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TOPIC OUTLINE
Tolosa-Hunt syndrome
INTRODUCTION
Author Section Editor Deputy Editor
PATHOGENESIS
Kenneth S Shindler, Paul W Brazis, MD Janet L Wilterdink,
CLINICAL FEATURES MD, PhD MD
DIFFERENTIAL DIAGNOSIS
Disclosures
DIAGNOSTIC EVALUATION
Magnetic resonance imaging
Blood and CSF evaluation Last literature review version 19.3: Fri Sep 30
00:00:00 GMT 2011 | This topic last updated: Mon Jun
Response to glucocorticoids and
06 00:00:00 GMT 2011 (More)
further investigation
TREATMENT INTRODUCTION The Tolosa-Hunt syndrome is a rare
Glucocorticoids syndrome with an estimated annual incidence of one case
Follow-up per million per year [1]. It is characterized by painful
Second-line treatments ophthalmoplegia (weakness of the eye muscles) and is
caused by an idiopathic granulomatous inflammation of the
PROGNOSIS
cavernous sinus. This syndrome was first described in
SUMMARY AND 1954, and its exquisite responsiveness to glucocorticoid
RECOMMENDATIONS
treatment was recognized a few years later [2-4].
REFERENCES
While considered a benign condition, permanent neurologic
GRAPHICSView All deficits can occur, and relapses are common, often
TABLES requiring prolonged immunosuppressive therapy.
Causes painful ophthalmoplegia Tolosa-Hunt syndrome must be carefully differentiated from
more malignant diagnoses, a mandate challenged by the
RELATED TOPICS lack of a specific diagnostic test abnormality.
Clinical manifestations and
PATHOGENESIS The Tolosa-Hunt syndrome is caused
diagnosis of granulomatosis with
polyangiitis (Wegener's) and by an inflammatory process of unknown etiology. On
microscopic polyangiitis histopathology, there is a nonspecific inflammation of the
septa and wall of the cavernous sinus, with a lymphocyte
Horner's syndrome
and plasma cell infiltration, giant cell granulomas, and
Neurologic sarcoidosis
proliferation of fibroblasts [2,3]. The inflammation produces
pressure and secondary dysfunction of the structures within
the cavernous sinus, including cranial nerves III, IV, and
VI, as well as the superior divisions of cranial nerve V.

While reports of intracranial extension of the inflammation

exist [1], there are no reports of systemic involvement.
Cases of Tolosa-Hunt syndrome have been reported in
patients with other inflammatory disorders, such as
systemic lupus erythematosus, but this may simply
represent an association of the two autoimmune conditions
[5]. Cases of orbital inflammation may be the initial
presentation of systemic inflammatory disorders such as
sarcoidosis and Wegener's granulomatosis (table 1). (See
"Neurologic sarcoidosis" and "Clinical manifestations and
diagnosis of granulomatosis with polyangiitis (Wegener's)
and microscopic polyangiitis".)
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TOPIC OUTLINE CLINICAL FEATURES The Headache Classification

Subcommittee of the International Headache Society
INTRODUCTION
describes the course and features of Tolosa-Hunt syndrome
PATHOGENESIS as "episodic orbital pain associated with paralysis of one or
CLINICAL FEATURES more of the third, fourth, and/or sixth cranial nerves which
DIFFERENTIAL DIAGNOSIS usually resolves spontaneously but tends to relapse and
DIAGNOSTIC EVALUATION remit" [6].
Magnetic resonance imaging Patients may present at any age, from the first through the
Blood and CSF evaluation eighth decade of life [7,8]. Men and women are affected at
Response to glucocorticoids and the same frequency.
further investigation
TREATMENT Patients report a constant pain behind the eye that may
begin several days (up to two weeks) prior to the
Glucocorticoids
ophthalmoplegia, or the pain may start after the cranial
Follow-up
neuropathy [7]. The pain is characteristically described as a
Second-line treatments
steady gnawing or boring pain. Tolosa-Hunt syndrome is
PROGNOSIS
typically unilateral; bilateral symptoms occur in 4 to 5
SUMMARY AND percent of cases [9,10].
RECOMMENDATIONS
REFERENCES Diplopia results from cranial mono- or polyneuropathy.
Involvement of cranial nerve III is reported most frequently
GRAPHICSView All (85 percent), followed by cranial nerve VI (70 percent), the
TABLES ophthalmic division of cranial nerve V (30 percent), and
Causes painful ophthalmoplegia cranial nerve IV (29 percent) [9]. Involvement of
periarterial sympathetic fibers causes a third order Horner's
RELATED TOPICS syndrome in approximately 20 percent of patients [10,11].
Clinical manifestations and (See "Horner's syndrome".)
diagnosis of granulomatosis with
In conjunction with ophthalmoplegia, the maxillary and
polyangiitis (Wegener's) and
mandibular divisions of the fifth nerve, the optic nerve, and
microscopic polyangiitis
the facial nerve have also been affected in individual cases,
Horner's syndrome
suggesting that inflammation extends beyond the
Neurologic sarcoidosis cavernous sinus in rare cases [3,4,9,12-15]. Involvement
of the optic nerve occurs at the orbital apex and may cause
optic disc edema or pallor [7]. Loss of visual acuity is
uncommon but may occur unpredictably and may be
permanent.

