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Joel Guss, Andrew Blitzer, Joanna B. D’Elia,
Jean S. Verheyden, and Boris L. Bentsianov

Blepharospasm is a dystonia of the facial musculature that ranges in severity from an increased blink rate to disabling contractions with pain and visual dysfunction. As with all dystonias, the disorder involves involuntary sustained muscle contractions. Not long ago, dystonias were thought to be manifestations of psychiatric disease. Today, they are understood to be complex neurologic disorders with abnormalities in sensory input, central processing, and motor output, interacting to produce the movement disorder.1 The primary muscle involved is the orbicularis oculi. Other protractors of the eyelids include the corrugator superciliaris and procerus muscles. Spasms are usually bilateral but may affect one side primarily. Blepharospasm most commonly presents as a focal dystonia often referred to as benign essential blepharospasm. Meige’s syndrome or segmental cranial dystonia is the association of blepharospasm with oromandibular and sometimes cervical or laryngeal dystonia.²

Blepharospasm is uncommon, with an estimated prevalence of up to one case per 10,000 people. There is an increased female to male prevalence. Mean age of diagnosis is in the sixth decade of life.³ The condition usually begins with increased blink rate or spasms of the eyelids, forehead, or midfacial muscles. Patients often complain of eye irritation, pain, photophobia, or abnormal tearing, and may initially be diagnosed with various other ocular disorders. Spasms may be triggered by sensory stimulation such as wind, air pollutants, or bright light, and may be worse in stressful situations. Some patients identify sensory tricks such as touching the face, using artificial tears, or talking and singing that may temporarily abate the spasm. The condition is often progressive and results in functional blindness in a significant minority of those affected.4 Patients may avoid social settings, reading, driving, or watching television, with ensuing anxiety and depression. Maximum disability is reached at an average of 3 years after onset. Although severity may fluctuate, spontaneous remissions are rare. Over the long term, the persistent contractions can result in weakening of the eyelid’s fascial attachments, resulting in brow ptosis, dermatochalasis, and ectropion, with further visual field obstruction. The disease typically remains focal but may involve other facial muscles or, rarely, other parts of the body.

Management begins with treatment of any underlying eye disorder such as blepharitis, conjunctivitis, or corneal irritation, and with lid hygiene and lubrication. Tinted sunglasses with ultraviolet blocking have demonstrated efficacy as well.⁵ Some patients resort to “eyelid crutches” or springs on glasses to hold lid open. Systemic medications such as benzodiazepines and the antiparkinsonian agent Artane have limited efficacy and significant adverse effects.² Surgery is reserved for patients who fail more conservative treatments. Neurectomy of facial nerve branches has generally been abandoned in favor of myectomy of the eyelid protractors.

Botulinum neurotoxin (BoNT) injection has emerged as a treatment of choice for blepharospasm, and it is approved by the United States Food and Drug Administration (FDA) for this application. Many studies ranging from large open label to small double-blind placebo-controlled have demonstrated BoNT injection to be both effective and safe, with success rates exceeding 90%.^6–10 The American Academy of Neurology Therapeutics and Technology Assessment Subcommittee rates the strength of evidence behind the use of BoNT for blepharospasm as level B, meaning it is “probably effective.”¹¹ A higher level recommendation requires at least two large double-blind placebo-controlled studies with rigorous requirements. Unfortunately, no such studies have been performed.¹² Long-term efficacy and safety have been demonstrated as well.^9,13,14

Workup

Diagnosis of blepharospasm is clinical, though it may initially be challenging as there is overlap between early symptoms of blepharospasm and other eye disorders. A history of other neurologic symptoms, use of psychotropic medications, and family history of dystonia should be elicited. All patients benefit from an ophthalmologic examination. Further studies and imaging should be obtained to rule out a central nervous pathology when appropriate.

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