eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases

Tolosa-Hunt Syndrome: Follow-up

Author: Danette C Taylor, DO, MS, Clinical Associate Professor, Department of Neurology, Michigan State University College of Osteopathic Medicine; Senior Staff Neurologist, Franklin Pointe Medical Center, Henry Ford Health Systems
Contributor Information and Disclosures

Updated: Mar 12, 2009

Follow-up

Further Outpatient Care

Supervise a tapering schedule for the steroids and monitor for steroid-related adverse effects. Because the diagnosis of Tolosa-Hunt syndrome is often made clinically without histopathologic confirmation, vigilance must be maintained for the possibility of alternative masquerading diagnosis.

Complications

  • Complications of high-dose and/or prolonged steroid use are common.
  • In patients with extracavernous sinus involvement affecting the optic nerve, loss of vision may occur.

Prognosis

  • Typically, the prognosis is considered good. Patients usually respond to corticosteroids, and spontaneous remission can occur, although permanent ocular motor deficits may remain.
  • Relapse occurs in 30-40% of patients successfully treated for Tolosa-Hunt syndrome. This typically occurs on the same side as the original lesion but can be observed on the opposite side. Spontaneous remission can occur; patients who have experienced spontaneous remission appear to have as much risk of reoccurrence as those treated with medication. Gimenez-Roldan et al have reported that relapses may occur as long as 13 years after initial diagnosis and treatment.6

Patient Education

  • Patients should understand that this is an idiopathic condition that is usually self-limited. Relapses may occur (30-40% of patients may experience relapse), and patients should know that the course of any relapse often follows the original event but may require additional testing. Patients should have an idea of the differential diagnosis of Tolosa-Hunt syndrome and report any new symptoms or side effects from treatment to the physician.
  • The risks associated with the use of high-dose steroids should be emphasized prior to the onset of treatment.

Miscellaneous

Medicolegal Pitfalls

  • Tolosa-Hunt syndrome is usually a diagnosis of exclusion, typically made in the absence of histopathologic verification. As such, other causes of orbital pain and/or ophthalmoparesis that potentially threaten the patient's vision or are potentially fatal should be fully evaluated prior to making this diagnosis; keep these other conditions in mind through prolonged follow-up (eg, patients with posterior communicating artery aneurysms often present with painful ophthalmoplegia [third nerve palsy pattern]). A response to steroids with resolution of pain is not diagnostic of Tolosa-Hunt syndrome; other pathophysiologies may respond to steroids, at least transiently, including infections and neoplasms. Lack of response to steroids within 72 hours should prompt additional evaluation for other causes of symptoms.
  • The use of steroids and possible associated complications may be a source of medicolegal difficulty.
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Kenneth A Mankowski, DO to the development and writing of this article.



More on Tolosa-Hunt Syndrome

Overview: Tolosa-Hunt Syndrome
Differential Diagnoses & Workup: Tolosa-Hunt Syndrome
Treatment & Medication: Tolosa-Hunt Syndrome
Follow-up: Tolosa-Hunt Syndrome
Multimedia: Tolosa-Hunt Syndrome
References
[ CLOSE WINDOW ]

References

  1. Lachanas VA, Karatzias GT, Tsitiridis I, Panaras I, Sandris VG. Tolosa-Hunt syndrome misdiagnosed as sinusitis complication. J Laryngol Otol. Jan 2008;122(1):97-9. [Medline].

  2. Colnaghi S, Versino M, Marchioni E, Pichiecchio A, Bastianello S, Cosi V, et al. ICHD-II diagnostic criteria for Tolosa-Hunt syndrome in idiopathic inflammatory syndromes of the orbit and/or the cavernous sinus. Cephalalgia. Jun 2008;28(6):577-84. [Medline].

  3. Goto Y, Hosokawa S, Goto I, et al. Abnormality in the cavernous sinus in three patients with Tolosa-Hunt syndrome: MRI and CT findings. J Neurol Neurosurg Psychiatry. Mar 1990;53(3):231-4. [Medline].

  4. Yagi A, Sato N, Taketomi A, et al. Normal cranial nerves in the cavernous sinuses: contrast-enhanced three-dimensional constructive interference in the steady state MR imaging. AJNR Am J Neuroradiol. Apr 2005;26(4):946-50. [Medline].

  5. Cohn DF, Carasso R, Streifler M. Painful ophthalmoplegia: the Tolosa-Hunt syndrome. Eur Neurol. 1979;18(6):373-81. [Medline].

  6. Gimenez-Roldan S, Guillem A, Munoz L. [Long-term risk of relapses in Tolosa-Hunt syndrome]. Neurologia. Sep 2006;21(7):382-5. [Medline].

  7. Barontini F, Maurri S, Marrapodi E. Tolosa-Hunt syndrome versus recurrent cranial neuropathy. Report of two cases with a prolonged follow-up. J Neurol. Feb 1987;234(2):112-5. [Medline].

