Advertisement

Pathogenesis and Treatment of Hemifacial Spasm

  • Aage R. Møller
Chapter

Abstract

Hemifacial spasm can be cured by microvascular decompression (MVD) operations of the root exit zone of the facial nerve. This fact was the basis for the (“ephaptic”) hypothesis stating that the anatomical location of the pathology that generates the signs of HFS, spasm in the mimic muscle on one side of the face and synkinesis, was the root exit zone of the facial nerve. However, later intracranial recording from the facial nerve provided strong experimental support of a different hypothesis about the pathology of HFS, namely, that the anatomical location of the pathology is the facial motonucleus.

Intraoperative measurements of neural conduction times provided evidence against the “ephaptic” hypothesis and showed evidence that hyperactivity of the facial motonucleus could explain the symptoms of HFS. Studies of the blink reflex supported the hypothesis that the facial motonucleus is hyperactive in people with HFS. The results of animal experiments showed that signs of HFS could be caused by facial motonucleus hyperactivity.

It was hypothesized that the abnormalities in the facial motonucleus in HFS were caused by activation of maladaptive neuroplasticity that was activated by the irritation of the root of the facial nerve by a blood vessel. These findings were supported by the results of animal studies.

Since a similar close contact with a blood vessel is present in at least 50 % of individuals who do not have any symptoms of spasm, it was concluded that a second factor in addition to vascular contact with the facial nerve root must be present in order to create the signs of HFS.

MVD operations have a success rate of over 85 %, and when combined with monitoring of the abnormal muscle contraction, success rates of 97 % have been reported. No other treatment has been shown to have noticeable success in relieving the signs of HFS.

Keywords

Hemifacial spasm Vascular compression Neuroplasticity Facial nucleus Hyperactivity 

