Abstract

Benign essential blepharospasm and hemifacial spasm are chronic and disabling medical conditions. Both disorders can result in uncontrollable blinking or frank spasms of the eyelids and face, which may interfere with the activities of daily living and may even render a patient functionally blind and occupationally handicapped. Often, when untreated, the eyelid and facial spasms are so emotionally unsettling that the patients become withdrawn, frustrated, and desperate. Essential blepharospasm is the most common manifestation of orofacial movement disorders. The term is used to describe a movement disorder limited to the eyelid protractors without a secondary inciting cause, and is characterized by spontaneous, repetitive, forceful eyelid closure. Benign essential blepharospasm is caused by forceful contraction of the eyelid protractors, which include the orbicularis oculi, corrugator supercilii, and procerus muscles. The prevalence is 32 in 100,000 and women are more commonly affected than men (3:2). The peak onset is in the fifth to sixth decades, and symptoms peak 3 years after onset. When contractions are limited to the orbital and periorbital muscles, the term “benign essential blepharospasm” is used. Often, subsequent contractions of lower face and neck occur concurrently. This is termed Meige syndrome, orofacial dystonia, or oromandibular dystonia (Brueghel syndrome). Furthermore, dystonia outside of the facial nerve distribution is called segmental cranial dystonia or craniocervical dystonia. Initially, benign essential blepharospasm can manifest as increased frequency in blinking in response to several stimuli, including wind, air pollution, sunlight, noise, and movements of the head or eyes. It can significantly impair quality of life by causing difficulty in reading, writing, and driving. In severe cases, patients may be functionally blind. Several eyelid changes have been noted with longstanding blepharospasm including dermatochalasis, eyelid and brow ptosis, entropion, and canthal tendon abnormalities. Common symptoms that precede the official diagnosis of benign essential blepharospasm include eye irritation, photophobia, tearing, and ocular pain. Early symptoms include an increase in blink rate (77%), lid spasms (66%), ocular irritation (55%), midfacial or lower facial spasm (59%), brow spasm (24%), and eyelid tic (22%). Several conditions relieve blepharospasm; these include sleep, relaxation, inferior gaze, artificial tears, traction on eyelids, and humming.

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