Left untreated, symptoms of Tolosa-Hunt syndrome may

resolve spontaneously after an average of about eight
weeks [3,7].

DIFFERENTIAL DIAGNOSIS Most patients (greater

than 75 percent) who present with painful ophthalmoplegia
will not have Tolosa-Hunt syndrome [16,17]. The syndrome
of painful ophthalmoplegia may be caused by any process
exerting a mass effect on the cavernous sinus (table 1).
These include a primary intracranial tumor, lymphoma or
other local or distant metastatic tumors, aneurysm,
carotid-cavernous fistula, carotid dissection, cavernous
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cranial nerve palsy.
TOPIC OUTLINE
Orbital pseudotumor is a related condition of idiopathic
INTRODUCTION inflammation involving the orbit [18]. Patients present with
PATHOGENESIS painful ophthalmoplegia and orbital signs (proptosis,
CLINICAL FEATURES conjunctival injection, and chemosis). This condition, which
DIFFERENTIAL DIAGNOSIS is also glucocorticoid responsive, may be identical to
Tolosa-Hunt syndrome, distinguished only by a different
DIAGNOSTIC EVALUATION
anatomic localization.
Magnetic resonance imaging
Blood and CSF evaluation In general, it is difficult to exclude alternative diagnoses
Response to glucocorticoids and clinically. Authors note that either a sudden or a gradual
further investigation onset of symptoms can be observed in patients with
TREATMENT aneurysm, tumor, as well as Tolosa-Hunt syndrome [7].
Glucocorticoids Other clinical signs may be suggestive but are not
Follow-up completely reliable. Signs of orbital congestion (proptosis,
Second-line treatments conjunctival injection, and chemosis) usually accompany
cavernous sinus thrombosis and diseases involving the
PROGNOSIS
orbit. Diabetic ophthalmoplegia usually results from a
SUMMARY AND
mononeuropathy rather than a polyneuropathy. Fever
RECOMMENDATIONS
suggests infection but may not be present in patients with
REFERENCES more indolent pathogens.
GRAPHICSView All DIAGNOSTIC EVALUATION The diagnosis of
TABLES Tolosa-Hunt syndrome is based upon the clinical
Causes painful ophthalmoplegia presentation in conjunction with neuroimaging results and
a clinical response to corticosteroids. Laboratory tests and
RELATED TOPICS lumbar puncture are also recommended. Direct tissue
Clinical manifestations and biopsy is rarely performed due to the technically difficult
diagnosis of granulomatosis with and potentially harmful approach to the cavernous sinus.
polyangiitis (Wegener's) and
microscopic polyangiitis The specific diagnostic criteria recommended by the
International Headache Society are summarized [6]:
Horner's syndrome
Neurologic sarcoidosis One or more episodes of unilateral orbital pain lasting
for weeks (untreated)

