. |last6=Anaclerio |first6=D. |last7=Guerra |first7=V. |last8=Martino |first8=D. |last9=Valluzzi |first9=F. |last10=Liguori |first10=R. |last11=Logroscino |first11=G. |date=2001-06-12 |title=Prevalence of primary blepharospasm in a community of Puglia region, Southern Italy |url=http://dx.doi.org/10.1212/wnl.56.11.1579 |journal=Neurology |volume=56 |issue=11 |pages=1579–1581 |doi=10.1212/wnl.56.11.1579 |pmid=11402121 |issn=0028-3878|url-access=subscription }}

The onset of blepharospasm tends to be during the ages 40–60. The condition is roughly more than twice as frequent among females than males, which may be related to menopause and hormone treatments.{{Cite journal |last1=Martino |first1=Davide |last2=Livrea |first2=Paolo |last3=Giorelli |first3=Maurizio |last4=Masi |first4=Gianluca |last5=Aniello |first5=Maria Stella |last6=Defazio |first6=Giovanni |date=2002 |title=Menopause and Menarche in Patients with Primary Blepharospasm: An Exploratory Case-Control Study |url=http://dx.doi.org/10.1159/000047975 |journal=European Neurology |volume=47 |issue=3 |pages=161–164 |doi=10.1159/000047975 |pmid=11914554 |issn=0014-3022|url-access=subscription }} In Taiwan, the condition is more frequent among white- than blue-collar workers.

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.

Patients suffering from blepharospasm also report sensory symptoms including sensitivity to light,{{cite journal |vauthors=Adams WH, Digre KB, Patel BC, Anderson RL, Warner JE, Katz BJ |date=July 2006 |title=The evaluation of light sensitivity in benign essential blepharospasm |journal=American Journal of Ophthalmology |volume=142 |issue=1 |pages=82–87 |doi=10.1016/j.ajo.2006.02.020 |pmid=16815254}}{{Cite journal |last1=Molloy |first1=Anna |last2=Williams |first2=Laura |last3=Kimmich |first3=Okka |last4=Butler |first4=John S |last5=Beiser |first5=Ines |last6=McGovern |first6=Eavan |last7=O'Riordan |first7=Sean |last8=Reilly |first8=Richard B |last9=Walsh |first9=Cathal |last10=Hutchinson |first10=Michael |date=2015-04-22 |title=Sun exposure is an environmental factor for the development of blepharospasm |url=http://dx.doi.org/10.1136/jnnp-2014-310266 |journal=Journal of Neurology, Neurosurgery & Psychiatry |volume=87 |issue=4 |pages=420–424 |doi=10.1136/jnnp-2014-310266 |issn=0022-3050 |pmid=25904812|url-access=subscription }} dry eyes,{{Cite journal |last1=Elsron |first1=J S |last2=Marsden |first2=C D |last3=Grandas |first3=F |last4=Quinn |first4=N P |date=July 1988 |title=The significance of ophthalmological symptoms in idiopathic blepharospasm |url=http://dx.doi.org/10.1038/eye.1988.79 |journal=Eye |volume=2 |issue=4 |pages=435–439 |doi=10.1038/eye.1988.79 |issn=0950-222X |pmid=3253136}} and burning sensation and grittiness in the eyes. Although such symptoms tend to precede the onset of the blepharospasm, they may both be due to a common third factor.{{Cite journal |last1=Defazio |first1=Giovanni |last2=Abbruzzese |first2=Giovanni |last3=Stella Aniello |first3=Maria |last4=Di Fede |first4=Roberta |last5=Esposito |first5=Marcello |last6=Fabbrini |first6=Giovanni |last7=Girlanda |first7=Paolo |last8=Liguori |first8=Rocco |last9=Marinelli |first9=Lucio |last10=Martino |first10=Davide |last11=Morgante |first11=Francesca |last12=Santoro |first12=Lucio |last13=Tinazzi |first13=Michele |last14=Berardelli |first14=Alfredo |date=2011-12-15 |title=Eye symptoms in relatives of patients with primary adult-onset dystonia |url=http://dx.doi.org/10.1002/mds.24026 |journal=Movement Disorders |volume=27 |issue=2 |pages=305–307 |doi=10.1002/mds.24026 |pmid=22173654 |issn=0885-3185|url-access=subscription }}

