Skip to main content

Advertisement

SpringerLink
Log in

Painful ophthalmoplegia: Overview with a focus on tolosa-hunt syndrome

  • Published:
Current Pain and Headache Reports Aims and scope Submit manuscript

Abstract

Painful ophthalmoplegia is an important presenting complaint to emergency departments, ophthalmologists, and neurologists. The etiological differential diagnosis of painful ophthalmoplegia is extensive and consists of numerous sinister etiologies including vascular (eg, aneurysm, carotid dissection, carotid-cavernous fistula), neoplasms (eg, primary intracranial tumors, local or distant metastases), inflammatory conditions (eg, orbital pseudotumor, sarcoidosis, Tolosa-Hunt syndrome), infectious etiologies (eg, fungal, mycobacterial), and other conditions (eg, microvascular infarcts secondary to diabetes, ophthalmoplegic migraine, giant cell arteritis). A systematic approach to the evaluation of painful ophthalmoplegia can lead to prompt recognition of serious disorders that if left untreated, can be associated with significant morbidity or mortality. Inflammatory conditions such as Tolosa-Hunt syndrome and orbital pseudotumor are highly responsive to corticosteroids, but should be diagnoses of exclusion.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References and Recommended Reading

  1. Lake JP: Superior orbital fissure syndrome. Arch Neurol 1962, 7:289–300.

    Google Scholar 

  2. Hallpike JF: Superior orbital fissure syndrome. J Neurol Neurosurg Psychiatry 1973, 36:486–490.

    PubMed  CAS  Google Scholar 

  3. Keane JR: Cavernous sinus syndrome: analysis of 151 cases. Arch Neurol 1996, 53:967–971.

    PubMed  CAS  Google Scholar 

  4. Lee JH, Lee HK, Park JK, et al.: Cavernous sinus syndrome: clinical features and differential diagnosis with MR imaging. AJR Am J Roentgenol 2003, 181:583–590. A concise review of the major cause of cavernous sinus syndrome with illustrative MRI.

    PubMed  Google Scholar 

  5. Johnston JL: Parasellar syndromes. Curr Neurol Neurosci Rep 2002, 2:423–431.

    Article  PubMed  Google Scholar 

  6. Lee AG, Beaver HA, Brazis PW: Painful ophthalmologic disorders and eye pain for the neurologist. Neurol Clin 2004, 22:75–97. A thorough review highlighting causes of headache and eye pain.

    Article  PubMed  Google Scholar 

  7. Kasner SE, Liu GT, Galetta SL: Neuro-ophthalmologic aspects of aneurysms. Neuroimaging Clin N Am 1997, 7:679–692.

    PubMed  CAS  Google Scholar 

  8. Biousse V, Newman NJ: Aneurysms and subarachnoid hemorrhage. Neurosurg Clin N Am 1999, 10:631–651.

    PubMed  CAS  Google Scholar 

  9. Kupersmith MJ, Hurst R, Berenstein A, et al.: The benign course of cavernous carotid artery aneurysms. J Neurosurg 1992, 77:690–693.

    PubMed  CAS  Google Scholar 

  10. Wiebers DO, Whisnant JP, Huston J 3rd, et al.: Unruptured intracranial aneurysms: natural history, clinical outcome, and risks of surgical and endovascular treatment. Lancet 2003, 362:103–110.

    Article  PubMed  Google Scholar 

  11. White PM, Teadsale E, Wardlaw JM, et al.: What is the most sensitive non-invasive imaging strategy for the diagnosis of intracranial aneurysms? J Neurol Neurosurg Psychiatry 2001, 71:322–328.

    Article  PubMed  CAS  Google Scholar 

  12. Okahara M, Kiyosue H, Yamashita M, et al.: Diagnostic accuracy of magnetic resonance angiography for cerebral aneurysms in correlation with 3D-digital subtraction angiographic images: a study of 133 aneurysms. Stroke 2002, 33:1803–1808.

    Article  PubMed  Google Scholar 

  13. Schievink WI: Intracranial aneurysms. N Engl J Med 1997, 336:28–40.

    Article  PubMed  CAS  Google Scholar 

  14. Mokri B: Headaches in cervical artery dissections. Curr Pain Headache Rep 2002, 6:209–216.

    Article  PubMed  Google Scholar 

  15. Biousse V, Toulboul PJ, D’Anglejan-Chatillon J, et al.: Ophthalmologic manifestations of internal carotid artery dissection. Am J Ophthalmol 1998, 126:565–577.