Third, fourth, and/or sixth cranial nerve palsy and/or

granuloma detected by magnetic resonance imaging
(MRI) or biopsy

Cranial nerve palsy begins within two weeks of onset

of orbital pain

Symptoms resolve within 72 hours when treated with

sufficient corticosteroids

Other etiologies are excluded by appropriate

investigation

Magnetic resonance imaging A contrast-enhanced

MRI is essential to the diagnostic evaluation of a patient
with
Help painful
improveophthalmoplegia, primarily
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tissue that is usually isointense to gray matter on T1
TOPIC OUTLINE and iso- or hypointense on T2, and strongly enhances
with gadolinium
INTRODUCTION
PATHOGENESIS Abnormal convexity of the wall of the cavernous sinus
CLINICAL FEATURES
Focal narrowing of the intracavernous internal carotid
DIFFERENTIAL DIAGNOSIS artery
DIAGNOSTIC EVALUATION
Magnetic resonance imaging In rare cases of Tolosa-Hunt syndrome, the MRI is normal
Blood and CSF evaluation [23]. Computed tomography (CT) may also show
Response to glucocorticoids and cavernous sinus signal changes in Tolosa-Hunt syndrome,
further investigation but is less sensitive than MRI [11,19,24,25].
TREATMENT These MRI findings are not specific to Tolosa-Hunt
Glucocorticoids syndrome. Similar findings are seen with other etiologies,
Follow-up such as lymphoma, sarcoidosis, and meningioma [7]. MRI
Second-line treatments findings in some of these disorders may also respond to
PROGNOSIS glucocorticoid therapy.
SUMMARY AND
Blood and CSF evaluation When the MRI is normal or
RECOMMENDATIONS
shows changes consistent with cavernous sinus
REFERENCES inflammation, further evaluation should include blood and
GRAPHICSView All
cerebrospinal fluid (CSF) testing to exclude other possible
causes of orbital inflammation [7]. Recommended blood
TABLES
testing includes:
Causes painful ophthalmoplegia
Complete blood count
RELATED TOPICS Electrolytes
Clinical manifestations and Glucose and hemoglobin A1C
diagnosis of granulomatosis with Renal and liver function tests
polyangiitis (Wegener's) and Angiotensin converting enzyme
microscopic polyangiitis Antinuclear antibody
Horner's syndrome Anti-dsDNA antibody
Neurologic sarcoidosis Anti-Sm antibody
Antinuclear cytoplasmic antibody
Fluorescent treponemal antibody test
Lyme serologies
Serum protein electrophoresis
Erythrocyte sedimentation rate (ESR)
C reactive protein

In patients with Tolosa-Hunt syndrome, these results are

generally normal [10]. Although case reports of elevated
ESR, mild leukocytosis, and antinuclear antibody
concentrations have been reported in Tolosa-Hunt
syndrome, these should suggest an underlying connective
tissue disorder.

Cerebrospinal fluid should also be evaluated for protein,

glucose, cell count with differential, cytology, Lyme and
syphilis serology, angiotensin converting enzyme (for
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infectious meningitis).
TOPIC OUTLINE
Response to glucocorticoids and further
INTRODUCTION investigation Glucocorticoid administration has
PATHOGENESIS diagnostic as well as therapeutic utility. Rapid resolution of
CLINICAL FEATURES pain (within 24 to 72 hours) helps to confirm suspected
DIFFERENTIAL DIAGNOSIS Tolosa-Hunt syndrome [4,8]. Improvement of cranial nerve
deficits and regression of MRI abnormalities over the next
DIAGNOSTIC EVALUATION
two to eight weeks provide further confirmation of the
Magnetic resonance imaging
diagnosis [10,26]. However, an initial clinical or MRI
Blood and CSF evaluation
response to steroids is not diagnostic; other entities, such
Response to glucocorticoids and
as lymphoma and vasculitis, may also respond clinically
further investigation
and radiographically to glucocorticoid therapy [15].
TREATMENT
Glucocorticoids The clinician needs to be vigilant regarding the continued
Follow-up possibility of misdiagnosis of Tolosa-Hunt syndrome (see
Second-line treatments 'Follow-up' below). More aggressive testing, including
repeat CSF evaluation and consideration of surgical biopsy,
PROGNOSIS
is recommended if symptoms do not respond promptly to
SUMMARY AND
steroids, or if glucocorticoid efficacy is lost after an initial
RECOMMENDATIONS
response.
REFERENCES
Even with careful adherence to clinical criteria and
GRAPHICSView All diagnostic evaluation, misdiagnosis still occurs. As an
TABLES example, a case of painful ophthalmoplegia with
Causes painful ophthalmoplegia characteristic clinical and MRI findings, both initially
glucocorticoid responsive, was subsequently demonstrated
RELATED TOPICS to be caused by actinomycosis infection [27]. An underlying
Clinical manifestations and neoplasm was finally diagnosed in other patients who either
diagnosis of granulomatosis with failed to respond to initial glucocorticoid therapy or did not
polyangiitis (Wegener's) and maintain a clinical response [10,28-30].
microscopic polyangiitis
TREATMENT
Horner's syndrome
Neurologic sarcoidosis Glucocorticoids Glucocorticoids have been the
recommended treatment for Tolosa-Hunt syndrome since
the 1960s [3,4]. However, there are little data other than
case series to determine the most effective dose, route and
schedule of administration, or length of glucocorticoid
therapy [7]. While glucocorticoids clearly hasten the
resolution of orbital pain, there is no definitive evidence
that the cranial neuropathies recover any faster with or
without treatment. Little consideration has been given to
alternative therapies, probably due to the typical rapid
response to glucocorticoids.

