Benign Essential Blepharospasm

Author: Robert H Graham, MD; Chief Editor: Hampton Roy Sr, MD

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.
Epidemiology
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]