Blepharospasm [aka, blinking eye] is a focal dystonia involving the orbicularis muscle causing repeated and abnormal movements of the eyelids. Patients with blepharospasm may be characterized by various types of involuntary activation of periocular muscles, and not only the orbicularis, producing different tics and movements. (Defazio et al, 2017) The symptoms are typically triggered by stress, fatigue, intense light, or individual factors. (Coscarelli, 2010).
The twitching often happens during times when the patient is tired, stressed, or anxious, or when exposed to bright light and sunlight. In some cases, a patient with a primary headache could experience ptosis, which could be confounded with blepharospasm. In those cases, the condition will be solved as the headache decreases intensity. (Anagnostou et al, 2017).
Clinical Characteristics
Although prolonged spasms of the orbicularis oculi muscles remain the clinical hallmark of blepharospasm, patients with blepharospasm may be characterized by various types of involuntary activation of periocular muscles. In addition to motor features, blepharospasm patients may also have nonmotor manifestations, including psychiatric, mild cognitive, and sensory disturbances. (Rippey, 2020)
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A more complex condition is Meige Syndrome, where the blepharospasm is accompanied by oromandibular movements of lower facial muscles, mouth, jaw, tongue, pharyngeal and cervical muscles. (Pandey & Sharma, 2016) The etiology and pathogenesis of this disorder are not well-understood, and dopaminergic and cholinergic hyperactivity has been proposed (Gautam et al, 2016). A small group of patients has a family history of the disease, but to-date no causative genes have been identified. (Ma et al, 2021) If the laryngeal muscles are involved, the patient might experience difficulties breathing, with the need to address those muscles specifically. (Watson et al, 2021)
A co-morbidity with dry eye syndrome has been reported. Dry eye disease is a chronic condition of the corneal surface marked by persistent symptoms of irritation or burning that can cause inflammatory damage to the cornea and conjunctiva if untreated. (Rouen and White, 2018)
Treatment
Several strategies have been proposed, but the first line of treatment is the application of botulinum neurotoxin. (Hassell & Charles, 2020) In some cases, the patients can’t open their eyes and might require a surgical approach with modification of the lids. (Dinjar et al, 2020) Pharmacotherapy in general has poor results. (Borodic & Cozzolino, 1989)
Similar conditions
- Ocular deviations related to tardive syndromes such as Tourette. (Savitt, 2018)
- Ocular tics (eye rolling, blinking, and widening). (Shawkat, 1992)
Ocular manifestation of viral diseases such as dengue. (Merle et al, 20218) - Intracranial diseases, such as hemorrhages. (Chang et al, 2021)
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References
- Defazio G, Hallett M, Jinnah HA, Conte A, Berardelli A. Blepharospasm 40 years later. Mov Disord. 2017 Apr;32(4):498-509.
- Coscarelli JM. Essential blepharospasm. Semin Ophthalmol. 2010 May;25(3):104-8.
- Anagnostou E, Vikelis M, Tzavellas E, Ghika A, Kouzi I, Evdokimidis I, Kararizou E. Photophobia in primary headaches, in essential blepharospasm and in major depression. Int J Neurosci. 2017 Aug;127(8):673-679.
- Pandey S, Sharma S. Meige’s syndrome: History, epidemiology, clinical features, pathogenesis and treatment. J Neurol Sci. 2017 Jan 15;372:162-170.
- Gautam P, Bhatia MS, Kaur J, Rathi A. Meige’s syndrome. Ind Psychiatry J. 2016 Jul-Dec;25(2):232-233.
- Hassell TJW, Charles D. Treatment of Blepharospasm and Oromandibular Dystonia with Botulinum Toxins. Toxins (Basel). 2020 Apr 22;12(4):269.
- Ma H, Qu J, Ye L, Shu Y, Qu Q. Blepharospasm, Oromandibular Dystonia, and Meige Syndrome: Clinical and Genetic Update. Front Neurol. 2021 Mar 29;12:630221.
- Watson NA, Hicklin LA, Marion MH. Breathing dystonia in Meige syndrome. Clin Park Relat Disord. 2021 Aug 28;5:100106.
- Rouen PA, White ML. Dry Eye Disease: Prevalence, Assessment, and Management. Home Healthc Now. 2018 Mar/Apr;36(2):74-83.
- Dinjar K, Zubčić V, Kopić A, Kopić V, Popić B, Holik D. SURGICAL ASPECT OF BLEPHAROSPASM TREATMENT: A CASE REPORT. Acta Clin Croat. 2020 Jun;59(2):377-381.
- Borodic GE, Cozzolino D. Blepharospasm and its treatment, with emphasis on the use of botulinum toxin. Plast Reconstr Surg. 1989 Mar;83(3):546-54.
- Savitt D, Jankovic J. Tardive syndromes. J Neurol Sci. 2018 Jun 15;389:35-42. doi: 10.1016/j.jns.2018.02.005. Epub 2018 Feb 5. PMID: 29506749.
- Shawkat F, Harris CM, Jacobs M, Taylor D, Brett EM. Eye movement tics. Br J Ophthalmol. 1992 Nov;76(11):697-9. doi: 10.1136/bjo.76.11.697. PMID: 1477052; PMCID: PMC504381.
- Merle H, Donnio A, Jean-Charles A, Guyomarch J, Hage R, Najioullah F, Césaire R, Cabié A. Manifestations oculaires des arboviroses émergentes : dengue, chikungunya, infection à virus Zika, fièvre du Nil occidental et fièvre jaune [Ocular manifestations of emerging arboviruses: Dengue fever, Chikungunya, Zika virus, West Nile virus, and yellow fever (French translation of the article)]. J Fr Ophtalmol. 2018 Sep;41(7):659-668. French. doi: 10.1016/j.jfo.2018.03.005. Epub 2018 Aug 31. PMID: 30173871.
- Chang TP, Gold DR, Otero-Millan J, Huang BR, Zee DS. Pendular Oscillation and Ocular Bobbing After Pontine Hemorrhage. Cerebellum. 2021 Oct;20(5):734-743. doi: 10.1007/s12311-019-01086-6. PMID: 31883062.