Typically, the symptoms—spasms and contractions of the eyelids—tend to worsen when the patient relaxes but abate during sleep.{{Cite journal |last1=Defazio |first1=Giovanni |last2=Livrea |first2=Paolo |date=2004 |title=Primary Blepharospasm |url=http://dx.doi.org/10.2165/00003495-200464030-00002 |journal=Drugs |volume=64 |issue=3 |pages=237–244 |doi=10.2165/00003495-200464030-00002 |pmid=14871168 |issn=0012-6667|url-access=subscription }} The symptoms may be temporarily alleviated by sensory tricks (geste antagoniste) including stretching or rubbing the eyebrows, eyelids, or forehead,{{Cite journal |last1=Martino |first1=Davide |last2=Liuzzi |first2=Daniele |last3=Macerollo |first3=Antonella |last4=Aniello |first4=Maria Stella |last5=Livrea |first5=Paolo |last6=Defazio |first6=Giovanni |date=2010-03-15 |title=The phenomenology of the geste antagoniste in primary blepharospasm and cervical dystonia |url=http://dx.doi.org/10.1002/mds.23011 |journal=Movement Disorders |volume=25 |issue=4 |pages=407–412 |doi=10.1002/mds.23011 |issn=0885-3185 |pmid=20108367|url-access=subscription }} and singing, talking, or humming.{{Cite journal |last1=Peckham |first1=E. L. |last2=Lopez |first2=G. |last3=Shamim |first3=E. A. |last4=Richardson |first4=S. Pirio |last5=Sanku |first5=S. |last6=Malkani |first6=R. |last7=Stacy |first7=M. |last8=Mahant |first8=P. |last9=Crawley |first9=A. |last10=Singleton |first10=A. |last11=Hallett |first11=M. |date=2011-02-17 |title=Clinical features of patients with blepharospasm: a report of 240 patients |url=http://dx.doi.org/10.1111/j.1468-1331.2010.03161.x |journal=European Journal of Neurology |volume=18 |issue=3 |pages=382–386 |doi=10.1111/j.1468-1331.2010.03161.x |issn=1351-5101 |pmc=3934127 |pmid=20649903}} Blepharospasm is aggravated by fatigue, stress, and environmental factors such as wind or air pollution.{{Cite journal |last=Coscarelli |first=Jandira Mourão |date=May 2010 |title=Essential Blepharospasm |url=http://www.tandfonline.com/doi/full/10.3109/08820538.2010.488564 |journal=Seminars in Ophthalmology |language=en |volume=25 |issue=3 |pages=104–108 |doi=10.3109/08820538.2010.488564 |pmid=20590421 |issn=0882-0538|url-access=subscription }}

Although blepharospasm is defined as a bilaterally symmetric disorder that affects both eyes, some research has reported unilateral onset.{{Cite journal |last1=Grandas |first1=F |last2=Elston |first2=J |last3=Quinn |first3=N |last4=Marsden |first4=C D |date=1988-06-01 |title=Blepharospasm: a review of 264 patients. |url=http://dx.doi.org/10.1136/jnnp.51.6.767 |journal=Journal of Neurology, Neurosurgery & Psychiatry |volume=51 |issue=6 |pages=767–772 |doi=10.1136/jnnp.51.6.767 |pmid=3404184 |issn=0022-3050|pmc=1033145 }}{{Cite journal |last=Hwang |first=WJ |date=2012 |title=Demographic and Clinical Features of Patients with Blepharospasm in Southern Taiwan: a university Hospital-Based Study |journal=Acta Neurol Taiwan |volume=21 |issue=3 |pages=108–114 |pmid=23196730 }}

Causes

Historically, it was believed that blepharospasm was due to the abnormal functioning of the brain's basal ganglia.{{Cite journal |last=Marsden |first=C D |date=1976-12-01 |title=Blepharospasm-oromandibular dystonia syndrome (Brueghel's syndrome). A variant of adult-onset torsion dystonia? |url=http://dx.doi.org/10.1136/jnnp.39.12.1204 |journal=Journal of Neurology, Neurosurgery & Psychiatry |volume=39 |issue=12 |pages=1204–1209 |doi=10.1136/jnnp.39.12.1204 |pmid=1011031 |issn=0022-3050|pmc=492566 }} The basal ganglia are structures in the brain that are involved in the regulation of motor and reward functions.

