ESSENTIAL BLEPHAROSPASM AND HEMIFACIAL SPASM
Benign essential blepharospasm is the uncontrolled contraction of muscles around the eyes. This condition affects both sides and may result in a variety of problems including difficulty opening the eyes, rapid-uncontrolled fluttering of the eyelids, or forced contraction of the eyelids and brows. When the mouth and neck are involved with the spasms, the condition is called Meige's syndrome. In its early stage, blepharospasm may present with excessive blinking which progresses to more forceful and frequent muscle contraction. The spasms disappear during sleep and may be made worse with bright lights, fatigue, or emotional stress.
Hemifacial spasm is the uncontrolled contraction of muscles on one side of the face, usually including the eyelids. The initial symptoms may be twitching of the eyelids which progresses to involve the muscles on one entire side of the face. The severity of symptoms may vary from mild fluttering to forceful contraction. Unlike blepharospasm, this condition persists during sleep.
Although the cause of blepharospasm is unknown, its manifestations arise from irritation of the facial nerve at the base of the skull. Uncommonly, this irritation may be the result of an abnormal blood vessel pulsating against the facial nerve. When the facial nerve is irritated, it causes the facial muscles to involuntary contract and go into spasm. Fewer than 1% of cases are caused by tumor-induced facial nerve irritation. This may require and MRI to document this uncommon cause. Otherwise, the diagnosis is made during clinical examination when the extent, severity, and frequency of the involuntary squeezing can be assessed. Blepharospasm, hemifacial spasm, and Meige's syndrome ordinarily require no further diagnostic testing.
Oral medications have no significant role in the management of blepharospasm as they provide negligible benefit.
The principal treatment for this condition is with botulinum toxin (Botox) injection. Botulinum toxin is FDA approved for the treatment of these disorders. The denatured toxin is injected directly into the muscles involved in the spasm to stop the unwanted contraction. The site of injection and the dose are predicated on the patient's individual presentation. In blepharospasm, there are most typically 7 injection sites on each side extending from the brow to the lower eyelid. The effects of botulinum toxin last an average of three to four months, at which time the injection is repeated - gaining the same beneficial effect. It is very uncommon for a patient to become unresponsive (refractory) to repeat Botox injections. Side effects of botulinum injection are quite uncommon and always temporary. They can include drooping upper eyelids or double vision.
In very rare cases, surgery may be considered for blepharospasm if botulinum toxin therapy is not completely successful. Protractor myectomy surgery selectively removes the eyelid muscles responsible for the involuntary eyelid closure. Some patients may require additional Botox injections following such surgery. In the very rare cases of hemifacial spasm caused by the pressure effect of an aberrant muscle or tumor, microvascular decompression or tumor excision near the brainstem can cure the symptoms.
Botox injection is a safe and very effective treatment of essential blepharospasm, hemifacial spasm, and Meige's syndrome. Although repeated treatment is usually required, it has been the definitive treatment for these conditions for over 20 years. Dr. Kohn served as one of the 50 original scientific investigators in the world whose work and the results of their over 10,000 cases led to the initial Food and Drug Administration (FDA) approval of Botox in 1985.
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