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Controversies in Neuro-Ophthalmic Management pp 147–156Cite as

Multiple Cranial Neuropathies

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Abstract

In considering multiple cranial neuropathies, we are examining several important conditions that can affect cranial nerves once they have exited the brainstem. Intrinsic brainstem disease can mimic these syndromes but is almost always accompanied by neighborhood signs. The most common disease location giving rise to multiple cranial neuropathies is the cavernous sinus. Any pathology therein can cause a cavernous sinus syndrome that may present as a combination of unilateral ophthalmoplegia, autonomic dysfunction, and trigeminal neuropathy. The majority of primary lesions in the cavernous sinus are meningiomas, but a cavernous sinus syndrome can result from an adjacent process such as a pituitary adenoma. Less common but much more serious causes of multiple cranial neuropathies are mucormycosis and metastatic cancer.

Keywords

  • Multiple cranial neuropathies
  • Cavernous sinus meningioma
  • Cavernous sinus syndrome
  • Orbital apex syndrome
  • Superior orbital fissure syndrome
  • Cavernous sinus meningioma
  • Mucormycosis
  • IgG4-related disease
  • Tolosa-Hunt syndrome

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Fig. 15.1
Fig. 15.2

References

  1. Keane JR. Multiple cranial nerve palsies: analysis of 979 cases. Arch Neurol. 2005;62(11):1714–7.

    PubMed  CrossRef  Google Scholar 

  2. Chaturvedi A, Baker K, Jeanmonod D, Jeanmonod R. Lyme disease presenting with multiple cranial nerve deficits: report of a case. Case Rep Emerg Med. 2016;2016:1–3.

    CrossRef  Google Scholar 

  3. Newman NJ. Multiple cranial neuropathies: presenting signs of systemic lymphoma. Surv Ophthalmol. 1992;37(2):125–9.

    CAS  PubMed  CrossRef  Google Scholar 

  4. Lacomis D. Neurosarcoidosis. Curr Neuropharmacol. 2011;9(3):429–36.

    CAS  PubMed  PubMed Central  CrossRef  Google Scholar 

  5. Nagashima T, Maguchi S, Terayama Y, Horimoto M, Nemoto M, Nunomura M, et al. P-ANCA-positive Wegener’s granulomatosis presenting with hypertrophic pachymeningitis and multiple cranial neuropathies: case report and review of literature. Neuropathology. 2000;20(1):23–30.

    CAS  PubMed  CrossRef  Google Scholar 

  6. Fujimoto S, Kumamoto T, Ito T, Sannomiya K, Inuzuka T, Tsuda T. A clinicopathological study of a patient with anti-Hu-associated paraneoplastic sensory neuronopathy with multiple cranial nerve palsies. Clin Neurol Neurosurg. 2002;104(2):98–102.

    PubMed  CrossRef  Google Scholar 

  7. Caldarelli C, Benech R, Iaquinta C. Superior orbital fissure syndrome in lateral orbital wall fracture: management and classification update. Craniomaxillofac Trauma Reconstr. 2016;9(4):277–83.

    PubMed  PubMed Central  CrossRef  Google Scholar 

  8. Yeh S, Foroozan R. Orbital apex syndrome. Curr Opin Ophthalmol. 2004;15(6):490–8.

    PubMed  CrossRef  Google Scholar 

  9. Ugradar S, Bonelli L, Rootman D. Facial numbness in the ophthalmology clinic. A portentous sign. Eye (Lond). 2020;34(4):663–8.

    CrossRef  Google Scholar 

  10. Inoue D, Zen Y, Sato Y, Abo H, Demachi H, Uchiyama A, et al. IgG4-related perineural disease. Int J Rheumatol. 2012;2012:401890.

    Google Scholar 

  11. Hardy TG, McNab AA, Rose GE. Enlargement of the infraorbital nerve: an important sign associated with orbital reactive lymphoid hyperplasia or immunoglobulin G4-related disease. Ophthalmology. 2014;121(6):1297–303.

    Google Scholar 

  12. Murad-Kejbou S, Eggenberger E. Pituitary apoplexy: evaluation, management, and prognosis. Curr Opin Ophthalmol. 2009;20(6):456–61.