However, blepharospasm is now known to involve several regions of the brain and to be a multifactorial condition in which "one or several as yet unknown genes together with epigenetic and environmental factors combine to reach the threshold that induces the disease".

Blepharospasm is often associated with dry eyes, but the causal mechanism is still not clear. Research in New York and Italy suggests that increased blinking (which may be triggered by dry eyes) leads to blepharospasm.{{Cite journal |last1=Evinger |first1=Craig |last2=Bao |first2=Jian-Bin |last3=Powers |first3=Alice S. |last4=Kassem |first4=Iris S. |last5=Schicatano |first5=Edward J. |last6=Henriquez |first6=Victor M. |last7=Peshori |first7=Kavita R. |date=2002-01-31 |title=Dry eye, blinking, and blepharospasm |url=http://dx.doi.org/10.1002/mds.10065 |journal=Movement Disorders |volume=17 |issue=S2 |pages=S75–S78 |doi=10.1002/mds.10065 |issn=0885-3185 |pmc=3327285 |pmid=11836761}}{{Cite journal |last1=Conte |first1=Antonella |last2=Ferrazzano |first2=Gina |last3=Defazio |first3=Giovanni |last4=Fabbrini |first4=Giovanni |last5=Hallett |first5=Mark |last6=Berardelli |first6=Alfredo |date=2017-06-02 |title=Increased Blinking May Be a Precursor of Blepharospasm: A Longitudinal Study |url=http://dx.doi.org/10.1002/mdc3.12499 |journal=Movement Disorders Clinical Practice |volume=4 |issue=5 |pages=733–736 |doi=10.1002/mdc3.12499 |pmid=29082270 |issn=2330-1619 |pmc=5654574}} A case control study in China found that blepharospasm aggravated dry eyes.{{Cite journal |last1=Lu |first1=Rong |last2=Huang |first2=Ruisheng |last3=Li |first3=Kang |last4=Zhang |first4=Xinchun |last5=Yang |first5=Hui |last6=Quan |first6=Yadan |last7=Li |first7=Qian |date=March 2014 |title=The Influence of Benign Essential Blepharospasm on Dry Eye Disease and Ocular Inflammation |url=http://dx.doi.org/10.1016/j.ajo.2013.11.014 |journal=American Journal of Ophthalmology |volume=157 |issue=3 |pages=591–597.e2 |doi=10.1016/j.ajo.2013.11.014 |pmid=24269849 |issn=0002-9394|url-access=subscription }}

