The relationship between eye-winking tics, frequent eye-blinking and blepharospasm.
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A family is reported in which three generations were affected with eye-winking tics and/or blepharospasm. The proband developed eye-winking tics in childhood and then developed excessive blinking progressing to blepharospasm by the age of 21 years. His mother presented with Meige's syndrome and spasmodic torticollis at the age of 59 years; his uncle had blinked excessively from his early forties. His eldest son developed an eye-winking tic with facial grimacing at the age of 8 years, and in another son, a self-limiting period of eye-blinking occurred at the age of 4 years. The recovery cycle of the blink reflex was abnormal in all three generations. Three other children with eye-winking tics have a parent or close relative with frequent eye-blinking or blepharospasm. Five patients with adult-onset blepharospasm or Meige's syndrome are also described who had excessive eye-blinking dating back to childhood. It is suggested that eye-winking tics, frequent blinking and blepharospasm may share common pathophysiological mechanisms; the clinical expression may be age-related. (+info)
Cranial dystonia, blepharospasm and hemifacial spasm: clinical features and treatment, including the use of botulinum toxin.
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Blepharospasm, the most frequent feature of cranial dystonia, and hemifacial spasm are two involuntary movement disorders that affect facial muscles. The cause of blepharospasm and other forms of cranial dystonia is not known. Hemifacial spasm is usually due to compression of the seventh cranial nerve at its exit from the brain stem. Cranial dystonia may result in severe disability. Hemifacial spasm tends to be much less disabling but may cause considerable distress and embarrassment. Patients affected with these disorders are often mistakenly considered to have psychiatric problems. Although the two disorders are quite distinct pathophysiologically, therapy with botulinum toxin has proven very effective in both. We review the clinical features, proposed pathophysiologic features, differential diagnosis and treatment, including the use of botulinum toxin, of cranial dystonia and hemifacial spasm. (+info)
Doxorubicin chemomyectomy: injection of monkey orbicularis oculi results in selective muscle injury.
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Doxorubicin was injected into the preseptal portion of the orbicularis oculi of one lower eyelid in each of two cynomolgus monkeys at a dose of 2 mg. One monkey was observed for 4 days and the other for 68 days after doxorubicin injection. Although some skin ulceration was seen, it was completely healed by 3 weeks postinjection. The preseptal portion of the orbicularis oculi in both monkeys showed extensive signs of injury. At 4 days after doxorubicin injection many necrotic muscle fibers could be seen. Very few muscle fibers in the preseptal portion of the muscle remained by 68 days after injection. The pretarsal portion of the injected orbicularis oculi was relatively normal, with little evidence of injury. It appears that doxorubicin injection into the lower lid resulted in a gradient of muscle injury, with increasing severity at decreasing distances from the injection site. The extent of muscle injured is related to the dose and injection site and indicates the ability to selectively control the injury within a muscle. The ramifications of this effect on the use of doxorubicin for permanent treatment of blepharospasm and other related diseases are discussed in this first report of the injection of this drug into the eyelids of nonhuman primates. (+info)
Blepharospasm: a review of 264 patients.
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The natural history and response to different treatments have been evaluated in 264 patients with blepharospasm. The mean age of onset was 55.8 years and there was a female preponderance of 1.8 to 1. Dystonia elsewhere was found in 78% of patients, usually in the cranial-cervical region, and appeared to follow a somatotopic progression. A family history of blepharospasm or dystonia elsewhere was found in 9.5% of cases, which suggests a genetic predisposition. Ocular lesions preceded the onset of blepharospasm in 12.1% of cases. The response to drugs was inconsistent, although initial improvement was experienced by one fifth of patients treated with anticholinergics. Twenty-nine bilateral facial nerve avulsion operations were performed with benefit in 27 cases; but recurrences appeared in 22, on average one year after surgery. Botulinum toxin injections were performed in 151 patients. Significant improvement was achieved in 118 cases. Mean duration of benefit was 9.2 weeks. Transient ptosis and diplopia were the commonest side effects. (+info)
Long-term results of treatment of idiopathic blepharospasm with botulinum toxin injections.
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One hundred and one patients with idiopathic blepharospasm have been treated with injections of botulinum toxin A into the orbicularis oculi. Ten had previously had facial nerve avulsions and responded well, normal visual function being restored in the majority (7/10) for an average of 14 weeks. Without prior surgical treatment the response was more variable, but 71/91 regained normal or near normal vision. Older patients, those with a family history of the condition, and those without oromandibular dystonia responded slightly better. The severity of the blepharospasm, the length of the history, and spontaneous resolution of an episode of focal dystonia in the past had no influence on the outcome. Results were poor in the presence of an associated neurological disorder. Side effects, particularly a temporary partial ptosis, were common but were well tolerated. The average duration of improvement was eight weeks in men, nine in women, and there was no evidence of any increase in duration after multiple injections. Eighty nine patients continued with injections, 11 opted for surgical treatment, and one resumed drugs. (+info)
Management of blepharospasm.
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From 1950 through 1984, 123 patients underwent surgical treatment of blepharospasm at the Mayo Clinic. During this period, four different operations (proximal and distal neurectomy and two types of myectomy) were used. Significant recurrent or residual blepharospasm was observed more frequently (P less than 0.01), and need for subsequent operations was greater (P less than 0.01), among patients who had undergone distal neurectomy than among those who had had myectomy. These data support the view that myectomy is a more effective procedure than neurectomy. Initial results with botulinum toxin injection seem to indicate that it is an effective short-term treatment for blepharospasm. However, its long-term efficacy and safety need further study, as does the role it should play in combination with myectomy. (+info)