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Tolosa-Hunt Syndrome Clinical Presentation

  • Author: Danette C Taylor, MS, DO, FACN; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
 
Updated: Sep 04, 2015
 

History

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  • 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.
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Physical

See the list below:

  • 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.[3]
  • 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 syndrome[4, 5] 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
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Causes

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

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Contributor Information and Disclosures
Author

Danette C Taylor, MS, DO, FACN Medical Director, Movement Disorders Program, Beaumont Health; Clinical Assistant Professor, Department of Neurology and Ophthalmology, Michigan State University College of Osteopathic Medicine

Danette C Taylor, MS, DO, FACN is a member of the following medical societies: American Academy of Neurology, American Osteopathic Association, International Parkinson and Movement Disorder Society, American College of Osteopathic Neurologists and Psychiatrists, American Medical Association

Disclosure: Received honoraria from Allergan for speaking and teaching; Received honoraria from Teva Pharmaceuticals for speaking and teaching.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Florian P Thomas, MD, PhD, Drmed, MA, MS Director, National MS Society Multiple Sclerosis Center; Professor and Director, Clinical Research Unit, Department of Neurology, Adjunct Professor of Physical Therapy, Associate Professor, Institute for Molecular Virology, St Louis University School of Medicine; Editor-in-Chief, Journal of Spinal Cord Medicine

Florian P Thomas, MD, PhD, Drmed, MA, MS is a member of the following medical societies: Academy of Spinal Cord Injury Professionals, American Academy of Neurology, American Neurological Association, Consortium of Multiple Sclerosis Centers, National Multiple Sclerosis Society, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS Professor Emeritus of Neurology and Psychiatry, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Neuroscience Director, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS is a member of the following medical societies: American College of International Physicians, American Heart Association, American Stroke Association, American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners Institute, National Association of Managed Care Physicians, American College of Physicians, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, Royal Society of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Eric R Eggenberger, MS, DO, 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 College of Human Medicine

Eric R Eggenberger, MS, DO, FAAN is a member of the following medical societies: American Academy of Neurology, American Academy of Ophthalmology, American Osteopathic Association, North American Neuro-Ophthalmology Society

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Biogen; Genzyme; Novartis; Teva <br/>Received research grant from: Biogen; Genzyme; Novartis<br/>Received consulting fee from Biogen for consulting; Received consulting fee from Teva for consulting; Received consulting fee from Acorda for consulting; Received grant/research funds from Novartis for independent contractor; Received honoraria from Genentech for speaking and teaching; Received honoraria from Genzyme for speaking and teaching.

Acknowledgements

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

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