Blepharospasm may be associated with dystonia in other parts of the body, particularly Meige's Syndrome.{{Cite journal |last1=Jankovic |first1=Joseph |last2=Ford |first2=Janet |date=April 1983 |title=Blepharospasm and orofacial-cervical dystonia: Clinical and pharmacological findings in 100 patients |url=https://onlinelibrary.wiley.com/doi/10.1002/ana.410130406 |journal=Annals of Neurology |language=en |volume=13 |issue=4 |pages=402–411 |doi=10.1002/ana.410130406 |pmid=6838174 |issn=0364-5134|url-access=subscription }}{{Cite journal |last1=Abbruzzese |first1=G |last2=Berardelli |first2=A |last3=Girlanda |first3=P |last4=Marchese |first4=R |last5=Martino |first5=D |last6=Morgante |first6=F |last7=Avanzino |first7=L |last8=Colosimo |first8=C |last9=Defazio |first9=G |date=2008-04-01 |title=Long-term assessment of the risk of spread in primary late-onset focal dystonia |url=http://dx.doi.org/10.1136/jnnp.2007.124594 |journal=Journal of Neurology, Neurosurgery & Psychiatry |volume=79 |issue=4 |pages=392–396 |doi=10.1136/jnnp.2007.124594 |pmid=17635969 |issn=0022-3050|url-access=subscription }} Blepharospasm may be associated with Parkinson's Disease, but the causal mechanism is still not clear.{{Cite journal |last1=Micheli |first1=Federico |last2=Scorticati |first2=María Clara |last3=Folgar |first3=Silvia |last4=Gatto |first4=Emilia |date=September 2004 |title=Development of Parkinson's disease in patients with blepharospasm |url=https://movementdisorders.onlinelibrary.wiley.com/doi/10.1002/mds.20084 |journal=Movement Disorders |language=en |volume=19 |issue=9 |pages=1069–1072 |doi=10.1002/mds.20084 |pmid=15372598 |issn=0885-3185|url-access=subscription }}{{Cite journal |last1=Rana |first1=Abdul-Qayyum |last2=Kabir |first2=Ashish |last3=Dogu |first3=Okan |last4=Patel |first4=Ami |last5=Khondker |first5=Sumaiya |date=2012-10-11 |title=Prevalence of Blepharospasm and Apraxia of Eyelid Opening in Patients with Parkinsonism, Cervical Dystonia and Essential Tremor |url=https://doi.org/10.1159/000341621 |journal=European Neurology |volume=68 |issue=5 |pages=318–321 |doi=10.1159/000341621 |pmid=23075668 |issn=0014-3022|url-access=subscription }} In rare cases, blepharospasm is associated with multiple sclerosis.{{Cite journal |last1=Nociti |first1=V |last2=Bentivoglio |first2=Ar |last3=Frisullo |first3=G |last4=Fasano |first4=A |last5=Soleti |first5=F |last6=Iorio |first6=R |last7=Loria |first7=G |last8=Patanella |first8=Ak |last9=Marti |first9=A |last10=Tartaglione |first10=T |last11=Tonali |first11=Pa |last12=Batocchi |first12=Ap |date=November 2008 |title=Movement disorders in multiple sclerosis: causal or coincidental association? |url=http://journals.sagepub.com/doi/10.1177/1352458508094883 |journal=Multiple Sclerosis Journal |language=en |volume=14 |issue=9 |pages=1284–1287 |doi=10.1177/1352458508094883 |pmid=18768580 |issn=1352-4585|url-access=subscription }}{{Cite journal |last1=Edechi |first1=Chidalu A. |last2=Micieli |first2=Jonathan A. |date=2022-04-28 |title=Blepharospasm and Sixth Nerve Palsy as the Presenting Sign of Multiple Sclerosis |url=https://journals.lww.com/jneuro-ophthalmology/citation/9900/blepharospasm_and_sixth_nerve_palsy_as_the.389.aspx |journal=Journal of Neuro-Ophthalmology |volume=44 |issue=3 |language=en-US |pages=e412–e413 |doi=10.1097/WNO.0000000000001916 |pmid=37389956 |issn=1070-8022|url-access=subscription }}