    Article  PubMed  CAS  Google Scholar 

  16. Feiner L, Bennett J, Volpe NJ: Cavernous sinus fistulas: carotid cavernous fistulas and dural arteriovenous malformations. Curr Neurol Neurosci Rep 2003, 3:415–420.

    Article  PubMed  Google Scholar 

  17. Eggenberger E: A bruital headache and double vision. Surv Ophthalmology 2000, 45:147–153.

    Article  CAS  Google Scholar 

  18. Ebright JR, Pace MT, Niazi AF: Septic thrombosis of the cavernous sinuses. Arch Intern Med 2001, 161:2671–2676.

    Article  PubMed  CAS  Google Scholar 

  19. DiNubile MJ: Septic thrombosis of the cavernous sinuses. Arch Neurol 1988, 45:567–572.

    PubMed  CAS  Google Scholar 

  20. Biousse V, Newman NJ, Oyesiku NM: Precipitating factors in pituitary apoplexy. J Neurol Neurosurg Psychiatry 2001, 71:542–545.

    Article  PubMed  CAS  Google Scholar 

  21. Bills DC, Meyer FB, Laws ER, et al.: A retrospective analysis of pituitary apoplexy. Neurosurgery 1993, 33:602–609.

    Article  PubMed  CAS  Google Scholar 

  22. Lanzino G, Hirsch WL, Pomonis S, et al.: Cavernous sinus tumors: neuroradiologic and neurosurgical considerations on 150 operated cases. J Neurosurg Sci 1992, 36:183–196.

    PubMed  CAS  Google Scholar 

  23. Wroe SJ, Thompson AJ, McDonald WI: Painful intraorbital meningiomas. J Neurol Neurosurg Psychiatry 1991, 54:1009–1010.

    PubMed  CAS  Google Scholar 

  24. Holland D, Maune S, Kovacs G, Behrendt S: Metastatic tumors of the orbit: a retrospective study. Orbit 2003, 22:15–24.

    Article  PubMed  Google Scholar 

  25. Jacobs D, Galetta S: Diagnosis and management of orbital pseudotumor. Curr Opin Ophthalmol 2002, 13:347–351. An excellent review of an important topic for every clinician involved in the treatment of headache and facial pain.

    Article  PubMed  Google Scholar 

  26. Wasmeier C, Pfadenhauer K, Rosler A: Idiopathic inflammatory pseudotumor of the orbit and Tolosa-Hunt syndrome: Are they the same disease? J Neurol 2002, 249:1237–1241.

    Article  PubMed  CAS  Google Scholar 

  27. Kupersmith MJ, Martin V, Heller G, et al.: Idiopathic hypertrophic pachymeningitis. Neurology 2004, 62:686–694.

    PubMed  CAS  Google Scholar 

  28. Bosch J, Ortega-Aznar A, Tintore M, et al.: Hypertrophic pachymeningitis: a review of the histories of two cases and pathological relationship with Tolosa-Hunt syndrome and the orbital pseudotumor. Rev Neurol 2000, 31:946–951.

    PubMed  CAS  Google Scholar 

  29. Gullapalli D, Phillips LH: Neurologic manifestations of sarcoidosis. Neurol Clin 2002, 20:59–83.

    Article  PubMed  Google Scholar 

  30. Zarei M, Anderson JR, Higgins JN, et al.: Cavernous sinus syndrome as the only manifestation of sarcoidosis. J Postgrad Med 2002, 48:119–121.

    PubMed  CAS  Google Scholar 

  31. Shaikh ZA, Bakshi R, Greenberg SJ, et al.: Orbital involvement as the initial manifestation of sarcoidosis: magnetic resonance imaging findings. J Neuroimaging 2000, 10:180–183.

    PubMed  CAS  Google Scholar 

  32. Smith JK, Matheus MG, Castillo M: Imaging manifestations of neurosarcoidosis. AJR Am J Roentgenol 2004, 182:289–295.

    PubMed  Google Scholar 

  33. Redillas C, Solomon S: Recent advances in temporal arteritis. Curr Pain Headache Rep 2003, 7:297–302.

    PubMed  Google Scholar 

  34. Hayreh SS, Podhajsky PA, Zimmerman B: Ocular manifestations of giant cell arteritis. Am J Ophthalmol 1998, 125:509–520.

