eMedicine Specialties > Neurology > Inflammatory and Demyelinating Diseases
Tolosa-Hunt Syndrome: Treatment & Medication
Updated: Mar 12, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
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 has been employed.
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
Steroids are used to treat the inflammation of Tolosa-Hunt syndrome. Pain relief usually occurs rapidly, ie, within 24-72 hours.5 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
Reduce pain and inflammation; diminish the size of the inflammatory mass.
Prednisone (Deltasone, Orasone, Meticorten)
May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and also suppresses lymphocytes and antibody production.
Adult
60-120 mg/d PO
Pediatric
Not established
Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Hyperglycemia, edema, insomnia, weight gain, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, mood alteration or psychosis, myasthenia gravis, growth suppression, avascular necrosis, and infections may occur with glucocorticoid use; abrupt discontinuation of glucocorticoids may cause potentially life-threatening adrenal crisis
Immunosuppressive agents
Decrease autoimmune reaction.
Methotrexate (Amethopterin)
Antimetabolite used to treat many autoimmune processes. The mode of action is not known; this drug does interfere with DNA synthesis.
Adult
7.5 mg PO weekly (administered as a single dose or as divided doses of 2.5 mg q12h for 3 doses)
Pediatric
Not recommended
Leucovorin, 5-fluorouracil, ara-C, asparaginase, carboxypeptidase, thiopurines, colchine, nitrous oxide, probenecid, aspirin, other nonsteroidal anti-inflammatory medications, sulfonamides, triamterene, trimethoprim, pyrimethamine, folic acid, phenytoin, procarbazine
Breastfeeding; pregnancy or risk of pregnancy; allergy or hypersensitivity to methotrexate
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Must be used with caution in patients with pleural effusions, ascites, hepatic impairment, renal impairment, obesity, and diabetes; can cause interstitial pneumonitis
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.
Adult
2-3 mg/kg/d (administered as single or divided dose with meals)
Total body weight used, even with obese patients; begin at a tester dose (50 mg/d) because some patients develop an idiosyncratic reaction with flulike symptoms and liver and bone marrow toxicity even at this lower dose; if well tolerated after 1 wk clinically and by laboratory studies, increase to effective dose
Pediatric
Not recommended
Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of methotrexate metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine
Documented hypersensitivity; pregnancy; breastfeeding; idiosyncratic reaction (10% of patients have idiosyncratic reaction of fever, malaise, and myalgias)
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Monitor patients for pancytopenia (or individually occurring leukopenia, thrombocytopenia, and anemia); effects on bone marrow are usually dose-related; risk of infection increased in patients on azathioprine; do not allow WBC count to fall below 3000/µL or lymphocyte count to fall below 1000/µL; also monitor patients for signs of hepatotoxicity; in general, patients should have CBC counts and liver function tests performed after the first wk, then monthly for several mo, before reducing the frequency of laboratory tests as clinically appropriate
More on Tolosa-Hunt Syndrome |
| Overview: Tolosa-Hunt Syndrome |
| Differential Diagnoses & Workup: Tolosa-Hunt Syndrome |
 Treatment & Medication: Tolosa-Hunt Syndrome |
| Follow-up: Tolosa-Hunt Syndrome |
| Multimedia: Tolosa-Hunt Syndrome |
| References |
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References
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Further Reading
Keywords
Tolosa-Hunt syndrome, THS, painful ophthalmoplegia, idiopathic cavernous sinus inflammation, inflammation of the superior orbital fissure, painful ophthalmoparesis, inflammation of the cavernous sinus fissure
