History

At onset, blepharospasm is characterized by increased frequency of blinking, particularly in response to various common stimuli, including wind, air pollution, sunlight, noise, movements of the head or eyes, and in response to stress or the environment.^{[20, 21, 22, 23, 24]}Patients may complain of photophobia and ocular surface discomfort, and especially of dry eye symptoms.^[25]These symptoms progress over a variable period to include involuntary unilateral spasms, which later become bilateral.^[26]

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.^[27]

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%).^{[28, 29]}

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.

A study by DeFazio et al examined the first-degree relatives of 122 patients with primary blepharospasm. The study investigated genetic and environmental connections regarding the disorder and found no major difference between familial and sporadic cases with regard to coffee drinking and existing eye diseases. These findings suggest that sporadic and familial blepharospasm most likely has a commonality in etiologic background and influences of environmental factors.^[30]

Wabbels has reported a single case of congenital blepharospasm.^[31]

Zhou et al used functional MRI (fMRI) to identify abnormal neurological pathways in 9 patients with benign essential blepharospasm compared with controls.^[32]

Low serum calcium has been associated with blepharospasm, and disease severity is negatively correlated with serum vitamin D levels.^[33]

Complications

Complications of benign essential blepharospasm may include the following:

Ptosis following botulinum toxin injection
Palsy after seventh denervation
Scarring and edema after myectomy

Differential Diagnoses

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