Blepharospasm

Right Animated image of BFS in the upper eyelid of a 19-year-old male. Symptoms subsided several days later.
Classification and external resources
Pronunciation	/ˌblɛfəroʊˈspæzəm/
Specialty	neurology
ICD-10	G24.5
ICD-9-CM	333.81
OMIM	606798
DiseasesDB	15748
MedlinePlus	000756
eMedicine	oph/202
Patient UK
MeSH	D001764

Blepharospasm is any abnormal contraction or twitch of the eyelid. In most cases, symptoms last for a few days then disappear without treatment, but sometimes the twitching is chronic and persistent, causing lifelong challenges. In those rare cases, the symptoms are often severe enough to result in functional blindness. The person's eyelids feel like they are clamping shut and will not open without great effort. People have normal eyes, but for periods of time are effectively blind due to their inability to open their eyelids. In contrast, the reflex blepharospasm is due to any pain in and around the eye.

It is of two types: essential and reflex blepharospasm. The benign essential blepharospasm is a focal dystonia—a neurological movement disorder involving involuntary and sustained contractions of the muscles around the eyes. The term essential indicates that the cause is unknown, but fatigue, stress, or an irritant are possible contributing factors.

Although there is no cure botulinum toxin injections may help temporarily.^[1][2] A surgical procedure known as myectomy may also be useful.^[1] It is a fairly rare disease, affecting only one in every 20,000 people in the United States. The word is from Greek: βλέφαρον / blepharon, eyelid, and σπασμός / spasmos, spasm, an uncontrolled muscle contraction.

Symptoms

• Excessive blinking and spasming of the eyes, usually characterized by uncontrollable eyelid closure of durations longer than the typical blink reflex, sometimes lasting minutes or even hours
• Uncontrollable contractions or twitches of the eye muscles and surrounding facial area. Some sufferers have twitching symptoms that radiate into the nose, face and sometimes, the neck area
• Dryness of the eyes
• Sensitivity to the sun and bright light^[3]

Causes

Some causes of blepharospasm have been identified; however, the causes of many cases of blepharospasm remain unknown, although some educated guesses are being made. Some blepharospasm patients have a history of dry eyes and/or light sensitivity, but others report no previous eye problems before onset of initial symptoms.

Some drugs can induce blepharospasm, such as those used to treat Parkinson's disease, as well as sensitivity to hormone treatments, including estrogen-replacement therapy for women going through menopause. Blepharospasm can also be a symptom of acute withdrawal from benzodiazepines. In addition to blepharospasm being a benzodiazepine withdrawal symptom, prolonged use of benzodiazepines can induce blepharospasm and is a known risk factor for the development of blepharospasm.^[4]

Blepharospasm may also come from abnormal functioning of the brain basal ganglia. 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.

Multiple sclerosis can cause blepharospasm.^[5]

Treatment

• Drug 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. 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.^[6]
• Botulinum toxin injections (Botox and Dysport are widely known examples) have been used to induce localized, partial paralysis. Among most sufferers, botolinum toxin injection is the preferred treatment method.^[7] 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.
• Surgery: 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.^[8]

• Neuroplasticity based reeducation: 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.^{[9][10][11][12][13][14][15]}

See also

• Levator palpebrae superioris muscle
• Myokymia
• Fasciculation

References

1. "Facts About Blepharospasm". August 2009. Retrieved 19 March 2015.
2. 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. doi:10.1212/WNL.0000000000002560.
3. 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.
4. Wakakura M, Tsubouchi T, Inouye J (March 2004). "Etizolam and benzodiazepine induced blepharospasm". Journal of Neurology, Neurosurgery, and Psychiatry. 75 (3): 506–7. doi:10.1136/jnnp.2003.019869. PMC 1738986. PMID 14966178.
5. Goldman, Lee. Goldman's Cecil Medicine (24th ed.). Philadelphia: Elsevier Saunders. p. 2429. ISBN 1437727883.
6. Piyasena INAP; Jayasinghe JAC (March 2014). "Mosapride (5HT4 agonist) in the treatment of blepharospasm.". Ceylon Med J. 59 (1): 26–7. doi:10.4038/cmj.v59i1.5527. PMID 24682196.
7. 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): 23–6. doi:10.1590/S0004-27492006000100005. PMID 16491229.
8. Anderson RL, Patel BC, Holds JB, Jordan DR (September 1998). "Blepharospasm: past, present, and future". Ophthalmic Plastic and Reconstructive Surgery. 14 (5): 305–17. doi:10.1097/00002341-199809000-00002. PMID 9783280.
9. TEDx Talk. Federico Bitti. Cervical Dystonia. Rewiring the brain through dance. https://www.youtube.com/watch?v=DwkHK3rfKO0
10. TEDx Talk . Joaquin Farias. Dystonia. Your movement can heal your brain. https://www.youtube.com/watch?v=czW-xBvDtHY
11. Glove and Mail. Choosing music over meds, one man's quest to retrain his brain to overcome dystonia. https://www.youtube.com/watch?v=IpcXkV_ex8Y
12. Farias J. Limitless. How your movements can heal your brain. An essay on the neurodynamics of dystonia. Galene editions 2016
13. Farias J. Intertwined. How to induce neuroplasticity. A new approach to rehabilitate dystonias. Galene editions 2012.
14. 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
15. Open Your Eyes. Freedom from Blepharospasm. Documentary. Out of the Box Productions. https://www.youtube.com/watch?v=BlWXcsjyswY

External links

• Blepharospasm – Resource Guide from the National Eye Institute (NEI)
• BEBRF: Benign Essential Blepharospasm Research Foundation – Information about blepharospasm
• Blepharospasm Definition – Dystonia Medical research Foundation