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Tolosa-Hunt Syndrome
Updated: Nov 05, 2018
Author: Danette C Taylor, DO, MS, FACN; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA,
FRCP, FRCPC, FRS, LRCP, MRCP, MRCS

Overview

Background
Tolosa-Hunt syndrome (THS) is a painful ophthalmoplegia caused by nonspecific inflammation of the cavernous sinus or
superior orbital fissure. In 2004, the International Headache Society provided a definition of the diagnostic criteria which
included granuloma.[1] See the image below.

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.

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

Tolosa-Hunt syndrome (THS) is uncommon in both the United States and internationally. The 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.[2] THS affects males and females equally. And,
as stated, although rare in children it is important to keep this condition in the differential diagnosis.[32]

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.

Presentation

History
See the list below:

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
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.

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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

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

DDx

Differential Diagnoses
Anisocoria

Benign Skull Tumors

Brain Metastasis

Cavernous Sinus Syndromes

Cerebral Aneurysms

Cerebral Venous Thrombosis

CNS Whipple Disease

Diabetic Neuropathy

Epidural Hematoma in Emergency Medicine

Extraocular Muscles, Actions

Extraocular Muscles, Anatomy

Lyme Disease

Meningioma

Migraine Headache

Neurosarcoidosis

Otolaryngologic Manifestations of Wegener Granulomatosis

Pediatric Craniopharyngioma

Pituitary Tumors

Polyarteritis Nodosa

Primary CNS Lymphoma

Primary Malignant Skull Tumors

Systemic Lupus Erythematosus (SLE)

Tuberculous Meningitis

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Varicella Zoster

Vascular Surgery for Arteriovenous Malformations

Workup

Workup

Laboratory Studies
The diagnosis of Tolosa-Hunt syndrome is usually one of exclusion.

CBC count, erythrocyte sedimentation rate (ESR), electrolytes with glucose, thyroid function tests, fluorescent treponemal
antibody (FTA), antinuclear antibody (ANA), lupus erythematosus (LE) preparation, antineutrophil cytoplasmic antibody
(ANCA), serum protein electrophoresis, Lyme titre, angiotensin-converting enzyme (ACE) level, and HIV titre are helpful in
eliminating other processes. This level of evaluation is required to exclude other conditions, which can have significant
morbidity associated.

Cell count and differential, protein, glucose, fungal and/or bacterial cultures, Gram stain, cytology, and opening pressure of
CSF are helpful in eliminating conditions mimicking Tolosa-Hunt syndrome; a mild (lymphocytic) pleocytosis within the spinal
fluid may occur in patients with Tolosa-Hunt syndrome.

Anti-GQ1b antibodies may be helpful in distinguishing early, painless Tolosa-Hunt syndrome from Miller Fisher syndrome.

Imaging Studies
MRI[6] of the brain and orbit with and without contrast, magnetic resonance (MR) angiography or digital subtraction
angiography (DSA), and CT scan of the brain and orbit with and without contrast may all be useful (see the images below).
Inflammatory changes in the cavernous sinus, superior orbital fissure, and/or orbital apex are typically observed on high-
resolution contrast-enhanced imaging. In the authors' experience, thin-slice high–magnetic field MRI of the cavernous sinus
region, including coronal sections with and without contrast and fat-suppressed cuts of the orbital regions, is the modality of
choice. These changes are not specific for Tolosa-Hunt syndrome and may also be present in neoplastic conditions of the
cavernous sinus. Enlargement of the optic nerve or external ocular muscles has been described, emphasizing the
continuum with idiopathic orbital inflammatory disorders.[31]

Note that findings on all imaging studies may be normal in some cases of Tolosa-Hunt syndrome.

Narrowing of the internal carotid artery within the cavernous sinus may be identified on angiography. Note that these
changes are not specific to Tolosa-Hunt syndrome.

MRI with 3-dimensional constructive interference in steady state (3D CISS) provides an enhanced picture within the
cavernous sinus. This type of imaging may assist with future diagnoses of TSH, but it is not yet used routinely.[7]

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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.

Procedures
Biopsy of the lesion may be required to confirm the diagnosis. The technical difficulty of cavernous sinus region biopsies
usually mitigates for a trial of steroids; nonetheless, biopsy may be needed to exclude neoplasm or if symptoms are
progressing, atypical, or recurrent.

Histologic Findings
Biopsy reveals nonspecific granulomatous or nongranulomatous inflammation. This is histologically indistinguishable from
the pathology of orbital pseudotumor, and these diseases may exist along a continuum.

Treatment

Medical Care
Corticosteroids are the treatment of choice, usually providing significant pain relief within 24-72 hours of therapy initiation.
Ophthalmoparesis usually requires weeks to months for resolution; indeed, ophthalmoparesis may not completely resolve in
some cases depending on the degree of inflammation and the aggressiveness of therapy. For refractory cases, azathioprine
(Imuran), methotrexate, or radiation therapy[8] has been employed.

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Surgical Care
Surgical extirpation is not generally a feasible treatment of Tolosa-Hunt syndrome; the primary value of surgical intervention
is a histopathologic diagnosis.

Consultations
Neuro-ophthalmology evaluation is helpful to confirm the diagnosis and to exclude other etiologies of presenting symptoms.
Consultation with a neurosurgeon may be useful in cases requiring biopsy.

