Major ReviewReview and Update of Involuntary Facial Movement Disorders Presenting in the Ophthalmological Setting
Section snippets
Benign Essential Blepharospasm (BEB)
Benign essential blepharospasm (BEB) is a disorder of progressive involuntary spasms of the eyelid protractors (orbicularis oculi, corrugator, and procerus muscles) resulting in eyelid closure. Blepharospasm was first reported in 1870 by the German physician Talkow.158 In 1887, Wood characterized facial and oromandibular dystonias,172 and in 1910, the French neurologist Meige in a series of patients with blepharospasm described one having mid-facial and jaw spasms.9, 97, 108
Apraxia of Eyelid Opening
Apraxia of eyelid opening (AEO) is a non-paralytic motor abnormality in which patients have difficulty opening their eyelids voluntarily. AEO was first described by Goldstein and Cogan in 196566 and is associated with extra-pyramidal disorders, in particular progressive supranuclear palsy.17, 49, 95 It is important to distinguish AEO from motor impersistence of lid opening, seen in right hemisphere infarction. AEO associated with blepharospasm may be termed pseudo-AEO; this is not a true
Hemifacial Spasm
Hemifacial spasm (HFS) is characterized by involuntary, irregular, tonic, and clonic synchronous contraction of the muscles innervated by the ipsilateral facial nerve. The spasms may occur during sleep.
Aberrant Facial Nerve Regeneration
Aberrant facial nerve regeneration (AFR), a recognized complication of peripheral facial nerve palsy, is believed to arise when regenerating nerve fibers from facial subnucleus motoneurons are misdirected to other facial muscles. Pathophysiological explanations of AFR include ephaptic transmission and reorganization within the facial motor nucleus.114, 120, 121, 159 Using retrograde fluorescent tracer techniques in animal models, Choi and Raisman established that after facial nerve damage,
Evaluating Patients with Focal Dystonia
Various disability scales have been designed to assess patients with BEB. Jones et al and Roggenkämper et al's subjective overall scoring systems are not reproducible because of a lack of published details.91, 136 Multi-item disability indexes are useful in ascertaining the effect of treatments such as botulinum toxin on driving, reading and watching television. The Jankovic rating scale assesses the severity and frequency of spasms using a five-point scoring system.88 The Blepharospasm
Treatment
Before medical treatment is begun, it is important to offer patients and their relatives advice and support. Family and friends play a vital role and should not be ignored. Practitioners should identify an individual's coping skills and ensure they are aware of support groups such as the Benign Essential Blepharospasm Research Foundation (BEBRF) and the Dystonia Society in the United Kingdom. Anderson et al showed that 90% of patients felt that the BEBRF had provided them with considerable
Conclusion
Facial movement disorders remain a fascinating subject. When evaluating patients, the use of disability scales can be helpful and it is important to provide information about available support groups. The etiology of BEB requires further study including research into associated gene loci. There is a wide spectrum of treatment modalities available for these various disorders, including modification of afferent sensory inputs. The finding of improvement in eyelid and lower facial spasms with the
Method of Literature Search
The authors performed Medline searches with Pubmed, including articles from 1970 to 2009. A few select articles published before 1970 are included for historical purposes. Searches included various combinations of the terms blepharospasm, botulinum toxin, facial palsy, apraxia of eyelid opening, hemifacial spasm, aberrant facial nerve regeneration, disability scales, FL-41 tints, pressure point devices, Bangerter occlusion foils and sensory tricks. The search was restricted to publications in
Disclosure
The authors reported no proprietary or commercial interest in any product mentioned or concept discussed in this article.
References (175)
- et al.
Absence of antibody production in patients treated with botulinum A toxin
Am J Ophthalmol
(1986) - et al.
FL-41 tint improves blink frequency, light sensitivity, and functional limitations in patients with benign essential blepharospasm
Ophthalmology
(2009) Genetics of dystonia: an overview
Parkinsonism Relat Disord
(2007)Surgical correction of intractable blepharospasm. Technical improvements
Am J Ophthalmol
(1965)Botulinum-A toxin in the treatment of craniocervical muscle spasms: short- and long-term, local and systemic effects
Surv Ophthalmol
(1996)- et al.
