Abstract
Introduction: Benign essential blepharospasm (BEB) is an adult-onset focal dystonia characterized by involuntary spasms of the orbicularis oculi (OO) muscle. BEB typically develops in the fifth to sixth decade, has a variable female preponderance, and a tendency to spread to adjacent body regions. Idiopathic BEB is thought to be a multifactorial disorder resulting from the contribution of both environmental and genetic factors. To date, BEB treatment is largely based on botulinum A toxin (BTX).
Areas covered: This article summarizes current knowledge on the therapeutic approaches that can be proposed to BEB patients who are truly unresponsive to BTX. Current and investigated alternatives to BTX can be included in the following categories: i) alternative therapeutic weakening of the OO muscle; ii) treatments of ophtalmological complaints; and iii) therapeutic interventions on central nervous system mechanisms underlying BEB.
Expert opinion: Therapeutic strategies to manage BEB of patients who are truly unresponsive to BTX are very limited. Currently available treatments include myectomy, oral medication and deep brain stimulation. Experimental therapeutics include topical acetyl hexapeptide-8, a promising new drug for extending the duration of action of BTX, and transcranial magnetic stimulation. At present, the choice of the best treatment strategy, including medical, surgical and non-invasive treatments remains largely empirical and depending on existing reports of toxicity rather than efficacy.
Declaration of interest
The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.
Notes
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