Medication

Medication Summary
Steroids are used to treat the inflammation of Tolosa-Hunt syndrome. Pain relief usually occurs rapidly, ie, within 24-72
hours.[9] Continue treatment at the initial dose for a short time (ie, 7-10 d) after pain resolves, then taper gradually. If no
response to steroid therapy has occurred within 72 hours, the diagnosis of Tolosa-Hunt syndrome should be reevaluated.

If a patient is unable to tolerate steroid therapy, other immunosuppressive therapy may be considered.

Corticosteroids

Class Summary
Reduce pain and inflammation; diminish the size of the inflammatory mass.

Prednisone (Sterapred)
May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes
lysosomal membranes and also suppresses lymphocytes and antibody production.

Immunosuppressive agents

Class Summary
Decrease autoimmune reaction.

Methotrexate (Trexall)
Antimetabolite used to treat many autoimmune processes. The mode of action is not known; this drug does interfere with
DNA synthesis.

Azathioprine (Imuran)
Immunosuppressive agent that works primarily on T cells. Works very slowly; may require 6-12 mo of trial prior to effect. Up
to 10% of patients may have idiosyncratic reaction disallowing use. Do not allow WBC count to drop below 3000/µL or
lymphocyte count to drop below 1000/µL.

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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
See the list below:

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
See the list below:

Typically, the prognosis is considered good. Patients usually respond to corticosteroids, and spontaneous remission
can occur, although permanent ocular motor deficits may remain.

Relapse can occur in as many as 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.[10]

Patient Education
See the list below:

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.

Questions & Answers

Overview

What is Tolosa-Hunt syndrome (THS)?

What is the pathophysiology of Tolosa-Hunt syndrome (THS)?

What is the prevalence of Tolosa-Hunt syndrome (THS)?

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What is the morbidity associated with Tolosa-Hunt syndrome (THS)?

Presentation

Which clinical history findings are characteristic of Tolosa-Hunt syndrome (THS)?

Which physical findings are characteristic of Tolosa-Hunt syndrome (THS)?

What causes Tolosa-Hunt syndrome (THS)?

DDX

What are the differential diagnoses for Tolosa-Hunt Syndrome?

Workup

What is the role of lab tests in the workup of Tolosa-Hunt syndrome (THS)?

What is the role of imaging studies in the workup of Tolosa-Hunt syndrome (THS)?

What is the role of biopsy in the workup of Tolosa-Hunt syndrome (THS)?

Which histologic findings are characteristic of Tolosa-Hunt syndrome (THS)?

Treatment

How is Tolosa-Hunt syndrome (THS) treated?

What is the role of surgery in the treatment of Tolosa-Hunt syndrome (THS)?

Which specialist consultations are beneficial to patients with Tolosa-Hunt syndrome (THS)?

Medications

What is the role of steroids in the treatment of Tolosa-Hunt syndrome (THS)?

Which medications in the drug class Immunosuppressive agents are used in the treatment of Tolosa-Hunt Syndrome?

Which medications in the drug class Corticosteroids are used in the treatment of Tolosa-Hunt Syndrome?

Follow-up

What is included in long-term monitoring of Tolosa-Hunt syndrome (THS)?

What are the possible complications of Tolosa-Hunt syndrome (THS) treatment?

What is the prognosis of Tolosa-Hunt syndrome (THS)?

What is included in patient education about Tolosa-Hunt syndrome (THS)?

Contributor Information and Disclosures

Author

Danette C Taylor, DO, MS, FACN Service Chief of Neurology, Henry Ford West Bloomfield Hospital; Senior Staff
Neurologist, Henry Ford Health Systems; Clinical Assistant Professor, Department of Neurology and Ophthalmology,
Michigan State University College of Osteopathic Medicine

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

Disclosure: Nothing to disclose.

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.

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Nicholas Lorenzo, MD, MHA, CPE Co-Founder and Former Chief Publishing Officer, eMedicine and eMedicine Health,
Founding Editor-in-Chief, eMedicine Neurology; Founder and Former Chairman and CEO, Pearlsreview; Founder and
CEO/CMO, PHLT Consultants; Chief Medical Officer, MeMD Inc

Nicholas Lorenzo, MD, MHA, CPE is a member of the following medical societies: Alpha Omega Alpha, American Academy
of Neurology, American Association for Physician Leadership

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, 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 College of Human Medicine

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, North American Neuro-Ophthalmology Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: LifeBlood<br/>Serve(d) as a
speaker or a member of a speakers bureau for: Biogen; Genzyme; Novartis; Teva; Mallinckrodt<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.

Florian P Thomas, MD, PhD, MA, MS Chair, Neuroscience Institute and Department of Neurology, Director, National MS
Society Multiple Sclerosis Center and Hereditary Neuropathy Foundation Center of Excellence, Hackensack University
Medical Center; Founding Chair and Professor, Department of Neurology, Hackensack Meridian School of Medicine at
Seton Hall University; Professor Emeritus, Department of Neurology, St Louis University School of Medicine; Editor-in-Chief,
Journal of Spinal Cord Medicine

Florian P Thomas, MD, PhD, 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.

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.

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