8.14.07 Tolosa Hunt Syndrome Pagliei

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Tolosa Hunt Syndrome

Jennifer Pagliei

August 14, 2007



Introduction

Tolosa Hunt syndrome (THS):

A rare syndrome first described in 1954.

Characterized by painful ophthalmoplegia(paralysis of the eye muscles).

Caused by inflammation of the cavernous

sinus or superior orbital fissure. Considered a benign condition.

Can result in permanent neurologic deficits.



Introduction

Spontaneous remissions can occur.

Relapse rates are 30 to 40%.

It frequently mimics other conditions. There is no single characteristic that is

pathognomonic for the syndrome.

It is a diagnosis of exclusion. The cause is unknown.

Systemic involvement does not occur.



Prevalence

THS is uncommon in both the UnitedStates and internationally.

The estimated annual incidence is onecase per million per year.

Males and females are equally affected.

It is rare during the first 2 decades of life. There is an equal distribution in people

over the age of 20.



Pathophysiology

THS is characterized by nonspecificinflammation, either noncaseating

granulomatous or nongranulomatous, within thecavernous sinus or superior orbital fissure.

The inflammation causes pressure and results indysfunction of the structures in the cavernous

sinus: Cranial nerves III, IV, and VI, and the superior

division of cranial nerve V, the ophthalmic nerve.



Physical Exam

The result is unilateral acute orbital pain, whichcharacterizes the onset of the disorder.

Ophthalmoparesis, or disordered eyemovements due to eye muscle weakness, occurwhen cranial nerves III, IV, and VI are damagedby inflammation.

Pupillary dysfunction and ptosis may result from

injury to the oculomotor nerve. Trigeminal nerve involvement (primarily V1, the

ophthalmic branch) will cause paresthesias of the forehead.



Physical Exam

Intracranial extension of the inflammation intothe orbit at the orbital apex can occur but israre, and can affect the maxillary (V2) andmandibular (V3) branches of the trigeminalnerve (V), the optic nerve (II), and the facialnerve (VII).

Lid swelling and mild proptosis may result if theorbit is affected.

Loss of the ipsilateral corneal reflex indicatestrigeminal nerve involvement.



Physical Exam

If the optic nerve is affected, optic discedema or pallor can result and vision loss

may occur. Vision loss is uncommon but unpredictable

and may be permanent.

Pathologic involvement beyond thecavernous sinus, superior orbital fissure,or apex of the orbit rarely occurs.



History

Patients usually present with severe retro-orbitalor periorbital pain of acute onset that is constantin nature.

The pain is characteristically described as asteady gnawing or boring pain.

Diplopia due to ophthalmoparesis usually follows

the onset of pain. In rare cases, the ophthalmoparesis may

precede the pain, sometimes by several days.



History

The symptoms are typically unilateral, althoughbilateral symptoms are seen in 4 to 5% of cases.

The frequency of cranial nerve involvement is: Cranial nerve III - 85%.

Cranial nerve VI - 70%.

The ophthalmic division of cranial nerve V - 30%.

Cranial nerve IV - 29%.



Laboratory Data

The diagnosis of THS is usually one of exclusion.

Laboratory workup, to exclude other possible processesincludes: CBC count Erythrocyte sedimentation rate (ESR)

Electrolytes

A1C

Liver function tests

Thyroid function tests

Fluorescent treponemal antibody (FTA)

Antinuclear antibody (ANA)



Laboratory Data

Antineutrophil cytoplasmic antibody (ANCA)

Anti-dsDNA antibody

Anti-Sm antibody Antinuclear cytoplasmic antibody

Serum protein electrophoresis

Lyme serologies

C reactive protein Angiotensin-converting enzyme (ACE) level

HIV titer



Laboratory Data

Cerebrospinal fluid (CSF) studies, helpful in eliminatingconditions mimicking THS, include: Cell count with differential

Protein Glucose

Cultures for bacteria, fungi, and mycobacteria

Gram stain

Cytology

Opening pressure Angiotensin converting enzyme

Lyme serology

Syphilis serology



Laboratory Data

In patients with THS, the results of blood andCSF analysis are generally normal.