    Article  PubMed  CAS  Google Scholar 

  35. Setama GW, Shmerling RH: Does this patient have temporal arteritis? JAMA 2002, 287:92–101.

    Article  Google Scholar 

  36. Fechner FP, Faquin WC, Pilch BZ: Wegener’s granulomatosis of the orbit: a clinicopathological study of 15 patients. Laryngoscope 2002, 112:1945–1950.

    Article  PubMed  Google Scholar 

  37. Scott IU, Siatkowski MR: Thyroid eye disease. Semin Ophthalmol 1999, 14:52–61.

    PubMed  CAS  Google Scholar 

  38. Chavis PS: Thyroid and the eye. Curr Opin Ophthalmol 2002, 13:352–356.

    Article  PubMed  Google Scholar 

  39. Warwar RE, Bullock JD: Rhino-orbital-cerebral mucormycosis: a review. Orbit 1998, 17:237–245.

    Article  PubMed  Google Scholar 

  40. Chandra S, Goyal M, Mishra NK, et al.: Invasive aspergillosis presenting as a cavernous sinus mass in immunocompetent individuals: report of three cases. Neuroradiology 2000, 42:108–111.

    Article  PubMed  CAS  Google Scholar 

  41. Rebai R, Boudawara MZ, Rahloul K, et al.: Cavernous sinus tuberculoma: diagnostic difficulties in a personal case. Surg Neurol 2001, 55:372–375.

    Article  PubMed  CAS  Google Scholar 

  42. Chang-Godinich A, Lee AG, Brazis PW, et al.: Complete ophthalmoplegia after zoster ophthalmicus. J Neuroophthalmol 1997, 17:262–265.

    PubMed  CAS  Google Scholar 

  43. Eshbaugh CG, Siatkowski RM, Smith JL, Kline LB: Simultaneous, multiple cranial neuropathies in diabetes mellitus. J Neuroophthalmol 1995, 15:219–224.

    PubMed  CAS  Google Scholar 

  44. Jacobson DM, Trobe JD: The emerging role of magnetic resonance angiography in the management of patients with third cranial nerve palsy. Am J Ophthalmol 1999, 128:94–96.

    Article  PubMed  CAS  Google Scholar 

  45. Lance JW, Zagami AS: Ophthalmoplegic migraine: a recurrent demyelinating neuropathy? Cephalalgia 2001, 21:84–89. An important review explaining the shift in our understanding of ophthalmoplegic migraine.

    Article  PubMed  CAS  Google Scholar 

  46. Tolosa EJ: Periarteritic lesions of the carotid siphon with clinical features of carotid intraclinoid aneurysms. J Neurol Neurosurg Psychiatry 1954, 17:300–302.

    PubMed  CAS  Google Scholar 

  47. Hunt WE, Meagher JN, LeFever H: Painful ophthalmoplegia: its relation to indolent inflammation of the cavernous sinus. Neurology 1961, 11:56–62.

    PubMed  CAS  Google Scholar 

  48. Smith JL, Taxdal DS: Painful ophthalmoplegia the Tolosa-Hunt syndrome. Am J Ophthalmol 1966, 61:1466–1472.

    PubMed  CAS  Google Scholar 

  49. Headache Classification Subcommittee of the International Headache Society: The International Classification of Headache Disorders, edn 2.Cephalalgia 2004, 24:supplement.

  50. Kline LB, Hoyt WF: The Tolosa-Hunt syndrome. J Neurol Neurosurg Psychiatry 2001, 71:557–582. State-of-the-art review of Tolosa-Hunt syndrome.

    Article  Google Scholar 

  51. Forderreuther S, Verlag S: The criteria of the International Headache Society for Tolosa-Hunt syndrome need to be revised. J Neurol 1999, 256:371–377.

    Article  Google Scholar 

  52. Alvarez de Arcaya A, Cerezal L, Canga A, et al.: Neuroimaging diagnosis of Tolosa-Hunt syndrome: MRI contribution. Headache 1999, 39:321–325.

    Article  Google Scholar 

  53. Cakirer S: MRI findings in Tolosa-Hunt syndrome before and after systemic corticosteroid therapy. Eur J Radiology 2003, 45:83–90.