Some drugs can induce blepharospasm, including those used to treat depression{{cite journal |vauthors=Wakakura M, Tsubouchi T, Inouye J |date=March 2004 |title=Etizolam and benzodiazepine induced blepharospasm |journal=Journal of Neurology, Neurosurgery, and Psychiatry |volume=75 |issue=3 |pages=506–507 |doi=10.1136/jnnp.2003.019869 |pmc=1738986 |pmid=14966178}}{{Cite journal |last1=Wakakura |first1=M. |last2=Yamagami |first2=A. |last3=Iwasa |first3=M. |date=2018-09-03 |title=Blepharospasm in Japan: A Clinical Observational Study From a Large Referral Hospital in Tokyo |journal=Neuro-Ophthalmology |language=en |volume=42 |issue=5 |pages=275–283 |doi=10.1080/01658107.2017.1409770 |issn=0165-8107 |pmc=6152494 |pmid=30258472}} and Parkinson's disease.{{Cite journal |last1=Mauriello |first1=Joseph A. |last2=Carbonaro |first2=Paul |last3=Dhillon |first3=Shamina |last4=Leone |first4=Tina |last5=Franklin |first5=Mark |date=June 1998 |title=Drug-Associated Facial Dyskinesias—A Study of 238 Patients |url=https://journals.lww.com/jneuro-ophthalmology/abstract/1998/06000/Drug_Associated_Facial_Dyskinesias_A_Study_of_238.16.aspx |journal=Journal of Neuro-Ophthalmology |language=en-US |volume=18 |issue=2 |pages=153–157 |doi=10.1097/00041327-199806000-00016 |pmid=9621275 |issn=1070-8022}} Hormone replacement therapy for women going through menopause has been found to be associated with dry eyes,{{Cite journal |last=Liesegang |first=Thomas J |date=March 2002 |title=Hormone replacement therapy and dry eye syndrome. Schaumberg DA,∗∗Division of Preventive Medicine, Brigham and Women's Hospital, Harvard Medical School, 900 Commonwealth Ave E, Boston, MA 02215. USA E-mail: schaumberg@rics.bwh.harvard.edu Buring JE, Sullivan DA, Dana MR. JAMA 2001;286:2114–2119. |url=http://dx.doi.org/10.1016/s0002-9394(02)01368-5 |journal=American Journal of Ophthalmology |volume=133 |issue=3 |pages=435–436 |doi=10.1016/s0002-9394(02)01368-5 |issn=0002-9394|url-access=subscription }} which in turn is associated with blepharospasm.

Blepharospasm can be caused by concussions in some rare cases, when a blow to the back of the head damages the basal ganglia.{{Cite journal|last1=Martino|first1=Davide|last2=Defazio|first2=Giovanni|last3=Abbruzzese|first3=Giovanni|last4=Girlanda|first4=Paolo|last5=Tinazzi|first5=Michele|last6=Fabbrini|first6=Giovanni|last7=Aniello|first7=Maria Stella|last8=Avanzino|first8=Laura|last9=Colosimo|first9=Carlo|last10=Majorana|first10=Giuseppe|last11=Trompetto|first11=Carlo|date=March 2007|title=Head trauma in primary cranial dystonias: a multicentre case–control study|journal=Journal of Neurology, Neurosurgery, and Psychiatry|volume=78|issue=3|pages=260–263|doi=10.1136/jnnp.2006.103713|issn=0022-3050|pmc=2117628|pmid=17056625}}

Blepharospasm is associated with exposure to the sun.{{Cite journal |last1=Molloy |first1=Anna |last2=Williams |first2=Laura |last3=Kimmich |first3=Okka |last4=Butler |first4=John S. |last5=Beiser |first5=Ines |last6=McGovern |first6=Eavan |last7=O'Riordan |first7=Sean |last8=Reilly |first8=Richard B. |last9=Walsh |first9=Cathal |last10=Hutchinson |first10=Michael |date=2016-04-01 |title=Sun exposure is an environmental factor for the development of blepharospasm |url=https://jnnp.bmj.com/content/87/4/420 |journal=Journal of Neurology, Neurosurgery & Psychiatry |language=en |volume=87 |issue=4 |pages=420–424 |doi=10.1136/jnnp-2014-310266 |issn=0022-3050 |pmid=25904812|url-access=subscription }}

Diagnosis

No laboratory tests exist with which to definitively diagnose blepharospasm. Historically, the condition was frequently misdiagnosed, often as a psychiatric condition.

