Hemifacial Spasm 

  • Author: Steven Gulevich, MD; Chief Editor: Nicholas Lorenzo, MD   more...
 
Updated: Jun 11, 2010
 

Background

Facial musculature is subject to the same movement disorders as muscles of the limbs or trunk. Myoclonus, dystonia, and other movement disorders present with specific syndromes in the facial musculature. An understanding of the underlying mechanism leads to appropriate diagnostic evaluation and potential treatment.

Although specific treatments are available for many craniofacial movement disorders, botulinum toxin (BTX) chemodenervation has proven useful in many of these disorders, supplanting surgery and medical therapy.

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Pathophysiology

First described by Gowers in 1884, hemifacial spasm (HFS) represents a segmental myoclonus of muscles innervated by the facial nerve. The disorder presents in the fifth or sixth decade of life, almost always unilaterally, although bilateral involvement may occur rarely in severe cases. Hemifacial spasm generally begins with brief clonic movements of the orbicularis oculi and spreads over years to other facial muscles (corrugator, frontalis, orbicularis oris, platysma, zygomaticus).

Clonic movements progress to sustained tonic contractions of involved musculature. Chronic irritation of the facial nerve or nucleus, the near-universal cause of hemifacial spasm, may arise from numerous underlying conditions.

Irritation of the facial nerve nucleus is believed to lead to hyperexcitability of the facial nerve nucleus, while irritation of the proximal nerve segment may cause ephaptic transmission within the facial nerve. Either mechanism explains the rhythmic involuntary myoclonic contractions observed in hemifacial spasm.

Compressive lesions (eg, tumor, arteriovenous malformation, Paget disease) and noncompressive lesions (eg, stroke, multiple sclerosis plaque, basilar meningitis) may present as hemifacial spasm. Most instances of hemifacial spasm previously thought to be idiopathic were probably caused by aberrant blood vessels (eg, distal branches of the anterior inferior cerebellar artery or vertebral artery) compressing the facial nerve within the cerebellopontine angle.

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Epidemiology

Race

All races are affected equally.

Sex

A slight female preponderance exists in hemifacial spasm.

Age

  • Idiopathic hemifacial spasm typically begins in the fifth or sixth decade of life.
  • Onset of hemifacial spasm in patients younger than 40 years is unusual and often heralds an underlying neurologic illness (eg, multiple sclerosis).
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Contributor Information and Disclosures
Author

Steven Gulevich, MD  Centennial Medical Center, Centennial, Colorado

Steven Gulevich, MD is a member of the following medical societies: American Academy of Neurology and Colorado Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Stephen A Berman, MD, PhD, MBA  Professor of Neurology, University of Central Florida College of Medicine

Stephen A Berman, MD, PhD, MBA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Glenn Lopate, MD  Associate Professor, Department of Neurology, Division of Neuromuscular Diseases, Washington University School of Medicine; Director of Neurology Clinic, St Louis ConnectCare; Consulting Staff, Department of Neurology, Barnes-Jewish Hospital

Glenn Lopate, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Ortho McNeil Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Speaking, consulting

Chief Editor

Nicholas Lorenzo, MD  Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants

Nicholas Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology

Disclosure: Nothing to disclose.

References

  1. Mauriello JA, Leone T, Dhillon S, et al. Treatment choices of 119 patients with hemifacial spasm over 11 years. Clin Neurol Neurosurg. Aug 1996;98(3):213-6. [Medline].

  2. Jankovic J, Schwartz K, Donovan DT. Botulinum toxin treatment of cranial-cervical dystonia, spasmodic dysphonia, other focal dystonias and hemifacial spasm. J Neurol Neurosurg Psychiatry. Aug 1990;53(8):633-9. [Medline].

  3. Colosimo C, Chianese M, Giovannelli M, et al. Botulinum toxin type B in blepharospasm and hemifacial spasm. J Neurol Neurosurg Psychiatry. May 2003;74(5):687. [Medline].

  4. Adler CH, Zimmerman RA, Savino PJ, et al. Hemifacial spasm: evaluation by magnetic resonance imaging and magnetic resonance tomographic angiography. Ann Neurol. Oct 1992;32(4):502-6. [Medline].

  5. Campos-Benitez M, Kaufmann AM. Neurovascular compression findings in hemifacial spasm. J Neurosurg. Sep 2008;109(3):416-20. [Medline].

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  7. Elston JS. The management of blepharospasm and hemifacial spasm. J Neurol. Jan 1992;239(1):5-8. [Medline].

  8. Jannetta PJ, Abbasy M, Maroon JC, et al. Etiology and definitive microsurgical treatment of hemifacial spasm. Operative techniques and results in 47 patients. J Neurosurg. Sep 1977;47(3):321-8. [Medline].

  9. Kraft SP, Lang AE. Cranial dystonia, blepharospasm and hemifacial spasm: clinical features and treatment, including the use of botulinum toxin. CMAJ. Nov 1 1988;139(9):837-44. [Medline].

  10. Moller AR. The cranial nerve vascular compression syndrome: I. A review of treatment. Acta Neurochir (Wien). 1991;113(1-2):18-23. [Medline].

  11. Moller AR. The cranial nerve vascular compression syndrome: II. A review of pathophysiology. Acta Neurochir (Wien). 1991;113(1-2):24-30. [Medline].

  12. Reimer J, Gilg K, Karow A, et al. Health-related quality of life in blepharospasm or hemifacial spasm. Acta Neurol Scand. Jan 2005;111(1):64-70. [Medline].

 
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