References

  1. Auger RG. Hemifacial spasm: clinical and electrophysiologic observations. Neurology. 1979;29:1261–72.CrossRefPubMedGoogle Scholar
  2. Badr-El-Dine M, El-Garem H, Talaat A, Magnan J. Endoscopically assisted minimally invasive microvascular decompression of hemifacial spasm. Otol Neurotol. 2002;23:122–8.CrossRefPubMedGoogle Scholar
  3. Barker FG, Jannetta PJ, Bissonette DJ, Shields PT, Larkins MV. Microvascular decompression for hemifacial spasm. J Neurosurg. 1995;82:201–10.CrossRefPubMedGoogle Scholar
  4. Barker FG, Jannetta PJ, Bissonette DJ, Larkins MV, Jho HD. The long-term outcome of microvascular decompression for trigeminal neuralgia. N Eng J Med. 1996;334:1077–83.CrossRefGoogle Scholar
  5. Campos-Benitez M, Kaufmann A. Neurovascular compression findings in hemifacial spasm. J Neurosurg. 2008;109:416–20.CrossRefPubMedGoogle Scholar
  6. Caspary DM, Raza A, Lawhorn Armour BA et al. Immunocytochemical and neurochemical evidence for age-related loss of GABA in the inferior colliculus: implications for neural presbycusis. The Journal of neuroscience:the official journal of the Society for Neuroscience 1990;10(7):2363–72.Google Scholar
  7. Cushing H. The major trigeminal neuralgias and their surgical treatment based on experience with 332 gasserian operations. Am J Med Sci. 1920;160:158–84.CrossRefGoogle Scholar
  8. Dandy W. An operation for the cure of tic douloureux. Partial section of the sensory root at the pons. Arch Surg. 1929;18:687–734.CrossRefGoogle Scholar
  9. Dandy W. Concerning the cause of trigeminal neuralgia. Am J Surg. 1934;24:447–55.CrossRefGoogle Scholar
  10. DE Ridder D, Møller AR, Verlooy J, Cornelissen M, DE Ridder L. Is the root entry/exit zone important in microvascular compression syndromes? Neurosurgery. 2002;51:427–34.CrossRefPubMedGoogle Scholar
  11. Dobie R, Fisch U. Primary and revision surgery (selective neurectomy) for facial hyperkinesis. Arch Otolaryngol Head Neck Surg. 1986;112:154–63.CrossRefPubMedGoogle Scholar
  12. Ehni G, Woltman H. Hemifacial spasm. Arch Neurol Psychiatry. 1945;53:205–11.CrossRefGoogle Scholar
  13. Engineer ND, Møller AR, Kilgard MP. Directing neural plasticity to understand and treat tinnitus. Hear Res. 2013;295:58–66.CrossRefPubMedGoogle Scholar
  14. Esslen E. Der Spasmus facialis -- eine Parabiosserscheinung: Elektrophysiologische Untersuchnungen zum Enstehungsmechanismus des Facialisspasmus. Dtsch Z Nervenheil. 1957;176:149–72.CrossRefGoogle Scholar
  15. Esteban A, Molina-Negro P. Primary hemifacial spasm: a neurophysiological study. JNNP. 1986;49:58–63.Google Scholar
  16. Ferguson JH. Hemifacial spasm and the facial nucleus. Ann Neurol. 1978;4(97):103.Google Scholar
  17. Fisch U, Esslen E. The surgical treatment of facial hyperkinesis. Arch Otolaryngol Head Neck Surg. 1972;5:400–5.CrossRefGoogle Scholar
  18. Freckmann N, Hagenah R, Herrmann HD, Muller D. Treatment of neurogenic torticollis by microvascular lysis of the accessory nerve roots: indication, technique, and first results. Acta Neurochir (Wien). 1981;59:167–75.CrossRefGoogle Scholar
  19. Fukuda M, Oishi M, Takao T, Hiraishi T, Sato Y, Fujii Y. Monitoring of abnormal muscle response and facial motor evoked potential during microvascular decompression for hemifacial spasm. Surg Neurol Int. 2012;3:118.CrossRefPubMedPubMedCentralGoogle Scholar
  20. Gardner WJ. Crosstalk -- The paradoxical transmission of a nerve impulse. Arch Neurol. 1966;14:149–56.CrossRefPubMedGoogle Scholar
  21. Gardner WJ, Sava GA. Hemifacial spasm -- a reversible pathophysiologic state. J Neurosurg. 1962;19:240–7.CrossRefGoogle Scholar
  22. Goddard GV. Amygdaloid stimulation and learning in the rat. J Comp Physiol Psychol. 1964;58:23–30.CrossRefPubMedGoogle Scholar
  23. Granit R, Leksell L, Skoglund CR. Fibre interaction in injured or compressed region of nerve. Brain. 1944;67:125–40.CrossRefGoogle Scholar
  24. Hai J, Pan Q. Experimental study on the correlation between abnormal muscle responses and F waves in hemifacial spasm. Neurol Res. 2007;29:553–6.CrossRefPubMedGoogle Scholar
  25. Hyun S, Kong D, Park K. Microvascular decompression for treating hemifacial spasm: lessons learned from a prospective study of 1,174 operations. Neurosurg Rev. 2010;33:325–34.CrossRefPubMedGoogle Scholar