    PubMed  CrossRef  Google Scholar 

  13. Ebright JR, Pace MT, Niazi AF. Septic thrombosis of the cavernous sinuses. Arch Intern Med. 2001;161(22):2671–6.

    CAS  PubMed  CrossRef  Google Scholar 

  14. Hsu C-W, Tsai W-C, Lien C-Y, Lee J-J, Chang W-N. The clinical characteristics, implicated pathogens and therapeutic outcomes of culture-proven septic cavernous sinus thrombosis. J Clin Neurosci. 2019;68:111–6.

    PubMed  CrossRef  Google Scholar 

  15. Henderson AD, Miller NR. Carotid-cavernous fistula: current concepts in aetiology, investigation, and management. Eye (Lond). 2018;32(2):164–72.

    CAS  CrossRef  Google Scholar 

  16. Kurata A, Takano M, Tokiwa K, Miyasaka Y, Yada K, Kan S. Spontaneous carotid cavernous fistula presenting only with cranial nerve palsies. AJNR Am J Neuroradiol. 1993;14(5):1097–101.

    CAS  PubMed  PubMed Central  Google Scholar 

  17. Wang Y, Wang X-H, Tian M-M, Xie C-J, Liu Y, Pan Q-Q, et al. Ophthalmoplegia starting with a headache circumscribed in a line-shaped area: a subtype of ophthalmoplegic migraine? J Headache Pain. 2014;15(1):19.

    PubMed  PubMed Central  CrossRef  Google Scholar 

  18. Huang C, Amasanti M, Lovell B, Young T. Recurrent painful ophthalmoplegic neuropathy. Pract Neurol. 2017;17(4):318–20.

    PubMed  CrossRef  Google Scholar 

  19. Lal V, Sahota P, Singh P, Gupta A, Prabhakar S. Ophthalmoplegia with migraine in adults: is it ophthalmoplegic migraine? Headache. 2009;49(6):838–50.

    PubMed  CrossRef  Google Scholar 

  20. Baptista AG, DeSouza HS. Pupillary abnormalities in myasthenia gravis. Neurology. 1961;11(3):210.

    CAS  PubMed  CrossRef  Google Scholar 

  21. Al Othman B, Raabe J, Kini A, Lee AG. Update: the Miller Fisher variants of Guillain-Barré syndrome. Curr Opin Ophthalmol. 2019;30(6):462–6.

    Google Scholar 

  22. Jeong W, Keighley C, Wolfe R, Lee WL, Slavin MA, Kong DCM, et al. The epidemiology and clinical manifestations of mucormycosis: a systematic review and meta-analysis of case reports. Clin Microbiol Infect. 2019;25(1):26–34.

    CAS  PubMed  CrossRef  Google Scholar 

  23. Ibrahim AS, Gebermariam T, Fu Y, Lin L, Husseiny MI, French SW, et al. The iron chelator deferasirox protects mice from mucormycosis through iron starvation. J Clin Invest. 2007;117(9):2649–57.

    CAS  PubMed  PubMed Central  CrossRef  Google Scholar 

  24. Mauriello JAJ, Yepez N, Mostafavi R, Barofsky J, Kapila R, Baredes S, et al. Invasive rhinosino-orbital aspergillosis with precipitous visual loss. Can J Ophthalmol. 1995;30(3):124–30.

    PubMed  Google Scholar 

  25. Sivak-Callcott JA, Livesley N, Nugent RA, Rasmussen SL, Saeed P, Rootman J. Localised invasive sino-orbital aspergillosis: characteristic features. Br J Ophthalmol. 2004;88(5):681–7.

    CAS  PubMed  PubMed Central  CrossRef  Google Scholar 

  26. Mody KH, Ali MJ, Vemuganti GK, Nalamada S, Naik MN, Honavar SG. Orbital aspergillosis in immunocompetent patients. Br J Ophthalmol. 2014;98(10):1379–84.

    PubMed  CrossRef  Google Scholar 

  27. Adulkar NG, Radhakrishnan S, Vidhya N, Kim U. Invasive sino-orbital fungal infections in immunocompetent patients: a clinico-pathological study. Eye (Lond). 2019;33(6):988–94.

    CrossRef  Google Scholar 

  28. Roden MM, Zaoutis TE, Buchanan WL, Knudsen TA, Sarkisova TA, Schaufele RL, et al. Epidemiology and outcome of zygomycosis: a review of 929 reported cases. Clin Infect Dis. 2005;41(5):634–53.

