Dr. Jankovic, Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine, received research and training funding from Allergan, F Hoffmann-La Roche, Medtronic Neuromodulation, Merz, Neurocrine Biosciences, Nuvelution, Revance, and Teva and consulting/advisory board honorariums from Abide, Lundbeck, Retrophin, Parexel, Teva, and Allergan.)
This article includes discussion of blepharospasm, cranial dystonia, and eyelid dystonia. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.
Blepharospasm is a form of focal dystonia manifested by involuntary eye closure due to excessive contractions of the eyelids. In addition, contractions of orbicularis oculi (pretarsal, preseptal, and periorbital portions) adjacent muscles, including procerus and corrugator, as well as paranasal and other facial muscles may be involved. Blepharospasm is often misdiagnosed as “dry eyes” or “nervousness.” The author of this article reviews the clinical features, pathogenesis, and treatment of blepharospasm, focusing on the use of botulinum toxin.
• Blepharospasm is a neurologic disorder classified as a focal dystonia.
• In addition to involuntary contractions of the orbicularis oculi resulting in eye closure, most patients with blepharospasm also develop contractions of other facial muscles, jaw muscles (oromandibular dystonia), and many also have associated contractions of neck muscles causing abnormal head posture or tremor (cervical dystonia).
• Botulinum toxin injection is the treatment of choice for blepharospasm and cranial-cervical dystonia.
Historical note and terminology
Involuntary facial movements have been recognized for a long time and were depicted by artists who were fascinated by how these movements distorted the facial expression. For example, the 16th century Flemish artist Brueghel painted a woman with apparent blepharospasm and involuntary jaw opening (Jankovic 1988). Although the eponym "Meige syndrome" sometimes has been used to designate idiopathic cranial-cervical dystonia (Pandey and Sharma 2017), this term is not appropriate because Talkow in Germany and Wood in the United States described blepharospasm and orofacial dystonia several decades before the 1910 publication by the French neurologist's report. It was not until the 1970s that blepharospasm was recognized as a form of focal dystonia (Jankovic 1988; Defazio and Livrea 2004; Albanese et al 2013; Defazio 2017).
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