  26. Itagaki S, Saito S, Nakai O. Intraoperative recording of evoked EMG in patients with hemifacial spasm – possible physiological mechanism. Facial N Res Jpn (Tokyo). 1988;8:143–6.Google Scholar
  27. Jannetta PJ. Microsurgical exploration and decompression of the facial nerve in hemifacial spasm. Curr Top Surg Res. 1970;2:217–22.Google Scholar
  28. Jannetta PJ. Hemifacial spasm caused by a venule: case report. Neurosurgery. 1984;14:89–92.CrossRefPubMedGoogle Scholar
  29. Jannetta PJ. Cranial rhizopathies. In: Yomans JR, editor. Neurological surgery. Philadelphia: W.B. Saunders; 1990.Google Scholar
  30. Kim P, Fukushima T. Observations on synkinesis in patients with hemifacial spasm. Effect of microvascular decompression and etiological considerations. J Neurosurg. 1984;60(4):821–827.Google Scholar
  31. Kraft SP, Lang AE. Botulinum toxin injections in the treatment of blepharospasm, hemifacial spasm, and eyelid fasciculations. Can J Neurol Sci. 1988;15(3):276–80.Google Scholar
  32. Kollewe K, Mohammad IB, Dengler R, Dressler D. Hemifacial spasm and reinnervation synkinesias: long-term treatment with either Botox or Dysport. J Neural Transm. 2010;117:759–63.CrossRefPubMedGoogle Scholar
  33. Kondo A, Ishikawa J, Yamasaki T, Konishi T. Microvascular decompression of cranial nerves, particularly of the seventh cranial nerve. Neurol Med Chir (Tokyo). 1980;20:739–51.CrossRefGoogle Scholar
  34. Kugelberg E. “Injury activity” and “trigger zones” in human nerves. Brain. 1946;69:310–24.CrossRefPubMedGoogle Scholar
  35. Laha RK, Jannetta PJ. Glossopharyngeal neuralgia. J Neurosurg. 1977;47:316–20.CrossRefPubMedGoogle Scholar
  36. Loeser J, Chen J. Hemifacial spasm treatment by microsurgical facial nerve decompression. Neurosurg. 1983;13:141–6.CrossRefGoogle Scholar
  37. Marion M-H. Hemifacial spasm: treatment with botulinum toxin (long term results). In: Sindou M, Keravel Y, Møller AR, editors. Hemifacial spasm. A multidisiplinary approach. Wien: Springer; 1997. p. 141–4.CrossRefGoogle Scholar
  38. Martinez ARM, Nunes MB, Immich ND, Piovesana L, França Jr M, Campos L, D’abreu A. Misdiagnosis of hemifacial spasm is a frequent event in the primary care setting. Arq Neuro Psiquiatr. 2014;72:119–22.CrossRefGoogle Scholar
  39. Mclaughlin M, Jannetta P, Clyde B, Subach B, Comey C, Resnick D. Microvascular decompression of cranial nerves: lessons learned after 4400 operations. J Neurosurg. 1999;90:1–8.CrossRefPubMedGoogle Scholar
  40. Møller AR. Hemifacial spasm: ephaptic transmission or hyperexcitability of the facial motor nucleus? Exp Neurol. 1987;98:110–9.CrossRefPubMedGoogle Scholar
  41. Møller AR. The cranial nerve vascular compression syndrome: II. A review of pathophysiology. Acta Neurochir (Wien). 1991;113:24–30.CrossRefGoogle Scholar
  42. Møller AR. Cranial nerve dysfunction syndromes: pathophysiology of microvascular compression, chap. 2. In: Barrow DL, editor. Neurosurgical topics book 13, ‘Surgery of cranial nerves of the posterior fossa’. Park Ridge: American Association of Neurological Surgeons; 1993.Google Scholar
  43. Møller AR. Pathophysiology of hemifacial spasm. In: Sindou M, Keravel Y, Møller AR, editors. Hemifacial spasm: a multidisciplinary approach. Wien: Springer; 1997.Google Scholar
  44. Møller AR. Vascular compression of cranial nerves. I: History of the microvascular decompression operation. Neurol Res. 1998;20:727–31.CrossRefPubMedGoogle Scholar
  45. Møller AR. Vascular compression of cranial nerves. II. Pathophysiology. Neurol Res. 1999;21:439–43.PubMedGoogle Scholar
  46. Møller AR. Intraoperative neurophysiological monitoring. 3rd ed. New York: Springer; 2011.CrossRefGoogle Scholar
  47. Møller AR. Neuroplasticity and its dark sides: disorders of the nervous system. Dallas: Aage R. Møller; 2014.Google Scholar
  48. Møller AR, Jannetta PJ. On the origin of synkinesis in hemifacial spasm: results of intracranial recordings. J Neurosurg. 1984;61:569–76.CrossRefPubMedGoogle Scholar
  49. Møller AR, Jannetta PJ. Microvascular decompression in hemifacial spasm: intraoperative electrophysiological observations. Neurosurgery. 1985;16:612–8.CrossRefPubMedGoogle Scholar
  50. Møller AR, Jannetta PJ. Blink reflex in patients with hemifacial spasm: observations during microvascular decompression operations. J Neurol Sci. 1986;72:171–82.CrossRefPubMedGoogle Scholar