    PubMed  CrossRef  Google Scholar 

  29. Kubota T, Moritani S. Orbital IgG4-related disease: clinical features and diagnosis. ISRN Rheumatol. 2012;2012:412896.

    PubMed  PubMed Central  CrossRef  Google Scholar 

  30. Lee CS, Harocopos GJ, Kraus CL, Lee AY, Van Stavern GP, Couch SM, et al. IgG4-associated orbital and ocular inflammation. J Ophthalmic Inflamm Infect. 2015;5:15.

    PubMed  PubMed Central  CrossRef  CAS  Google Scholar 

  31. Tiegs-Heiden CA, Eckel LJ, Hunt CH, Diehn FE, Schwartz KM, Kallmes DF, et al. Immunoglobulin G4-related disease of the orbit: imaging features in 27 patients. AJNR Am J Neuroradiol. 2014;35(7):1393–7.

    CAS  PubMed  PubMed Central  CrossRef  Google Scholar 

  32. Yadlapati S, Verheyen E, Efthimiou P. IgG4-related disease: a complex under-diagnosed clinical entity. Rheumatol Int. 2018;38(2):169–77.

    CAS  PubMed  CrossRef  Google Scholar 

  33. Kamisawa T, Okazaki K. Diagnosis and treatment of IgG4-related disease. Curr Top Microbiol Immunol. 2017;401:19–33.

    PubMed  Google Scholar 

  34. Carruthers MN, Topazian MD, Khosroshahi A, Witzig TE, Wallace ZS, Hart PA, et al. Rituximab for IgG4-related disease: a prospective, open-label trial. Ann Rheum Dis. 2015;74(6):1171–7.

    CAS  PubMed  CrossRef  Google Scholar 

  35. Ebbo M, Grados A, Samson M, Groh M, Loundou A, Rigolet A, et al. Long-term efficacy and safety of rituximab in IgG4-related disease: data from a French nationwide study of thirty-three patients. PLoS One. 2017;12(9):e0183844.

    PubMed  PubMed Central  CrossRef  CAS  Google Scholar 

  36. Smith JL, Taxdal DS. Painful ophthalmoplegia. The Tolosa-Hunt syndrome. Am J Ophthalmol. 1966;61(6):1466–72.

    CAS  PubMed  CrossRef  Google Scholar 

  37. Kline LB, Hoyt WF. The Tolosa-Hunt syndrome. J Neurol Neurosurg Psychiatry. 2001;71(5):577–82.

    CAS  PubMed  PubMed Central  CrossRef  Google Scholar 

  38. Pérez CA, Evangelista M. Evaluation and management of Tolosa-Hunt Syndrome in children: a clinical update. Pediatr Neurol. 2016;62:18–26.

    PubMed  CrossRef  Google Scholar 

  39. Mullen E, Green M, Hersh E, Iloreta A-M, Bederson J, Shrivastava R. Tolosa-Hunt syndrome: appraising the ICHD-3 beta diagnostic criteria. Cephalalgia. 2018;38(10):1696–700.

    PubMed  CrossRef  Google Scholar 

  40. Sondheimer FK, Knapp J. Angiographic findings in the Tolosa-Hunt syndrome: painful ophthalmoplegia. Radiology. 1973;106(1):105–12.

    Google Scholar 

  41. Nassrallah G, Sun V, Guiot M-C, Mikhail M, Arthurs B. Cavernous sinus syndrome associated with metastatic colorectal cancer and perineural spread along the trigeminal nerve. Am J Ophthalmol Case Rep. 2017;6:67–70.

    PubMed  PubMed Central  CrossRef  Google Scholar 

  42. Seixas NB, Belsuzarri TAB, Belsuzarri NCB, Pozetti M, Araujo JFM. Cavernous sinus syndrome as the first manifestation of metastatic breast disease. Surg Neurol Int. 2017;8:40.

    CAS  PubMed  PubMed Central  CrossRef  Google Scholar 

  43. Hughes JD, Kapurch J, Van Gompel JJ, Meyer FB, Pollock BE, Atkinson J, et al. Diagnosis and outcome of biopsies of indeterminate lesions of the cavernous sinus and Meckel’s cave: a retrospective case series in 85 patients. Neurosurgery. 2018;83(3):529–39.

