Tolosa-Hunt Syndrome 

  • Author: Danette C Taylor, DO, MS; Chief Editor: B Mark Keegan, MD, FRCPC   more...
 
Updated: Apr 29, 2010
 

Background

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

MRI of a 40-year-old man with severe periorbital pMRI of a 40-year-old man with severe periorbital pain ocular sinister (OS; ie, left eye), complete oculomotor nerve palsy OS, and partial abducens nerve palsy OS. Axial imaging without (left) and with (right) enhancement demonstrates nonspecific fullness involving the left cavernous sinus, consistent with Tolosa-Hunt syndrome within the context of the history. Treatment with steroids produced complete resolution of symptoms. Image courtesy of Eric Eggenberger, DO.
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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.

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Epidemiology

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]

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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

Specialty Editor Board

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.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

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; 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, St Louis University School of Medicine

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.

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: Novartis Consulting fee Consulting

References

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  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].

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  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].

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  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].

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  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].

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MRI of a 40-year-old man with severe periorbital pain ocular sinister (OS; ie, left eye), complete oculomotor nerve palsy OS, and partial abducens nerve palsy OS. Axial imaging without (left) and with (right) enhancement demonstrates nonspecific fullness involving the left cavernous sinus, consistent with Tolosa-Hunt syndrome within the context of the history. Treatment with steroids produced complete resolution of symptoms. Image courtesy of Eric Eggenberger, DO.
Coronal T1-weighted MRI with (below) and without (above) enhancement demonstrates left cavernous sinus fullness consistent with Tolosa-Hunt syndrome (THS). The imaging features are nonspecific and must be placed into the context of the history, examination, and clinical course to avoid misdiagnosis of infiltrating, infectious, or neoplastic cavernous sinus processes. Image courtesy of Eric Eggenberger, DO.
 
 
 
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