Diagnosis of blepharospasm has been enhanced by the proposal of objective diagnostic criteria that start from "stereotyped, bilateral and synchronous orbicularis oculi spasms" and proceed to the identification of a "sensory trick" or "increased blinking".{{Cite journal |last1=Defazio |first1=Giovanni |last2=Hallett |first2=Mark |last3=Jinnah |first3=Hyder A. |last4=Berardelli |first4=Alfredo |date=2013-07-16 |title=Development and validation of a clinical guideline for diagnosing blepharospasm |url=http://dx.doi.org/10.1212/wnl.0b013e31829bfdf6 |journal=Neurology |volume=81 |issue=3 |pages=236–240 |doi=10.1212/wnl.0b013e31829bfdf6 |pmid=23771487 |issn=0028-3878|pmc=3770163 }} The criteria have been validated across multiple ethnicities in multiple centers.{{Cite journal |last1=Defazio |first1=Giovanni |last2=Jinnah |first2=Hyder A. |last3=Berardelli |first3=Alfredo |last4=Perlmutter |first4=Joel S. |last5=Berkmen |first5=Gamze Kilic |last6=Berman |first6=Brian D. |last7=Jankovic |first7=Joseph |last8=Bäumer |first8=Tobias |last9=Comella |first9=Cynthia |last10=Cotton |first10=Adam C. |last11=Ercoli |first11=Tommaso |last12=Ferrazzano |first12=Gina |last13=Fox |first13=Susan |last14=Kim |first14=Han-Joon |last15=Moukheiber |first15=Emile Sami |date=October 2021 |title=Diagnostic criteria for blepharospasm: A multicenter international study |url=http://dx.doi.org/10.1016/j.parkreldis.2021.09.004 |journal=Parkinsonism & Related Disorders |volume=91 |pages=109–114 |doi=10.1016/j.parkreldis.2021.09.004 |pmid=34583301 |issn=1353-8020|pmc=9048224 }}

Treatment

Standard first line treatments of blepharospasm are conservative therapies, oral medication, and periodic injections of botulinum toxin.

Particularly when associated with dry eyes, blepharospasm may be relieved with warm compresses, eye drops, and eye wipes.{{Cite journal |last1=Murakami |first1=David K. |last2=Blackie |first2=Caroline A. |last3=Korb |first3=Donald R. |date=September 2015 |title=All Warm Compresses Are Not Equally Efficacious |url=https://journals.lww.com/optvissci/abstract/2015/09000/all_warm_compresses_are_not_equally_efficacious.29.aspx |journal=Optometry and Vision Science |language=en-US |volume=92 |issue=9 |pages=e327-33 |doi=10.1097/OPX.0000000000000675 |pmid=26164316 |issn=1538-9235|url-access=subscription }}{{Cite journal |last1=Defazio |first1=Giovanni |last2=Livrea |first2=Paolo |date=2004-02-01 |title=Primary Blepharospasm |url=https://doi.org/10.2165/00003495-200464030-00002 |journal=Drugs |language=en |volume=64 |issue=3 |pages=237–244 |doi=10.2165/00003495-200464030-00002 |pmid=14871168 |issn=1179-1950|url-access=subscription }} A Japanese study showed that warm compresses containing menthol were more effective in increasing tear film.{{Cite journal |last1=Arita |first1=Reiko |last2=Morishige |first2=Naoyuki |last3=Sakamoto |first3=Ichiro |last4=Imai |first4=Natsuko |last5=Shimada |first5=Yuko |last6=Igaki |first6=Michihito |last7=Suzuki |first7=Atsushi |last8=Itoh |first8=Kouzo |last9=Tsubota |first9=Kazuo |date=2017-04-05 |title=Effects of a warm compress containing menthol on the tear film in healthy subjects and dry eye patients |journal=Scientific Reports |language=en |volume=7 |issue=1 |pages=45848 |doi=10.1038/srep45848 |pmid=28378793 |issn=2045-2322|pmc=5381094 |bibcode=2017NatSR...745848A }}

Drugs used to treat blepharospasm are anticholinergics, benzodiazepines, baclofen, and tetrabenazine.{{Cite journal |last1=Vijayakumar |first1=Dhanya |last2=Jankovic |first2=Joseph |date=2018-07-04 |title=Medical treatment of blepharospasm |url=https://www.tandfonline.com/doi/full/10.1080/17469899.2018.1503535 |journal=Expert Review of Ophthalmology |language=en |volume=13 |issue=4 |pages=233–243 |doi=10.1080/17469899.2018.1503535 |issn=1746-9899|url-access=subscription }} The proportion of patients who benefited from anticholinergics ranged from 1 in 9 in Oregon{{Cite journal |last1=Nutt |first1=John G. |last2=Hammerstad |first2=John P. |last3=de Garmo |first3=Pat |last4=Carter |first4=Julie |date=February 1984 |title=Cranial dystonia: Double-blind crossover study of anticholinergics |url=https://www.neurology.org/doi/10.1212/WNL.34.2.215 |journal=Neurology |language=en |volume=34 |issue=2 |pages=215–217 |doi=10.1212/WNL.34.2.215 |pmid=6363970 |issn=0028-3878|url-access=subscription }} to 1 in 5 in England. Besides failing to resolve the blepharospasm, some drugs present the risk of side effects. In Japan, use of etizolam and benzodiazepine was associated with the development of blepharospasm. A case report from Sri Lanka suggests treatment with Mosapride.{{cite journal |author1=Piyasena INAP |author2=Jayasinghe JAC |title=Mosapride (5HT4 agonist) in the treatment of blepharospasm. |journal=Ceylon Med. J. |volume=59 |issue=1 |pages=26–27 |date = March 2014|pmid=24682196 |doi=10.4038/cmj.v59i1.5527|doi-access=free }}