    PubMed  CrossRef  Google Scholar 

  44. Newman S. A prospective study of cavernous sinus surgery for meningiomas and resultant common ophthalmic complications (an American Ophthalmological Society thesis). Trans Am Ophthalmol Soc. 2007;105:392–447.

    PubMed  PubMed Central  Google Scholar 

  45. Heth JA, Al-Mefty O. Cavernous sinus meningiomas. Neurosurg Focus. 2003;14(6):e3.

    PubMed  CrossRef  Google Scholar 

  46. Morisako H, Goto T, Ohata H, Goudihalli SR, Shirosaka K, Ohata K. Safe maximal resection of primary cavernous sinus meningiomas via a minimal anterior and posterior combined transpetrosal approach. Neurosurg Focus. 2018;44(4):E11.

    PubMed  CrossRef  Google Scholar 

  47. O’Sullivan MG, van Loveren HR, Tew JMJ. The surgical resectability of meningiomas of the cavernous sinus. Neurosurgery. 1997;40(2):238–44. discussion 245–7

    PubMed  CrossRef  Google Scholar 

  48. Schatz NJ, Savino PJ, Corbett JJ. Primary aberrant oculomotor regeneration: a sign of intracavernous meningioma. Arch Neurol. 1977;34(1):29–32.

    Google Scholar 

  49. Pollock BE, Stafford SL, Link MJ, Garces YI, Foote RL. Single-fraction radiosurgery of benign cavernous sinus meningiomas. J Neurosurg. 2013;119(3):675–82.

    PubMed  CrossRef  Google Scholar 

  50. Klinger DR, Flores BC, Lewis JJ, Barnett SL. The treatment of cavernous sinus meningiomas: evolution of a modern approach. Neurosurg Focus. 2013;35(6):E8.

    PubMed  CrossRef  Google Scholar 

  51. Sughrue ME, Rutkowski MJ, Aranda D, Barani IJ, McDermott MW, Parsa AT. Factors affecting outcome following treatment of patients with cavernous sinus meningiomas. J Neurosurg. 2010;113(5):1087–92.

    PubMed  CrossRef  Google Scholar 

  52. Leroy H-A, Tuleasca C, Reyns N, Levivier M. Radiosurgery and fractionated radiotherapy for cavernous sinus meningioma: a systematic review and meta-analysis. Acta Neurochir. 2018;160(12):2367–78.

    PubMed  CrossRef  Google Scholar 

  53. Sindou M, Nebbal M, Guclu B. Cavernous sinus meningiomas: imaging and surgical strategy. Adv Tech Stand Neurosurg. 2015;42:103–21.

    PubMed  CrossRef  Google Scholar 

  54. Haghighi N, Seely A, Paul E, Dally M. Hypofractionated stereotactic radiotherapy for benign intracranial tumours of the cavernous sinus. J Clin Neurosci. 2015;22(9):1450–5. https://doi.org/10.1016/j.jocn.2015.03.026.

    CrossRef  PubMed  Google Scholar 

  55. Chen JC, Giannotta SL, Yu C, Petrovich Z, Levy ML, Apuzzo ML. Radiosurgical management of benign cavernous sinus tumors: dose profiles and acute complications. Neurosurgery. 2001;48(5):1022–32. https://doi.org/10.1097/00006123-200105000-00011.

    CAS  CrossRef  PubMed  Google Scholar 

  56. Maclean J, Fersht N, Bremner F, Stacey C, Sivabalasingham S, Short S. Meningioma causing visual impairment: outcomes and toxicity after intensity modulated radiation therapy. Int J Radiat Oncol Biol Phys. 2013;85(4):e179–86. https://doi.org/10.1016/j.ijrobp.2012.10.032.

    CrossRef  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Ophthalmology, Queen’s Medical Centre, Nottingham University Hospitals NHS Trust, Nottingham, UK

    Anna M. Gruener

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  1. Anna M. Gruener
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Editors and Affiliations

  1. Division of Neuro-Ophthalmology Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    MD Amanda D. Henderson

  2. Division of Neuro-Ophthalmology Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    MD Andrew R. Carey

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Gruener, A.M. (2021). Multiple Cranial Neuropathies. In: Henderson, A.D., Carey, A.R. (eds) Controversies in Neuro-Ophthalmic Management. Springer, Cham. https://doi.org/10.1007/978-3-030-74103-7_15

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