eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases
Tolosa-Hunt Syndrome
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).
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Overview: Tolosa-Hunt Syndrome |
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References
Zanus C, Furlan C, Costa P, Cosentini D, Carrozzi M. The Tolosa-Hunt syndrome in children: a case report. Cephalalgia. 2009;29:1232-1237.
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].
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].
[Guideline] The International Classification of Headache Disorders: 2nd edition. Cephalalgia. 2004;Suppl 1:9-160. [Medline]. [Full Text].
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].
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].
Cohn DF, Carasso R, Streifler M. Painful ophthalmoplegia: the Tolosa-Hunt syndrome. Eur Neurol. 1979;18(6):373-81. [Medline].
Gimenez-Roldan S, Guillem A, Munoz L. [Long-term risk of relapses in Tolosa-Hunt syndrome]. Neurologia. Sep 2006;21(7):382-5. [Medline].
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].
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].
Hunt WE. Tolosa-Hunt syndrome: one cause of painful ophthalmoplegia. J Neurosurg. May 1976;44(5):544-9. [Medline].
Johnston JL. Parasellar syndromes. Curr Neurol Neurosci Rep. Sep 2002;2(5):423-31. [Medline].
Kline LB. The Tolosa-Hunt syndrome. Surv Ophthalmol. Sep-Oct 1982;27(2):79-95. [Medline].
Kline LB, Hoyt WF. The Tolosa-Hunt syndrome. J Neurol Neurosurg Psychiatry. Nov 2001;71(5):577-82. [Medline].
Kobor J, Voros E, Deak A. Magnetic resonance imaging in Tolosa-Hunt syndrome. Eur J Pediatr. Dec 2004;163(12):753-4. [Medline].
Kwan ESK, Wolpert SM, Hedges TR III. Tolosa-Hunt revisited: Not necessarily a diagnosis of exclusion. Am J Radiol. 1989;71:932.
La Mantia L, Erbetta A, Bussone G. Painful ophthalmoplegia: an unresolved clinical problem. Neurol Sci. May 2005;26 Suppl 2:s79-82. [Medline].
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].
Roca PD. Painful ophthalmoplegia: the Tolosa-Hunt syndrome. Ann Ophthalmol. Jun 1975;7(6):828-34. [Medline].
Schutta HS. Diseases of the dura mater. In: Joynt R, Griggs R, eds. Clinical Neurology. Philadelphia, Pa: Lippincott, Williams & Wilkins; 1993:34-44.
Smith JL, Taxdal DS. Painful ophthalmoplegia. The Tolosa-Hunt syndrome. Am J Ophthalmol. Jun 1966;61(6):1466-72. [Medline].
Sondheimer FK, Knapp J. Angiographic findings in the Tolosa-Hunt syndrome: painful ophthalmoplegia. Radiology. Jan 1973;106(1):105-12. [Medline].
Spector RH, Fiandaca MS. The "sinister" Tolosa-Hunt syndrome. Neurology. Feb 1986;36(2):198-203. [Medline].
Troost BT. Miller NR, Newman NJ, eds. Walsh & Hoyt's Clinical Neuro-Ophthalmology. Philadelphia, Pa: Williams & Wilkins Company; 1998:1727-29.
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].
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].
Further Reading
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
Overview: Tolosa-Hunt Syndrome