  8. Bruyn GW, Ferrari M, de Beer FC. Migraine, Tolosa-Hunt syndrome and pleocytosis. Correlation or coincidence?. Clin Neurol Neurosurg. 1984;86(1):33-41. [Medline].

  9. Hunt WE. Tolosa-Hunt syndrome: one cause of painful ophthalmoplegia. J Neurosurg. May 1976;44(5):544-9. [Medline].

  10. Johnston JL. Parasellar syndromes. Curr Neurol Neurosci Rep. Sep 2002;2(5):423-31. [Medline].

  11. Kline LB. The Tolosa-Hunt syndrome. Surv Ophthalmol. Sep-Oct 1982;27(2):79-95. [Medline].

  12. Kline LB, Hoyt WF. The Tolosa-Hunt syndrome. J Neurol Neurosurg Psychiatry. Nov 2001;71(5):577-82. [Medline].

  13. Kobor J, Voros E, Deak A. Magnetic resonance imaging in Tolosa-Hunt syndrome. Eur J Pediatr. Dec 2004;163(12):753-4. [Medline].

  14. Kwan ESK, Wolpert SM, Hedges TR III. Tolosa-Hunt revisited: Not necessarily a diagnosis of exclusion. Am J Radiol. 1989;71:932.

  15. La Mantia L, Erbetta A, Bussone G. Painful ophthalmoplegia: an unresolved clinical problem. Neurol Sci. May 2005;26 Suppl 2:s79-82. [Medline].

  16. Lo YL, Chan LL, Pan A, Ratnagopal P. Acute ophthalmoparesis in the anti-GQ1b antibody syndrome: electrophysiological evidence of neuromuscular transmission defect in the orbicularis oculi. J Neurol Neurosurg Psychiatry. Mar 2004;75(3):436-40. [Medline].

  17. Roca PD. Painful ophthalmoplegia: the Tolosa-Hunt syndrome. Ann Ophthalmol. Jun 1975;7(6):828-34. [Medline].

  18. Schutta HS. Diseases of the dura mater. In: Joynt R, Griggs R, eds. Clinical Neurology. Philadelphia, Pa: Lippincott, Williams & Wilkins; 1993:34-44.

  19. Smith JL, Taxdal DS. Painful ophthalmoplegia. The Tolosa-Hunt syndrome. Am J Ophthalmol. Jun 1966;61(6):1466-72. [Medline].

  20. Sondheimer FK, Knapp J. Angiographic findings in the Tolosa-Hunt syndrome: painful ophthalmoplegia. Radiology. Jan 1973;106(1):105-12. [Medline].

  21. Spector RH, Fiandaca MS. The "sinister" Tolosa-Hunt syndrome. Neurology. Feb 1986;36(2):198-203. [Medline].

  22. Troost BT. Miller NR, Newman NJ, eds. Walsh & Hoyt's Clinical Neuro-Ophthalmology. Philadelphia, Pa: Williams & Wilkins Company; 1998:1727-29.

  23. Vallat JM, Vallat M, Julien J, et al. Painful ophthalmoplegia (Tolosa-Hunt) accompanied by peripheral facial paralysis. Ann Neurol. Dec 1980;8(6):645. [Medline].

  24. Yousem DM, Atlas SW, Grossman RI, et al. MR imaging of Tolosa-Hunt syndrome. AJR Am J Roentgenol. Jan 1990;154(1):167-70. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

Tolosa-Hunt syndrome, THS, painful ophthalmoplegia, idiopathic cavernous sinus inflammation, inflammation of the superior orbital fissure, painful ophthalmoparesis, inflammation of the cavernous sinus fissure

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Danette C Taylor, DO, MS, Clinical Associate Professor, Department of Neurology, Michigan State University College of Osteopathic Medicine; Senior Staff Neurologist, Franklin Pointe Medical Center, Henry Ford Health Systems
Danette C Taylor, DO, MS is a member of the following medical societies: American Academy of Neurology, American Medical Association, and American Osteopathic Association
Disclosure: GlaxoSmithKline Honoraria Speaking and teaching; Boeringher Ingelheim Honoraria Speaking and teaching; Teva  Honoraria Speaking and teaching

Medical Editor

Eric R Eggenberger, DO, MS, FAAN, Professor, Vice-Chairman, Department of Neurology and Ophthalmology, Colleges of Osteopathic Medicine and Human Medicine, Michigan State University; Director of Michigan State University Ocular Motility Laboratory; Director of National Multiple Sclerosis Society Clinic, Michigan State University
Eric R Eggenberger, DO, MS, FAAN is a member of the following medical societies: American Academy of Neurology, American Academy of Ophthalmology, American Osteopathic Association, and North American Neuro-Ophthalmology Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Florian P Thomas, MD, MA, PhD, Drmed, Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University
Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Paraplegia Society, and National Multiple Sclerosis Society
Disclosure: Nothing to disclose.

CME Editor

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: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.