    Article  Google Scholar 

  54. Cakirer S: MRI findings in the patients with presumptive clinical diagnosis of Tolosa-Hunt syndrome. Eur Radiol 2003, 13:17–28. An important article highlighting that most patients diagnosed with presumptive Tolosa-Hunt actually have an underlying secondary cause for their painful ophthalmolplegia.

    PubMed  Google Scholar 

  55. Kwan ES, Wolpert SM, Hedges TR 3rd: Tolosa-Hunt revisited: not necessarily a diagnosis of exclusion. Am J Radiol 1998, 150:413–418.

    Google Scholar 

  56. Pascual J, Cereaza L, Canga A, et al.: Tolosa-Hunt syndrome: focus on MRI diagnosis. Cephalalgia 1999, 19(suppl 25):36–38. This article reviews the CT and MRI findings in presumed Tolosa-Hunt syndrome.

    PubMed  Google Scholar 

  57. Leijzer CT, Prevo RL, Hageman G: Meningioma presenting as Tolosa-Hunt syndrome. Clin Neurol Neurosurg 1999, 101:19–22.

    Article  PubMed  CAS  Google Scholar 

  58. Ohta S, Nishizawa S, Namba H, et al.: Bilateral cavernous sinus actinomycosis resulting in painful ophthalmoplegia: case report. J Neurosurg 2002, 96:600–602.

    PubMed  Google Scholar 

  59. Shimizu T, Suzuki N, Ottomo M: A fungal aneurysm in a patient with presumed Tolosa-Hunt syndrome. No Shinkei Geka 1991, 19:477–483.

    PubMed  CAS  Google Scholar 

  60. Esmaeli B, Ginsberg L, Goepfert H, et al.: Squamous cell carcinoma with perineural invasion presenting as a Tolosa-Huntlike syndrome: a potential pitfall in diagnosis. Ophthal Plast Reconstr Surg 2000, 16:450–452.

    Article  PubMed  CAS  Google Scholar 

  61. Attout H, Rahmeh F, Ziegler F: Cavernous sinus lymphoma mimicking Tolosa-Hunt syndrome. Rev Med Interne 2000, 21:795–798.

    Article  PubMed  CAS  Google Scholar 

  62. Kline LB, Galbraith JG: Parasellar epidermoid presenting as painful ophthalmoplegia. J Neurosurg 1981, 54:113–117.

    Article  PubMed  CAS  Google Scholar 

  63. Leijzer CT, Prevo RL, Hageman G: Meningioma presenting as Tolosa-Hunt syndrome. Clin Neurol Neurosurg 1999, 101:19–22.

    Article  PubMed  CAS  Google Scholar 

  64. Harnett AN, Kemp EG, Fraser G: Metastatic breast cancer presenting as Tolosa-Hunt syndrome. Clin Oncol (R Coll Radiol) 1999, 11:407–409.

    CAS  Google Scholar 

  65. Ghosn J, Grazille P, Zeller V, et al.: Tolosa-Hunt syndrome revealing Burkitt lymphoma in an HIV-seropositive patient. Presse Med 2003, 32:1319–1322.

    PubMed  Google Scholar 

  66. Messmer EP, Font RL, McCrary JA, et al.: Epithelioid angiosarcoma of the orbit presenting as Tolosa-Hunt syndrome: a clinicopathologic case report with review of the literature. Ophthalmology 1983, 90:1414–1421.

    PubMed  CAS  Google Scholar 

  67. Freeman NR, Shraberg D: Alternating painful ophthalmoplegia. South Med J 1980, 73:1398–1400.

    PubMed  CAS  Google Scholar 

  68. Spector RH, Fiandaca MS: The "sinister" Tolosa-Hunt syndrome. Neurology 1986, 36:198–203.

    PubMed  CAS  Google Scholar 

  69. Maezawa M, Seki T, Imura S, et al.: Steroid-responsive painful ophthalmoplegia in childhood: sphenoid sinusitis presenting as Tolosa-Hunt syndrome. Brain Dev 1991, 13:279–282.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Mayo Clinic Scottsdale, 13400 East Shea Boulevard, 85259, Scottsdale, AZ, USA

    David W. Dodick MD

Authors
  1. Jonathan P. Gladstone MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. David W. Dodick MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Gladstone, J.P., Dodick, D.W. Painful ophthalmoplegia: Overview with a focus on tolosa-hunt syndrome. Current Science Inc 8, 321–329 (2004). https://doi.org/10.1007/s11916-004-0016-x

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11916-004-0016-x

Keywords

Search

Navigation