eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases

Tolosa-Hunt Syndrome

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

Updated: Apr 29, 2010

Introduction

Background

Tolosa-Hunt syndrome (THS) is a painful ophthalmoplegia caused by nonspecific inflammation of the cavernous sinus or superior orbital fissure.

Pathophysiology

Nonspecific inflammation (noncaseating granulomatous or nongranulomatous) within the cavernous sinus or superior orbital fissure is the cause of the constant pain, which characterizes the onset of this disorder. Ophthalmoparesis or disordered eye movements occur when cranial nerves III, IV, and VI are damaged by granulomatous inflammation. Pupillary dysfunction may be present and is related to injury to the sympathetic fibers in the cavernous portion of ICA or parasympathetic fibers that surround the oculomotor nerve. Trigeminal nerve involvement (primarily V1) may cause paresthesias of the forehead. Pathological involvement beyond the cavernous sinus, superior orbital fissure, or apex of the orbit occurs rarely, and the disorder is part of a continuum with idiopathic orbital pseudotumor, with which it shares histopathologic features. Spontaneous remissions can occur; relapses may occur in up to 40% of the patients.

Frequency

United States

This disorder is uncommon in both the United States and internationally.

Mortality/Morbidity

Tolosa-Hunt syndrome is not a fatal disorder; patients experience unilateral onset of acute orbital pain and ophthalmoparesis, and the disorder may threaten sight if untreated inflammation extends beyond the cavernous sinus to affect the optic nerve.

Sex

Males and females are equally affected.

Age

This disorder is rare during the first 2 decades of life; in people older than 20 years, it appears to have an even distribution. When THS occurs in children, the course of the disorder appears to be similar to that experienced by adults.1

Clinical

History

  • Patients present with usually severe retro-orbital or periorbital pain of acute onset. This pain may be described as constant and "boring" in nature.
  • Diplopia related to ophthalmoparesis follows the onset of pain (in rare cases, the ophthalmoparesis precedes the pain, sometimes by several days).
  • Patients may report visual loss. This is noted if the inflammation extends into the orbit to affect the optic nerve, and it is not a factor in disease limited to the cavernous sinus.
  • Paresthesias along the forehead may be described if the first division of the trigeminal nerve is involved.
  • Tolosa-Hunt syndrome is most often unilateral, although bilateral cases have been described.
  • Tolosa-Hunt syndrome frequently mimics other conditions; a single characteristic that is pathognomonic for this process does not exist. As such, realizing that this is a diagnosis of exclusion becomes even more important. Many of the processes that are found within the differential diagnosis of Tolosa-Hunt syndrome can have significant associated morbidity if not diagnosed and treated appropriately.

Physical

  • Painful ophthalmoparesis or ophthalmoplegia is the hallmark of this syndrome.
  • In addition to the optic and trigeminal nerves (V1, rarely V2 distribution), any of the ocular motor nerves may be involved. The oculomotor and abducens nerves are most commonly affected. Evidence of incomplete third nerve palsy with or without pupillary sparing may be present. Conversely, inflammatory involvement of the sympathetic nerves passing through the interior of the cavernous sinus may produce Horner syndrome with miosis. The combination of unilateral oculomotor palsy and Horner syndrome increases the localization specificity for the cavernous sinus.
  • Ptosis may be observed related to oculomotor palsy. Lid swelling is more likely to occur with orbital disease rather than inflammation limited to the cavernous sinus. These changes have been misdiagnosed as a complication of sinusitis, as reported by Lachanas et al.2
  • Mild proptosis and/or optic disc edema may be noted if the orbit is involved.
  • Evidence of trigeminal nerve involvement is suggested by loss of the ipsilateral corneal reflex.
  • The International Headache Society criteria for Tolosa-Hunt syndrome3,4 include the following:
    • Episode(s) of unilateral orbital pain for an average of 8 weeks if left untreated
    • Associated paresis of the third, forth, or sixth cranial nerves, which may coincide with onset of pain or follow it by a period of up to 2 weeks
    • Pain that is relieved within 72 hours of steroid therapy initiation
    • Exclusion of other conditions by neuroimaging and (not compulsory) angiography

Causes

The cause of Tolosa-Hunt syndrome is unknown (idiopathic).

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. Zanus C, Furlan C, Costa P, Cosentini D, Carrozzi M. The Tolosa-Hunt syndrome in children: a case report. Cephalalgia. 2009;29:1232-1237.

  2. 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].

  3. 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].

  4. [Guideline] The International Classification of Headache Disorders: 2nd edition. Cephalalgia. 2004;Suppl 1:9-160. [Medline]. [Full Text].

  5. 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].

  6. 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].

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

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

  9. 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].

  10. 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].

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

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

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

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

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

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

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

  18. 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].

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

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

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

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

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

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

  25. 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].

  26. 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 Assistant Professor, Department of Neurology, Michigan State University College of Osteopathic Medicine; Senior Staff Neurologist, Henry Ford Health Systems
Danette C Taylor, DO, MS is a member of the following medical societies: American Academy of Neurology, American Medical Association, American Osteopathic Association, and Movement Disorders Society
Disclosure: Allergan Honoraria Speaking and teaching; Boeringher Ingelheim Honoraria Speaking and teaching; Teva  Honoraria Speaking and teaching; Ipsen 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: eMedicine Salary Employment

Managing Editor

Florian P Thomas, MD, MA, PhD, Drmed, Director, Spinal Cord Injury Unit, Saint Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Director, Neuropathy Association Center of Excellence, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, Saint Louis University
Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Paraplegia Society, Consortium of Multiple Sclerosis Centers, 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: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Ortho McNeil Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace  Speaking, consulting

Chief Editor

B Mark Keegan, MD, FRCPC, Assistant Professor of Neurology, College of Medicine, Mayo Clinic; Master's Faculty, Mayo Graduate School; Consultant, Department of Neurology, Mayo Clinic, Rochester
B Mark Keegan, MD, FRCPC is a member of the following medical societies: American Academy of Neurology, American Medical Association, and Minnesota Medical Association
Disclosure: Neurology (Journal of the American Academy of Neurology)  Honoraria Section Editor

 
 
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.