Elsevier

Survey of Ophthalmology

Volume 56, Issue 1, January–February 2011, Pages 54-67
Survey of Ophthalmology

Major Review
Review and Update of Involuntary Facial Movement Disorders Presenting in the Ophthalmological Setting

https://doi.org/10.1016/j.survophthal.2010.03.008Get rights and content

Abstract

We review the existing literature on the involuntary facial movement disorders—benign essential blepharospasm, apraxia of eyelid opening, hemifacial spasm, and aberrant facial nerve regeneration. The etiology of idiopathic blepharospasm, a disorder of the central nervous system, and hemifacial spasm, a condition involving the facial nerve of the peripheral nervous system, is markedly different. We discuss established methods of managing patients and highlight new approaches.

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)

  • T.W. Jones et al.

    Myectomy for essential blepharospasm

    Mayo Clin Proc

    (1985)
  • M.S. LeDoux

    Meige syndrome: what's in a name?

    Parkinsonism Relat Disord

    (2009)
  • R.L. Levy et al.

    Supramaximal doses of botulinum toxin for refractory blepharospasm

    Ophthalmology

    (2006)
  • W.H. Adams et al.

    The evaluation of light sensitivity in benign essential blepharospasm

    Am J Ophthalmol

    (2006)
  • A. Albanese et al.

    Pretarsal injections of botulinum toxin improve blepharospasm in previously unresponsive patients

    J Neurol Neurosurg Psychiatry

    (1996)
  • G.E. Alexander et al.

    Carbamazepine for hemifacial spasm

    Neurology

    (1982)
  • R.L. Anderson et al.

    Blepharospasm: past, present, and future

    Ophthal Plast Reconstr Surg

    (1998)
  • F. Antonucci et al.

    Long-distance retrograde effects of botulinum neurotoxin A

    J Neurosci

    (2008)
  • A.C. Ashton et al.

    Characterization of the inhibitory action of botulinum neurotoxin type A on the release of several transmitters from rat cerebrocortical synaptosomes

    J Neurochem

    (1988)
  • R.G. Auger et al.

    Hemifacial spasm in Rochester and Olmsted County, Minnesota, 1960 to 1984

    Arch Neurol

    (1990)
  • R.S. Baker et al.

    Maladaptive neural compensatory mechanisms in Bell's palsy–induced blepharospasm

    Neurology

    (1997)
  • G.J. Ben Simon et al.

    Benign essential blepharospasm

    Int Ophthalmol Clin

    (2005)
  • A. Berardelli et al.

    Pathophysiology of blepharospasm and oromandibular dystonia

    Brain

    (1985)
  • B. Bernardi et al.

    Magnetic resonance tomographic angiography in the investigation of hemifacial spasm

    Neuroradiology

    (1993)
  • J. Blasi et al.

    Botulinum neurotoxin A selectively cleaves the synaptic protein SNAP-25

    Nature

    (1993)
  • J. Blasi et al.

    Botulinum neurotoxin C1 blocks neurotransmitter release by means of cleaving HPC–1/syntaxin

    EMBO J

    (1993)
  • F.S. Bodker et al.

    Acquired blepharoptosis secondary to essential blepharospasm

    Ophthalmic Surg

    (1993 Aug)
  • D. Boghen

    Apraxia of lid opening: a review

    Neurology

    (1997)
  • G. Borodic et al.

    Botulinum toxin for aberrant facial nerve regeneration: double-blind, placebo-controlled trial using subjective endpoints

    Plast Reconstr Surg

    (2005)
  • G.E. Borodic et al.

    Botulinum A toxin for treatment of aberrant facial nerve regeneration

    Plast Reconstr Surg

    (1993)
  • Botulinum toxin therapy of eye muscle disorders. Safety and effectiveness. American Academy of Ophthalmology

    Ophthalmology

    (1989)
  • A.S. Burgen et al.

    The action of botulinum toxin on the neuro-muscular junction

    J Physiol

    (1949)
  • M.L. Byrnes et al.

    The corticomotor representation of upper limb muscles in writer's cramp and changes following botulinum toxin injection

    Brain

    (1998 May)
  • R. Cakmur et al.

    Comparison of preseptal and pretarsal injections of botulinum toxin in the treatment of blepharospasm and hemifacial spasm

    J Neurol

    (2002)
  • M. Celik et al.

    The development of synkinesis after facial nerve paralysis

    Eur Neurol

    (2000)
  • C. Chen et al.

    Aberrant facial nerve regeneration (AFR): an under-recognized cause of ptosis

    Eye

    (2004)
  • D. Choi et al.

    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

    (2004)
  • C.N. Chua et al.

    Treatment of aberrant facial nerve regeneration with botulinum toxin A

    Orbit

    (2004)
  • J.C. Chuke et al.

    Bell's Palsy–associated blepharospasm relieved by aiding eyelid closure

    Ann Neurol

    (1996)
  • S.S. Chung et al.

    Microvascular decompression for hemifacial spasm: a long-term follow-up of 1,169 consecutive cases

    Stereotact Funct Neurosurg

    (2001)
  • J. Clarimon et al.

    Assessing the role of DRD5 and DYT1 in two different case-control series with primary blepharospasm

    Mov Disord

    (2007)
  • C.S. Cleeland

    Behavioral technics in the modification of spasmodic torticollis

    Neurology

    (1973)
  • J.A. Coffield et al.

    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

    (1997)
  • C. Colosimo et al.

    A comparative study of primary and secondary hemifacial spasm

    Arch Neurol

    (2006)
  • G. Cossu et al.

    Prevalence of primary blepharospasm in Sardinia, Italy: a service-based survey

    Mov Disord

    (2006)
  • J. Costa et al.

    Botulinum toxin type A therapy for hemifacial spasm

    Cochrane Database Syst Rev

    (2005 Jan 25)
  • O. Daniele et al.

    Gabapentin in the treatment of hemifacial spasm

    Acta Neurol Scand

    (2001)
  • G. Defazio et al.

    Facial dystonia: clinical features, prognosis and pharmacology in 31 patients

    Ital J Neurol Sci

    (1989)
  • G. Defazio et al.

    Epidemiology of primary blepharospasm

    Mov Disord

    (2002)
  • G. Defazio et al.

    Prevalence of primary blepharospasm in a community of Puglia region, Southern Italy

    Neurology

    (2001)
  • Cited by (39)

    • Eyelid and Facial Nerve Disorders

      2018, Liu, Volpe, and Galetta's Neuro-Ophthalmology: Diagnosis and Management
    • <sup>123</sup>I-FP-CIT SPECT imaging in blepharospasm

      2017, Revue Neurologique
      Citation Excerpt :

      Blepharospasm (BSP) is one of the most common forms of focal dystonia, with a prevalence of 5–133/100,000 population [1,2]. The condition is characterized by involuntary bilateral increased blinking and intermittent eye spasms, associated with contraction of the eyelid protractors (orbicularis oculi, corrugator supercilii and procerus muscles) [1,3]. BSP is probably related to dysfunction of the descending basal ganglia pathways, which control the blink reflex [4].

    View all citing articles on Scopus
    View full text