Elevated ESR, mild leukocytosis, and elevated ANA concentrations have been reported, butusually suggest an underlying connective tissuedisorder.

A mild lymphocytic pleocytosis or mildly highprotein in the CSF may occur, but shouldsuggest other diagnoses such as neoplasm,inflammatory or infectious meningitis.





MRI

Contrast-enhanced MRI is the modality of choice.

MRI findings in THS can include: Inflammation of the cavernous sinus, superior orbital fissure, or

orbital apex. Narrowing of the intracavernous internal carotid artery.

Enlargement of the optic nerve or external ocular muscles.

These findings are not specific to THS and may be seenwith other etiologies such as lymphoma, sarcoidosis, andmeningioma.

In rare cases, the MRI is normal.



Diagnosis

The diagnosis of THS is based on:

Clinical presentation

Laboratory tests and LP results

Neuroimaging results

Clinical response to corticosteroids



Diagnosis

The International Headache Society criteria forTHS include the following: One or more episodes of unilateral orbital pain lasting

for an average of 8 weeks if left untreated.

Third, forth, and/or sixth cranial nerve palsy, whichbegins within two weeks of the onset of orbital pain.

Symptoms that resolve within 48-72 hours of

initiation of steroid therapy. Exclusion of other etiologies by appropriate

investigation, including neuroimaging.

http://www.i-h-s.org/

http://www.i-h-s.org/



Diagnosis

Direct tissue biopsy of the lesion:

May be required to confirm the diagnosis of THS or

exclude neoplasm. Is rarely performed due to technical difficulty and

potentially harmful approach to the cavernous sinus.

Histolpathology shows:

Lymphocyte infiltration Plasma cell infiltration

Giant cell granulomas

Proliferation of fibroblasts



Differential Diagnosis

Over 75% of patients who present withpainful ophthalmoplegia will not have THS.

The syndrome of painful ophthalmoplegiamay be caused by any process exerting amass effect on the cavernous sinus.

Of the other conditions possible, tumorsare the most common, representing 30%of cases.




Anisocoria

Arteriovenous malformations

Benign skull tumors

Cavernous sinus syndromes Cerebral aneurysms

Cerebral venous thrombosis

Cranioppharyngioma

Diabetic Neuropathy

Epidural hematoma

Extraocular muscle dysfunction

Lyme disease

Meningioma

Metastatic disease to the brain Migraine headache

Neurosarcoidosis

Pituitary tumors

Polyarteritis nodosa

Primary CNS lymphoma




Primary malignant skulltumors

Systemic lupus

erythematosus Tuberculosis meningitis Varicella zoster Wegeners granulomatosis Whipple disease Carotid-cavernous fistula Cavernous angioma Fungal infections

Lymphoma Melanoma Miller Fisher Syndrome

Orbital myositis Orbital pseudotumor Sarcoidosis Syphilis

Vasculitis Giant cell arteritis Carotid dissection



Treatment

Corticosteroids have been the treatment of choice since the 1960s.

They provide significant pain relief within 24-72hours of initiation, helping to confirm thediagnosis.

Improvement of cranial nerve deficits and

regression of MRI abnormalities should occurover the next 2-8 weeks, also providingconfirmation of the diagnosis.



Corticosteroids

However, an initial clinical or MRI response tosteroids is not diagnostic.

Other diseases, such as lymphoma andvasculitis, may also respond clinically andradiographically to steroid therapy.

More aggressive testing, including repeat CSF

evaluation, should be performed if steroidefficacy is lost after an initial response or if noresponse occurs within 72 hours.