The main first-line therapy is periodic injections of botulinum toxin type A to induce localized, partial paralysis of the eyelid muscles.{{Cite journal |last1=Duarte |first1=Gonçalo S |last2=Rodrigues |first2=Filipe B |last3=Marques |first3=Raquel E |last4=Castelão |first4=Mafalda |last5=Ferreira |first5=Joaquim |last6=Sampaio |first6=Cristina |last7=Moore |first7=Austen P |last8=Costa |first8=João |date=2020-11-19 |editor-last=Cochrane Movement Disorders Group |title=Botulinum toxin type A therapy for blepharospasm |journal=Cochrane Database of Systematic Reviews |language=en |volume=2020 |issue=11 |pages=CD004900 |doi=10.1002/14651858.CD004900.pub3 |pmc=8094161 |pmid=33211907}}{{cite journal |last1=Simpson |first1=D. M. |last2=Hallett |first2=M. |last3=Ashman |first3=E. J. |last4=Comella |first4=C. L. |last5=Green |first5=M. W. |last6=Gronseth |first6=G. S. |last7=Armstrong |first7=M. J. |last8=Gloss |first8=D. |last9=Potrebic |first9=S. |last10=Jankovic |first10=J. |last11=Karp |first11=B. P. |last12=Naumann |first12=M. |last13=So |first13=Y. T. |last14=Yablon |first14=S. A. |date=18 April 2016 |title=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 |journal=Neurology |volume=86 |issue=19 |pages=1818–1826 |doi=10.1212/WNL.0000000000002560 |pmc=4862245 |pmid=27164716}} Injections are generally administered at intervals of around 10 weeks, with variations based on patient response and usually give fairly quick relief from the muscle spasms. An English study reported that 118 (78%) of 151 patients experienced significant relief of symptoms for a mean duration of 9.2 weeks. However, in a minority of patients, the injections do not provide any symptomatic relief. Injections of botulinum toxin may diminish in effectiveness with prolonged use and require increased dosage.{{Cite journal |last1=Ababneh |first1=Osama H |last2=Cetinkaya |first2=Altug |last3=Kulwin |first3=Dwight R |date=April 2014 |title=Long-term efficacy and safety of botulinum toxin A injections to treat blepharospasm and hemifacial spasm |url=https://onlinelibrary.wiley.com/doi/10.1111/ceo.12165 |journal=Clinical & Experimental Ophthalmology |language=en |volume=42 |issue=3 |pages=254–261 |doi=10.1111/ceo.12165 |pmid=23844601 |issn=1442-6404|url-access=subscription }} Injections of botulinum toxin increase the risk of visual complaints and ptosis (eyelid droop).