Specific glucocorticoid regimens reported for treatment of

Tolosa-Hunt syndrome vary, but in general they include
initial high-dose glucocorticoids for two to four weeks
followed by a gradual taper over the course of at least four
to six weeks and up to several months [10,31,32]. Prompt
administration of IV glucocorticoids is often recommended,
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A suggested glucocorticoid regimen is:

TOPIC OUTLINE

INTRODUCTION Prednisone 80 to 100 mg daily for three days.

PATHOGENESIS
If the pain has resolved, taper to 60 mg daily, then
CLINICAL FEATURES 40 mg, then 20 mg, then 10 mg every two weeks.
DIFFERENTIAL DIAGNOSIS
DIAGNOSTIC EVALUATION Follow-up Close clinical follow-up with repeat MRI is
Magnetic resonance imaging necessary to be sure the glucocorticoid treatment remains
Blood and CSF evaluation effective and no evidence of another etiology develops.
Response to glucocorticoids and Radiographic improvement often lags several weeks behind
further investigation clinical improvement [1,8]. In uncomplicated patients, MRI
TREATMENT scans to monitor improvement and maintenance of
improvement on and then off treatment should be
Glucocorticoids
performed every one to two months until findings
Follow-up
normalize [1]. This should be followed by MRI scans every
Second-line treatments
six months for a period of two years following the diagnosis
PROGNOSIS
[15]. MRI and other diagnostic testing should be performed
SUMMARY AND promptly if symptoms recur.
RECOMMENDATIONS
REFERENCES Second-line treatments A small group of patients will
require other immunosuppressive medications either to
GRAPHICSView All limit the complications of corticosteroid use or to keep the
TABLES disorder in remission. Typically, such patients will have
Causes painful ophthalmoplegia biopsy confirmation of the diagnosis.

Cyclosporine, azathioprine, methotrexate,

RELATED TOPICS
Clinical manifestations and
diagnosis of granulomatosis with
polyangiitis (Wegener's) and
microscopic polyangiitis
Horner's syndrome
Neurologic sarcoidosis
mycophenolate mofetil, and infliximab have been used in
this setting [15,33-35].
A few case reports have described long-term remittance of
symptoms with radiotherapy after a history of relapsing
Tolosa-Hunt syndrome and corticosteroid dependence
[33,36], or as primary treatment when corticosteroids are
contraindicated [37].

PROGNOSIS Glucocorticoids drastically hasten the

resolution of symptoms, with pain resolving within 24 to 72
hours after initiating treatment [4]. Cranial neuropathies
tend to recover more slowly over two to eight weeks even
with glucocorticoid therapy [4,10,26,38]. In unusual cases,
residual deficits remain [3,7].

Recurrences occur in about one-half of reported patients

over an interval of months to years [3,7]. Ipsilateral,
contralateral, and bilateral relapses have been reported.
Relapses require repeated investigations to rule out
inflammatory and neoplastic disorders such as sarcoid,
Wegener's granulomatosis, and lymphoma [15,36]. (See
'Diagnostic evaluation' above.)

There is no evidence that corticosteroid therapy alters the

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TOPIC OUTLINE The Tolosa-Hunt syndrome is caused by an

INTRODUCTION inflammation in the cavernous sinus or superior
PATHOGENESIS orbital fissure of unknown etiology. (See
CLINICAL FEATURES 'Pathogenesis' above.)

DIFFERENTIAL DIAGNOSIS Cardinal features include retroorbital pain and

DIAGNOSTIC EVALUATION
Magnetic resonance imaging
Blood and CSF evaluation
Response to glucocorticoids and
further investigation
TREATMENT
Glucocorticoids
Follow-up
Second-line treatments
PROGNOSIS
SUMMARY AND
RECOMMENDATIONS
REFERENCES
GRAPHICSView All
TABLES
Causes painful ophthalmoplegia
RELATED TOPICS
Clinical manifestations and
diagnosis of granulomatosis with
polyangiitis (Wegener's) and
microscopic polyangiitis
Horner's syndrome
Neurologic sarcoidosis
ophthalmoplegia affecting the third, fourth, and/or
sixth cranial nerves. All age groups may be affected.
(See 'Clinical features' above.)
The differential diagnosis includes malignancies and
infections, as well as vascular and other inflammatory
etiologies (table 1). (See 'Differential
diagnosis' above.)
Contrast-enhanced magnetic resonance imaging
(MRI), blood, and cerebrospinal fluid evaluation are
required to exclude other conditions. Results of these
tests, with a characteristic response to corticosteroid
therapy, support but do not definitively confirm the
diagnosis. (See 'Diagnostic evaluation' above.)
We recommend treatment with glucocorticoids for
those who meet clinical and diagnostic criteria for
Tolosa-Hunt syndrome (Grade 1A). We use
prednisone 80 to 100 mg daily for three days. If pain
has resolved, we then taper prednisone to 60 mg,
then to 40 mg, 20 mg, and 10 mg in two-week
intervals. (See 'Treatment' above.)
Close clinical and MRI follow-up is essential. We
recommend repeating diagnostic investigations and
consideration of a surgical biopsy for patients who fail
to respond clinically or radiographically to treatment,
or who relapse on treatment. (See 'Response to
glucocorticoids and further investigation' above.)

