Semin Neurol 2009; 29(1): 005-013
DOI: 10.1055/s-0028-1124018
© Thieme Medical Publishers

Seventh Cranial Neuropathy

James M. Gilchrist1
  • 1Warren Alpert Medical School of Brown University, Rhode Island Hospital, Providence, Rhode Island
Further Information

Publication History

Publication Date:
12 February 2009 (online)

ABSTRACT

Facial neuropathy, or seventh cranial neuropathy, is the most common cranial neuropathy. The anatomy of the facial nerve is rather complex for a cranial nerve, with a long intracranial course, in which the nerve takes three bends (or genu). Electrodiagnosis can be helpful in prognosis, but not before several days. Imaging is rarely indicated in Bell's palsy, but is often abnormal nonetheless, and can be very useful in other causes of facial neuropathy. The clinical presentation is of unilateral facial weakness of upper and lower face, hyperacusis, dysgeusia, and disordered lacrimation and salivation. Many different disease processes can result in facial neuropathy, but 70% of cases are idiopathic, or as it is best known, Bell's palsy. Ramsay Hunt syndrome, defined as facial neuropathy with herpes zoster oticus, is another common cause. Steroids given acutely are beneficial in improving outcome in Bell's palsy, and antiviral therapy seems helpful in more severe cases. Antiviral therapy is definitely helpful in Ramsay Hunt disease when given within 3 days of onset. Antibiotics are helpful in Lyme facial neuropathy, which has a very good prognosis.

