Benign Essential Blepharospasm Resulting in Blindness

Progressive, uncontrolled blinking and eyelid spasms had begun 3 years ago and now rendered a 77-year-old man functionally blind. Various treatments, including oral and topical medications and acupuncture, had been unsuccessful.

Dr Leonid Skorin, Jr, of Dixon, Ill, diagnosed the problem as benign essential blepharospasm, a form of focal dystonia. Its characteristics consist of involuntary spasms and contractions of the orbicular muscle of the eye and the upper facial muscles, such as the procerus and corrugator supercilii musculature. These spontaneous, bilateral, and progressive spasms occur almost exclusively in older patients; the mean age at onset is 56.

The disease begins with fluctuating episodes of increased blinking that last from seconds to minutes. As the blinking progresses, involuntary spasms of eyelid closure lead to periods of functional blindness, as occurred in this patient. Blepharospasm can also involve periodic lower facial movement, in which case the disorder is known as Meige's or Brueghel's syndrome.

Management has included everything from hypnosis, acupuncture, and biofeedback to relaxation therapy, none of which has been effective. Medical therapy, such as anticholinergic agents, muscle relaxants, and anticonvulsants, has its limitations. The best treatment for benign essential blepharospasm is botulinum A toxin injection, which helps more than 90% of affected persons. It lasts for an average of 3 to 4 months and may be repeated when symptoms recur. In recalcitrant cases, myectomy is most effective.