  51. Møller AR, Jannetta PJ. Monitoring facial EMG during microvascular decompression operations for hemifacial spasm. J Neurosurg. 1987;66:681–5.CrossRefPubMedGoogle Scholar
  52. Møller AR, Møller MB. Does intraoperative monitoring of auditory evoked potentials reduce incidence of hearing loss as a complication of microvascular decompression of cranial nerves? Neurosurgery. 1989;24:257–63.CrossRefPubMedGoogle Scholar
  53. Nagata K, Matsui T, Joshita H, Shigeno T, Asano T. Surgical treatment of spasmodic torticollis: effectiveness of microvascular decompression. Brain Nerve (Tokyo). 1989;41:97–102.Google Scholar
  54. Nakai O, Itagaki S, Saito S. Electromyographic analysis of spasmodic torticollis. Tenth meeting of the World Society for Stereotactic and Functional Neurosurgery. Abstract. 1989.Google Scholar
  55. Nielsen VK. Pathophysiological aspects of hemifacial spasm. Part. I Evidence of ectopic excitation and ephaptic transmission. Neurology. 1984a;34:418–26.CrossRefPubMedGoogle Scholar
  56. Nielsen VK. Pathophysiology of hemifacial spasm: II. Lateral spread of the supraorbital nerve reflex. Neurology. 1984b;34:427–31.CrossRefPubMedGoogle Scholar
  57. Osburn LL, Møller AR, Bhatt JR, Cohen-Gadol AA. Hemilingual spasm: defining a new entity, its electrophysiological correlates and surgical treatment through microvascular decompression. Neurosurgery. 2010;67:192–5.CrossRefPubMedGoogle Scholar
  58. Saito S, Møller AR. Chronic electrical stimulation of the facial nerve causes signs of facial nucleus hyperactivity. Neurol Res. 1993;15:225–31.CrossRefPubMedGoogle Scholar
  59. Sandyk R, Gillman MA. Baclofen in hemifacial spasm. Int J Neurosci. 1987;33:261–4.CrossRefPubMedGoogle Scholar
  60. Sen CN, Møller AR. Signs of hemifacial spasm created by chronic periodic stimulation of the facial nerve in the rat. Exp Neurol. 1987;98:336–49.CrossRefPubMedGoogle Scholar
  61. Sindou M. Microvascular decompression for primary hemifacial spasm. Importance of intraoperative neurophysiological monitoring. Acta Neurochir (Wien). 2005;147:1019–26.CrossRefGoogle Scholar
  62. Sunderland S. Microvascular relations and anomalies at the base of the brain. J Neurol Neurosurg Psychiatry. 1948;11:243–57.CrossRefPubMedPubMedCentralGoogle Scholar
  63. Taarnhøj P. Decompression of the trigeminal root and the posterior part of the ganglion as treatment in trigeminal neuralgia; preliminary communication. J Neurosurg. 1952;9(3):288–90.Google Scholar
  64. Taarnhøj P. Trigeminal neuralgia and decompression of the trigeminal root. Surg Clin North Am. 1956;1145–57.Google Scholar
  65. Tang I, Freeman S, Kontorinis G, Tang M, Rutherford S, King A, Lloyd S. Geniculate neuralgia: a systematic review. J Laryngol Otol. 2014;128:394–9.CrossRefPubMedGoogle Scholar
  66. Valls-Sole J, Tolosa ES. Blink reflex excitability cycle in hemifacial spasm. Neurology. 1989;39:1061–6.CrossRefPubMedGoogle Scholar
  67. Vial C, Vighetto A. Hemifacial spasm: treatment with botulinum toxin (a report of 50 patients). In: Sindou M, Keravel Y, Møller AR, editors. Hemifacial spasm. A multidisciplinary approach. Wien: Springer; 1997.Google Scholar
  68. Wang X, Thirumala P, Shah A, Gardner P, Habeych M, Crammond D, Balzer J, Burkhart L, Horowitz M. The role of vein in microvascular decompression for hemifacial spasm: a clinical analysis of 15 cases. Neurol Res. 2013;35:389–94.CrossRefPubMedGoogle Scholar
  69. Wilkins R. Hemifacial spsam and other facial nerve dysfunction syndromes. In: Barrow D, editor. Surgery of the cranial nerves of the posterior fossa. Park Ridge: American Association of Neurological Surgeons; 1993.Google Scholar
  70. Williams H, Lambert E, Woltman H. The problem of synkinesis and contracture in cases of hemifacial spasm and Bell’s palsy. Ann Otol Rhinol Laryngol. 1952;40:857–70.Google Scholar
  71. Yeh HSH, Tew JM. Tic convulsif, the combination of geniculate neuralgia and hemifacial spasm relieved by vascular decompression. Neurology. 1984;34:682–3.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media Dordrecht 2016

Authors and Affiliations

  • Aage R. Møller
    • 1
  1. 1.School of Behavioral and Brain SciencesThe University of TexasDallasUSA

Personalised recommendations