REFERENCES

  • 1 Hauser W A, Karnes W E, Annis J, Kurland L T. Incidence and prognosis of Bell's palsy in the population of Rochester, Minnesota.  Mayo Clin Proc. 1971;  46 258-264
  • 2 Yanagihara N. Incidence of Bell's palsy.  Ann Otol Rhinol Laryngol Suppl. 1988;  137 3-4
  • 3 Klein C M. Diseases of the seventh cranial nerve. In: Dyck PJ, Thomas PK Peripheral Neuropathy. 4th ed. Philadelphia; Elsevier Saunders 2005: 1219-1220
  • 4 Ge X X, Spector G J. Labyrinthine segment and geniculate ganglion of facial nerve in fetal and adult human temporal bones.  Ann Otol Rhinol Laryngol Suppl. 1981;  90 1-12
  • 5 Katusic S K, Beard M, Wiederholt W C, Bergstralh E J, Kurland K T. Incidence, clinical findings, and prognosis in Bell's palsy, Rochester, Minnesota, 1968–1982.  Ann Neurol. 1986;  20 622-627
  • 6 Keane J R. Bilateral seventh nerve palsy: analysis of 43 cases and review of the literature.  Neurology. 1994;  44 1198-1202
  • 7 Berg T, Jonsson L, Engstrom M. Agreement between the Sunnybrook, House-Brackman, and Yanagihara facial nerve grading systems in Bell's palsy.  Otol Neurotol. 2004;  25 1020-1026
  • 8 Gantz B J, Rubinstein J T, Gidley P, Woodworth G G. Surgical management of Bell's palsy.  Laryngoscope. 1999;  109 1177-1188
  • 9 Vanopdenbosch L J, Verhoeven K, Casselman J W. Bell's palsy with ipsilateral numbness.  J Neurol Neurosurg Psychiatry. 2005;  76 1017-1018
  • 10 Sweeney C J, Gilden D H. Ramsay Hunt syndrome.  J Neurol Neurosurg Psychiatry. 2001;  71 149-154
  • 11 Kimura J, Rodnitzky R L, Okawara S. Electrophysiologic analysis of aberrant regeneration after facial nerve paralysis.  Neurology. 1975;  25 989-993
  • 12 Gilliatt R W, Taylor J C. Electrical changes following section of the facial nerve.  Proc R Soc Med. 1959;  52 1080-1083
  • 13 Olsen P Z. Prediction of recovery in Bell's palsy.  Acta Neurol Scand Suppl. 1975;  61 1-121
  • 14 Dumitru D, Walsh N E, Porter L D. Electrophysiologic evaluation of the facial nerve in Bell's palsy.  Am J Phys Med Rehabil. 1988;  67 137-144
  • 15 Kennelly K D. Electrophysiological evaluation of cranial nerves.  Neurologist. 2006;  12 188-203
  • 16 Engstrom M, Jonsson R, Grindlund M, Stalberg E. Electroneurographic facial muscle pattern in Bell's palsy.  Otolaryngol Head Neck Surg. 2000;  122 290-297
  • 17 Taverner D. Electrodiagnosis in facial palsy.  Arch Otolaryngol. 1965;  81 470-477
  • 18 Gilchrist J M. AAEM case report #26: seventh cranial neuropathy.  Muscle Nerve. 1993;  16 447-452
  • 19 Devi S, Challenor Y, Duarte N et al.. Prognostic value of minimal excitability of facial nerve in Bell's palsy.  J Neurol Neurosurg Psychiatry. 1978;  41 649-652
  • 20 Groves J, Gibson W PR. Bell's (idiopathic facial) palsy: the nerve excitability test in selection of cases for early treatment.  J Laryngol Otol. 1974;  88 851-854
  • 21 Korzec K, Sobol S M, Kubal W, Mester S J, Winzelberg G, May M. Gadolinium-enhanced magnetic resonance imaging of the facial nerve in herpes zoster oticus and Bell's palsy: clinical implications.  Am J Otol. 1991;  12 163-168
  • 22 Sartoretti-Schefer S, Wichmann W, Valavanis A. Idiopathic, herpetic, and HIV-associated facial nerve palsies: abnormal MR enhancement patterns.  AJNR Am J Neuroradiol. 1994;  15 479-485
  • 23 Engström M, Abdsaleh S, Ahlström H, Johansson L, Stålberg E, Jonsson L. Serial gadolinium-enhanced magnetic resonance imaging and assessment of facial nerve function in Bell's palsy.  Otolaryngol Head Neck Surg. 1997;  117 559-566
  • 24 Adour K K, Byl F M, Hilsinger Jr R L, Kahn Z M, Sheldon M I. The true nature of Bell's palsy: analysis of 1,000 consecutive patients.  Laryngoscope. 1978;  88 787-801
  • 25 Hato N, Kisaki H, Honda N, Gyo K, Murakami S, Yanagihara N. Ramsay Hunt syndrome in children.  Ann Neurol. 2000;  48 254-256
  • 26 Sullivan F M, Swan I RC, Donnan P T et al.. Early treatment with prednisolone or acyclovir in Bell's palsy.  N Engl J Med. 2007;  357 1598-1607
  • 27 McCormick D P. Herpes-simplex virus as cause of Bell's palsy.  Lancet. 1972;  1 937-939