The prognosis for most patients is favorable.

However, some patients follow a relapsing-remitting
course requiring prolonged corticosteroid or other
immunosuppressive therapy, and a few have
permanent cranial nerve deficits. (See
'Prognosis' above.)

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REFERENCES
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aneurysm. J Neurol Neurosurg Psychiatry 1954;
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INTRODUCTION 3. HUNT WE, MEAGHER JN, LEFEVER HE, ZEMAN W.
Painful opthalmoplegia. Its relation to indolent
PATHOGENESIS
inflammation of the carvernous sinus. Neurology
CLINICAL FEATURES 1961; 11:56.
DIFFERENTIAL DIAGNOSIS 4. Smith JL, Taxdal DS. Painful ophthalmoplegia. The
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Response to glucocorticoids and syndrome in a patient with systemic lupus
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TREATMENT 6. Headache Classification Subcommittee of the
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Follow-up classification of headache disorders, 2nd edition.
Second-line treatments Cephalalgia 2004; 24 Suppl 1:8.
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RECOMMENDATIONS 8. Colnaghi S, Versino M, Marchioni E, et al. ICHD-II
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RELATED TOPICS
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12. Sondheimer FK, Knapp J. Angiographic findings in the
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13. Inzitari D, Sit D, Marconi GP, Barontini F. The
tolosa-Hunt syndrome: further clinical and
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Neurol 2002; 249:1237.
TOPIC OUTLINE 19. Goto Y, Hosokawa S, Goto I, et al. Abnormality in the
INTRODUCTION cavernous sinus in three patients with Tolosa-Hunt
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PATHOGENESIS
Psychiatry 1990; 53:231.
CLINICAL FEATURES
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Follow-up patients with 20 episodes of painful ophthalmoplegia.
Second-line treatments Eur J Radiol 2009; 69:445.
PROGNOSIS 23. Kbor J, Vrs E, Dek A. Magnetic resonance imaging
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REFERENCES 24. Kwan ES, Wolpert SM, Hedges TR 3rd, Laucella M.

GRAPHICSView All
TABLES
Causes painful ophthalmoplegia
RELATED TOPICS
Clinical manifestations and
diagnosis of granulomatosis with
polyangiitis (Wegener's) and
microscopic polyangiitis
Horner's syndrome
Neurologic sarcoidosis
Tolosa-Hunt syndrome revisited: not necessarily a
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25. Yousem DM, Atlas SW, Grossman RI, et al. MR
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32. Sugano H, Iizuka Y, Arai H, Sato K. Progression of
Tolosa-Hunt syndrome to a cavernous dural
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the management of non-infectious orbital

TOPIC OUTLINE inflammatory disease. Br J Ophthalmol 2001;
INTRODUCTION 85:1220.

PATHOGENESIS 35. O' Connor G, Hutchinson M. Tolosa-Hunt syndrome

responsive to infliximab therapy. J Neurol 2009;
CLINICAL FEATURES
256:660.
DIFFERENTIAL DIAGNOSIS
36. Foubert-Samier A, Sibon I, Maire JP, Tison F.
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Magnetic resonance imaging low-dose focal radiotherapy. Headache 2005; 45:389.
Blood and CSF evaluation 37. Furukawa Y, Yamaguchi W, Ito K, et al. The efficacy of
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Glucocorticoids Tolosa-Hunt syndrome: computed tomographic
Follow-up changes and reversal after steroid therapy. Can J
Second-line treatments Ophthalmol 1986; 21:287.
PROGNOSIS
SUMMARY AND
RECOMMENDATIONS
REFERENCES

GRAPHICSView All

TABLES
Causes painful ophthalmoplegia

RELATED TOPICS

Clinical manifestations and

diagnosis of granulomatosis with
polyangiitis (Wegener's) and
microscopic polyangiitis
Horner's syndrome
Neurologic sarcoidosis

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