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Blepharospasm, Benign Essential

Author: Robert H Graham, MD, Senior Associate Consultant, Department of Ophthalmology, Mayo Clinic, Scottsdale, Arizona
Contributor Information and Disclosures

Updated: Jun 8, 2009

Introduction

Background

The first record of blepharospasm and lower facial spasm was found in the 16th century in a painting titled De Gaper. At that time, and for several ensuing centuries, patients with such spasms were regarded as being mentally unstable and often were institutionalized in insane asylums. Little progress was made in the diagnosis or treatment of blepharospasm until the early 20th century, when Henry Meige (pronounced "mehzh"), a French neurologist, described a patient with eyelid and midface spasms, spasm facial median, a disorder now known as Meige syndrome.1 At about the same time, the first medical treatments became available, including alcohol injections into the facial nerve, facial nerve avulsion, neurotomy, and neurectomy. The adverse effects of these treatments, including loss of facial expression and movements, functional and cosmetic deformities of ptosis, and eyelid malposition, were often as bad as the disease.

Pathophysiology

Modern physicians realize that blepharospasm is a neuropathologic disorder, rather than psychopathologic, as was once believed. The cause of blepharospasm is multifactorial. Although it is likely that a central control center for coordination and regulation of blink activity exists, somewhere in the basal ganglia, midbrain, and/or brain stem, it is unlikely that a single defect in this elusive control center is the primary cause of this disease.2,3,4,5,6,7,8,9

Today, most view blepharospasm as a defect in circuit activity, rather than a defect at a specific locus. If the central control center fails to regulate blinking in blepharospasm, it is believed to be only one component of an overloaded, defective circuit. This circuit forms a blepharospasm vicious cycle, which has a sensory limb, a central control center located in the midbrain, and a motor limb. The sensory limb responds to multifactorial stimuli, including light, corneal or eyelid irritation, pain, emotion, stress, or various other trigeminal stimulants. These stimuli are transmitted to the central control center, which may be genetically predisposed or weakened by injury or age. This abnormal central control center fails to regulate the positive feedback circuit. The motor pathway is composed of the facial nucleus, facial nerve, and orbicularis oculi, corrugator, and procerus muscles. Other facial muscles also may be involved.

Frequency

United States

It is estimated that there are at least 50,000 cases of blepharospasm in the United States, with up to 2000 new cases diagnosed annually. The prevalence of blepharospasm in the general population is approximately 5 in 100,000.

Mortality/Morbidity

At one end of the clinical spectrum, essential blepharospasm is manifested by simple increased blink rate and intermittent eyelid spasms, while at the other end of the spectrum, blepharospasm is a disabling condition with ocular pain and functional blindness. Patients may report that they are disabled to the point where they have stopped watching television, reading, driving, and/or walking. Patients may develop anxiety, avoid social contact, become depressed, become occupationally disabled, and become suicidal.10,11,12

Sex

A female preponderance of 1.8:1 exists.

Age

The mean age of onset of blepharospasm is 56 years, and two thirds of patients are age 60 years or older.13

Clinical

History

At onset, there is increased frequency of blinking, particularly in response to a variety of common stimuli, including wind, air pollution, sunlight, noise, movements of the head or eyes, and in response to stress or the environment.14,15,16,17,18 Patients may complain of photophobia and ocular surface discomfort, and especially of dry eye symptoms.19 These symptoms progress over a variable period to include involuntary unilateral spasms, which later become bilateral.20

Patients may report that they are disabled to the point where they have stopped watching television, reading, driving, and/or walking. A family history positive for dystonia or blepharospasm further aids in the diagnosis.21

Blepharospasm commonly is associated with dystonic movements of other facial muscles. Anatomic changes associated with long-standing blepharospasm include eyelid and brow ptosis, dermatochalasis, entropion, and canthal tendon abnormalities.

  • The early symptoms of blepharospasm include increased blink rate (77%), eyelid spasms (66%), eye irritation (55%), midfacial or lower facial spasm (59%), brow spasm (24%), and eyelid tic (22%).
  • Symptoms commonly preceding diagnosis include tearing, eye irritation, photophobia, and vague ocular pain. While these complaints are common in the average ophthalmology practice, awareness of this disorder and proper suspicion may aid in early diagnosis.
  • Conditions relieving blepharospasm included sleep (75%), relaxation (55%), inferior gaze (27%), artificial tears (24%), traction on eyelids (22%), talking (22%), singing (20%), and humming (19%).
  • Comorbid diagnoses include dry eyes (49%) and other neurologic disease (8%).22,23

Physical

In normal blinking, eyelid closure is the result of activity and co-inhibition of 2 groups of muscles, the protractors of the eyelids (ie, orbicularis oculi, corrugator superciliaris, procerus muscles) and the voluntary retractors of the eyelids (ie, levator palpebrae superioris, frontalis muscles). During the normal blink, the protractors and retractors have co-inhibition and function only at separate times. In patients with blepharospasm, this inhibition between the protractors and retractors is lost.

Causes

A specific etiology for blepharospasm has yet to be identified. Some patients with blepharospasm report a familial occurrence of the affliction. In families with autosomal dominant familial dystonia, affected members may have a generalized or segmental dystonia, while other members have various focal dystonias, such as isolated blepharospasm.

More on Blepharospasm, Benign Essential

Overview: Blepharospasm, Benign Essential
Differential Diagnoses & Workup: Blepharospasm, Benign Essential
Treatment & Medication: Blepharospasm, Benign Essential
Follow-up: Blepharospasm, Benign Essential
References
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Further Reading

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Keywords

benign essential blepharospasm, BEB, essential blepharospasm

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Contributor Information and Disclosures

Author

Robert H Graham, MD, Senior Associate Consultant, Department of Ophthalmology, Mayo Clinic, Scottsdale, Arizona
Robert H Graham, MD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, and Arizona Ophthalmological Society
Disclosure: WebMD/eMedicine Salary Employment

Medical Editor

Ron W Pelton, MD, PhD, Private Practice, Colorado Springs, Colorado
Ron W Pelton, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Colorado Medical Society, Utah Medical Association, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Mark T Duffy, MD, PhD, Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Medical Director, Advanced Cosmetic Solutions, A BayCare Clinic
Mark T Duffy, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Sigma Xi, and Society for Neuroscience
Disclosure: Allergan - Botox Cosmetic Consulting fee Consulting; Quest medical - lacrimal balloons Honoraria Speaking and teaching; Ortho-Neutrogenia Consulting fee Consulting

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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