Corticosteroids

Corticosteroid regimens used for treatment vary,but in general include initial high-dose

corticosteroids for two to four weeks followed bya gradual taper over at least four to six weeksand up to several months.

A suggested regimen is:

Prednisone 80 to 100 mg daily for three days.

If the pain has resolved, taper to 60 mg daily, then40 mg, then 20 mg, then 10 mg every two weeks.



Corticosteroids

Prompt administration of IV corticosteroidsis often recommended, but oral

prednisone is also effective. The rate of taper should be guided by

clinical symptoms.

Persistent MRI findings should notdiscourage dose reductions as long asfindings are regressing.



Treatment

If a patient is unable to tolerate steroid therapy,or has refractory disease, other

immunosuppressive therapy may be considered,including:

Azathioprine

Methotrexate

Cyclosporine Mycophenolate mofetil

Radiation therapy



Treatment

Surgical removal is not generally a feasibletreatment.

The primary value of surgical interventionis a histopathologic diagnosis.

Neurosurgery consultation is helpful in

making the decision whether to biopsy.



Follow-up

Close clinical follow-up with repeat MRI is necessary tobe sure that corticosteroid treatment remains effectiveand that evidence of another etiology does not develop.

Radiographic improvement often lags several weeksbehind clinical improvement. MRI scans should be performed every 1-2 months to

monitor improvement and maintenance of improvementon and then off treatment, until findings normalize.

MRI should be performed every 6 months for a period of 2 years following diagnosis. MRI and other diagnostic testing should be performed

promptly if symptoms recur.



Prognosis

Typically, the prognosis is good. Spontaneous remissions often occur after an

average of about eight weeks. Patients usually respond to corticosteroids with

pain relief within 24 to 72 hours of initiatingtreatment.

Cranial neuropathies tend to recover more

slowly over 2-8 weeks. In rare cases, permanent ocular motor deficits

may remain.



Prognosis

Thirty to 40% of successfully treated patientsmay experience relapse.

Patients with spontaneous remissions have equal

risk of reoccurrence as those treated withmedication.

Relapse typically occurs on the same side as theoriginal lesion but can be observed on the

opposite side or bilaterally. Relapses require repeated investigations to rule

out inflammatory and neoplastic disorders.



Summary

Tolosa-Hunt syndrome is caused byinflammation in the cavernous sinus or

superior orbital fissure of unknownetiology.

Cardinal features include retroorbital painand ophthalmoplegia affecting the third,

fourth, and/or sixth cranial nerves. All age groups may be affected.



Summary

The differential diagnosis is broad and includesmalignancies and infections, as well as vascular

and other inflammatory etiologies. Contrast-enhanced MRI, blood, and CSF

evaluation are required to exclude otherconditions.

Results of these tests, and a characteristicresponse to corticosteroid therapy, support butdo not definitively confirm the diagnosis of THS.



Summary

Treatment with corticosteroids is recommendedfor those who meet clinical and diagnosticcriteria for THS.

Prednisone at 80 to 100 mg daily for three daysis recommended.

If pain has resolved, prednisone can be tapered

to 60 mg, then 40 mg, 20 mg, and 10 mg intwo-week intervals.

Close clinical and MRI follow-up is necessary.



Summary

Repeat diagnostic intervention and considerationof surgical biopsy is recommended in patientswho fail to respond to treatment, or who relapse

on treatment. The prognosis for most patients is favorable. However, some patients follow a relapsing-

remitting course requiring prolonged

corticosteroid or other immunosuppressivetherapy. Some patients will have permanent cranial nerve

deficits.



References

Shindler, Kenneth S. Tolosa-Huntsyndrome. UpToDate. 2007.

Tolosa-Hunt Syndrome. eMedicine 2006.

Tran, Duc. Tolosa Hunt Syndrome.Baylor College of Medicine 2000.

Zafrulla, KM. Tolosa Hunt syndrome.Indain J Opthalmol. 2007; 25:22-26.

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