Oral Pharmacotherapy for Benign Essential Blepharospasm

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Benign essential blepharospasm (BEB) is a focal dystonia characterized by bilateral involuntary synchronous spasms of the eyelid protractors, which may be brief or sustained. It has an estimated prevalence of 4 per 100,000, affects women approximately three times more commonly than men, and generally presents in the fifth to seventh decade of life.1–3 Clinical presentation varies from a mildly increased blink rate to forceful and sustained contraction of the periocular muscles resulting in functional blindness. The uncontrollable nature of the spasms can be anxiety provoking and socially disabling. It may also affect contiguous areas of the mid and lower face and/or neck, and in these instances, is termed a segmental cranial dystonia.
The disease course is generally progressive, especially during the initial years, with few patients ever achieving remission.2,4,5 Mainstay treatment options can be divided into three areas including management of sensory input, motor output, and central control. Although established treatment modalities have been identified and developed for the former two, central control of the disease has been more elusive for clinicians. Because the neuronal pathways of blinking have yet to be fully elucidated, drug therapy has proven to be challenging. Without a clear pharmacologic target, a variety of medications from a wide range of drug classes have been tried with varying success and associated side effect profiles. Treatment response to oral medication is typically characterized as partial, if any, resulting in individuals being tried on a variety of drugs.
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