The ‘geste antagonistique’ induces transient modulation of the blink reflex in human patients with blepharospasm
Neurosci Lett
(1998) Negative antibody response to long-term treatment of facial spasm with botulinum toxin
Am J Ophthalmol
(1988)- et al.
Production, purification and toxoiding of Clostridium botulinum type A toxin
- et al.
Modulation of sensory photophobia in essential blepharospasm with chromatic lenses
Ophthalmology
(2005) - et al.
Improvement of apraxia of eyelid opening by wearing goggles
Lancet
(2000)
Myectomy for essential blepharospasm
Mayo Clin Proc
Meige syndrome: what's in a name?
Parkinsonism Relat Disord
Supramaximal doses of botulinum toxin for refractory blepharospasm
Ophthalmology
The evaluation of light sensitivity in benign essential blepharospasm
Am J Ophthalmol
Pretarsal injections of botulinum toxin improve blepharospasm in previously unresponsive patients
J Neurol Neurosurg Psychiatry
Carbamazepine for hemifacial spasm
Neurology
Blepharospasm: past, present, and future
Ophthal Plast Reconstr Surg
Long-distance retrograde effects of botulinum neurotoxin A
J Neurosci
Characterization of the inhibitory action of botulinum neurotoxin type A on the release of several transmitters from rat cerebrocortical synaptosomes
J Neurochem
Hemifacial spasm in Rochester and Olmsted County, Minnesota, 1960 to 1984
Arch Neurol
Maladaptive neural compensatory mechanisms in Bell's palsy–induced blepharospasm
Neurology
Benign essential blepharospasm
Int Ophthalmol Clin
Pathophysiology of blepharospasm and oromandibular dystonia
Brain
Magnetic resonance tomographic angiography in the investigation of hemifacial spasm
Neuroradiology
Botulinum neurotoxin A selectively cleaves the synaptic protein SNAP-25
Nature
Botulinum neurotoxin C1 blocks neurotransmitter release by means of cleaving HPC–1/syntaxin
EMBO J
Acquired blepharoptosis secondary to essential blepharospasm
Ophthalmic Surg
Apraxia of lid opening: a review
Neurology
Botulinum toxin for aberrant facial nerve regeneration: double-blind, placebo-controlled trial using subjective endpoints
Plast Reconstr Surg
Botulinum A toxin for treatment of aberrant facial nerve regeneration
Plast Reconstr Surg
Botulinum toxin therapy of eye muscle disorders. Safety and effectiveness. American Academy of Ophthalmology
Ophthalmology
The action of botulinum toxin on the neuro-muscular junction
J Physiol
The corticomotor representation of upper limb muscles in writer's cramp and changes following botulinum toxin injection
Brain
Comparison of preseptal and pretarsal injections of botulinum toxin in the treatment of blepharospasm and hemifacial spasm
J Neurol
The development of synkinesis after facial nerve paralysis
Eur Neurol
Aberrant facial nerve regeneration (AFR): an under-recognized cause of ptosis
Eye
After facial nerve damage, regenerating axons become aberrant throughout the length of the nerve and not only at the site of the lesion: an experimental study
Br J Neurosurg
Treatment of aberrant facial nerve regeneration with botulinum toxin A
Orbit
Bell's Palsy–associated blepharospasm relieved by aiding eyelid closure
Ann Neurol
Microvascular decompression for hemifacial spasm: a long-term follow-up of 1,169 consecutive cases
Stereotact Funct Neurosurg
Assessing the role of DRD5 and DYT1 in two different case-control series with primary blepharospasm
Mov Disord
Behavioral technics in the modification of spasmodic torticollis
Neurology
In vitro characterization of botulinum toxin types A, C and D action on human tissues: combined electrophysiologic, pharmacologic and molecular biologic approaches
J Pharmacol Exp Ther
A comparative study of primary and secondary hemifacial spasm
Arch Neurol
Prevalence of primary blepharospasm in Sardinia, Italy: a service-based survey
Mov Disord
Botulinum toxin type A therapy for hemifacial spasm
Cochrane Database Syst Rev
Gabapentin in the treatment of hemifacial spasm
Acta Neurol Scand
Facial dystonia: clinical features, prognosis and pharmacology in 31 patients
Ital J Neurol Sci
Epidemiology of primary blepharospasm
Mov Disord
Prevalence of primary blepharospasm in a community of Puglia region, Southern Italy
Neurology
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