Patients who do not respond well to medication or botulinum toxin injections are candidates for surgical therapy. The most effective surgical treatment has been protractor myectomy, the removal of muscles responsible for eyelid closure.{{cite journal |vauthors=Anderson RL, Patel BC, Holds JB, Jordan DR |title=Blepharospasm: past, present, and future |journal=Ophthalmic Plastic and Reconstructive Surgery |volume=14 |issue=5 |pages=305–317 |date = September 1998|pmid=9783280 |doi=10.1097/00002341-199809000-00002|s2cid=13678666 }} Myectomy is more effective than distal neurectomy.{{Cite journal |last1=Waller |first1=R R |last2=Kennedy |first2=R H |last3=Henderson |first3=J W |last4=Kesty |first4=K R |date=1985 |title=Management of blepharospasm. |journal=Transactions of the American Ophthalmological Society |volume=83 |pages=367–386 |issn=0065-9533 |pmc=1298706 |pmid=3832534}}

A case report from California suggests the use of intense pulsed light therapy to relieve blepharospasm.{{Cite journal |last1=Monterastelli |first1=Anna N. |last2=Bailey |first2=Lucy A. |last3=Cockerham |first3=Kimberly |date=June 2022 |title=Intense Pulsed Light: A Therapeutic Option for Patients With Rosacea-Related Dry Eye and Blepharospasm |url=https://journals.lww.com/jneuro-ophthalmology/fulltext/2022/06000/Intense_Pulsed_Light__A_Therapeutic_Option_for.36.aspx |journal=Journal of Neuro-Ophthalmology |language=en-US |volume=42 |issue=2 |pages=e494–e496 |doi=10.1097/WNO.0000000000001290 |pmid=34270518 |issn=1070-8022|url-access=subscription }} Patients suffering from blepharospasm may get relief by wearing spectacles fitted to lift the upper eyelid. Among complementary therapies, two simulate sensory tricks: Attaching a device to spectacle frames to press on the patient's temple,{{Cite journal |last1=Fantato |first1=Alexina |last2=Parulekar |first2=Manoj |last3=Elston |first3=John |date=2019-07-02 |title=A trial of a mechanical device for the treatment of blepharospasm |url=http://dx.doi.org/10.1038/s41433-019-0495-y |journal=Eye |volume=33 |issue=11 |pages=1803–1808 |doi=10.1038/s41433-019-0495-y |pmid=31267089 |issn=0950-222X|pmc=7002569 }} and applying thin cosmetic tapes to the forehead and eyebrows.{{Cite journal |last1=Uchida |first1=Kazuko |last2=Kiyosawa |first2=Motohiro |last3=Wakakura |first3=Masato |date=2020 |title=Efficacy of a Non-invasive Cosmetic Forcible Trick Treatment for Blepharospasm: Increase in Quality of Life Due to Use of Ultra-thin Adhesive Tape |url=https://doi.org/10.11476/shinkeiganka.37.237 |journal=NeuroOphthalmology Japan |volume=37 |issue=2 |pages=237–243 |doi=10.11476/shinkeiganka.37.237 |issn= |via=JStage}} Another complementary therapy is retraining the brain to "rewire" itself and eliminate dystonic movements. Associated with [https://www.fariastechnique.com/ Joaquin Farias], sensorimotor retraining activities and proprioceptive stimulation aim to induce neuroplasticity, making it possible for patients to recover substantial function that was lost due to blepharospasm.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 Complementary therapies lack evidence of the highest quality (Level 1).

Research

The U.S. National Library of Medicine maintains a register of clinical trials of therapies to treat [https://classic.clinicaltrials.gov/ct2/results?cond=blepharospasm&term=&cntry=&state=&city=&dist= blepharospasm].

Multimedia

Blepharospasm Research Foundation

  • Victoria S. Pelak, [https://www.youtube.com/watch?v=gT6c_grVTRI Relationships of Blepharospasm to Ophthalmic Conditions such as Dry Eye], BEBRF Symposium, August 6, 2016.
  • Charles N.S. Soparkar, [https://www.youtube.com/watch?v=7b6dOoqhsl0 Blepharospasm and dry eyes], BEBRF Symposium, September 9, 2017.
  • [https://www.youtube.com/watch?v=gLJPC30Ca9s Complementary/alternative therapies], Philadelphia, October 9, 2021

Neuroplasticity training

  • Federico Bitti, [https://www.youtube.com/watch?v=DwkHK3rfKO0 "Dystonia. Rewiring the brain through movement and dance", TEDxNapoli]
  • Joaquin Farias, [https://www.youtube.com/watch?v=czW-xBvDtHY "How your movements can heal your brain", TEDxNapoli]

References

{{Reflist}}