  • 28 Hato N, Murakami S, Gyo K. Steroid and antiviral treatment for Bell's palsy.  Lancet. 2008;  371 1818-1820
  • 29 Furuta Y, Fukuda S, Chida E et al.. Reactivation of herpes simplex virus type 1 in patients with Bell's palsy.  J Med Virol. 1998;  54 162-166
  • 30 Takasu T, Furuta Y, Sato K C et al.. Detection of latent herpes simplex virus DNA and RNA in human geniculate ganglia by the polymerase chain reaction.  Acta Otolaryngol. 1992;  112 1004-1011
  • 31 Burgess R C, Micheals L, Bates Jr J F, Smith R J. Polymerase chain reaction amplification of herpes simplex viral DNA from the geniculate ganglion of a patient with Bell's palsy.  Ann Otol Rhinol Laryngol. 1994;  103 775-779
  • 32 Murakami S, Mizobuchi M, Nakashiro Y et al.. Bell's palsy and herpes simplex virus: identification of viral DNA in endoneural fluid and muscle.  Ann Intern Med. 1996;  124 27-30
  • 33 Sugita T, Murakami S, Yanagihara N et al.. Facial nerve paralysis induced by herpes simplex virus in mice, an animal model of acute and transient facial paralysis.  Ann Otol Rhinol Laryngol. 1995;  104 574-581
  • 34 Hato N, Hitsumoto Y, Honda N et al.. Immunologic aspects of facial nerve paralysis induced by herpes simplex virus infection in mice.  Ann Otol Rhinol Laryngol. 1998;  107 633-637
  • 35 Takahashi H, Hitsumoto Y, Honda N et al.. Mouse model of Bell's palsy induced by reactivation of herpes simplex virus type 1.  J Neuropathol Exp Neurol. 2001;  60 621-627
  • 36 Honda N, Hato N, Takahashi H et al.. Pathophysiology of facial nerve paralysis induced by herpes simplex virus type 1 infection.  Ann Otol Rhinol Laryngol. 2002;  111 616-622
  • 37 Finsterer J. Management of peripheral facial nerve palsy.  Eur Arch Otorhinolaryngol. 2008;  265 743-752
  • 38 Salinas R A, Alvarez G, Ferreira J. Corticosteroids for Bell's palsy (idiopathic facial paralysis).  Cochrane Database Sys Rev. 2004;  (4) , CD001942
  • 39 Allen D, Dunn L. Aciclovir or valaciclovir for Bell's palsy (idiopathic facial paralysis).  Cochrane Database Syst Rev. 2004;  (3) , CD001869
  • 40 Hato N, Yamada H, Kohno H et al.. Valacyclovir and prednisolone treatment for Bell`s palsy: A multicenter, randomized, placebo-controlled study.  Otol Neurotol. 2007;  28 408-413
  • 41 Korf E S, Killestein J. Prednisolone or acyclovir in Bell's palsy.  N Engl J Med. 2008;  358 306-307
  • 42 He L, Zhou M K, Zhou D et al.. Acupuncture for Bell's palsy.  Cochrane Database Syst Rev. 2007;  (4) , 002914
  • 43 Adour K K, Diamond C. Decompression of the facial nerve in Bell's palsy: a historical review.  Otolaryngol Head Neck Surg. 1982;  90 453-460
  • 44 May M, Klein S R, Taylor F H. Idiopathic (Bell's) facial palsy: natural history defies steroid or surgical treatment.  Laryngoscope. 1985;  95 406-409
  • 45 Murakami S, Honda N, Mizobuchi M et al.. Rapid diagnosis of varicella zoster virus infection in acute facial palsy.  Neurology. 1998;  51 1202-1205
  • 46 Devriese P P, Moesker W H. The natural history of facial paralysis in herpes zoster.  Clin Otolaryngol Allied Sci. 1988;  13 289-298
  • 47 Murakami S, Hato N, Horiuchi J et al.. Treatment of Ramsay Hunt syndrome with acyclovir-prednisone: significance of early diagnosis and treatment.  Ann Neurol. 1997;  41 353-357
  • 48 Halperin J J. Nervous system Lyme disease.  Infect Dis Clin North Am. 2008;  22 261-274
  • 49 Pachner A R, Steere A C. The triad of neurologic manifestations of Lyme disease.  Neurology. 1985;  35 47-53
  • 50 Clark J R, Carlson R D, Sasaki C T et al.. Facial paralysis in Lyme disease.  Laryngoscope. 1985;  95 1341-1345
  • 51 Halperin J J. Facial nerve palsy associated with Lyme disease.  Muscle Nerve. 2003;  28 516-517
  • 52 Halperin J J, Shapiro E D, Logigian E L et al.. Practice parameter: treatment of nervous system Lyme disease.  Neurology. 2007;  69 91-102
  • 53 Shapiro E D, Gerber M A. Lyme disease and facial nerve palsy.  Arch Pediatr Adolesc Med. 1997;  151 1183-1184

James M GilchristM.D. 

Senior Vice Chairman of Neurology, Rhode Island Hospital

593 Eddy Street, APC 689, Providence, RI 02903

Email: jgilchrist@lifespan.org