What is the best immunosuppressant regimen in an otherwise healthy 50-year-old white man with residual left optic neuropathy due to orbital apex syndrome (Tolosa-Hunt syndrome) after an excellent response to high doses of prednisone? We have twice attempted to taper the steroids, but each attempt precipitated a relapse, with worsening visual function associated with pain. Excessive weight gain, refractory insomnia, fatigue, and elevated blood pressure and intraocular pressure are of great concern at this time.
Jose G. Valedon, MD
Response from Rohit Bakshi, MD
The etiology and differential diagnosis of Tolosa-Hunt syndrome (THS) is a matter of controversy.[1,2] I assume that your patient has clinical[2,3] and neuroimaging features consistent with THS and that you have excluded other underlying neoplastic and inflammatory causes. Corticosteroids are the mainstay of treatment for THS. In fact, a lack of response to steroids should raise the suspicion of other causes.
In patients who fail glucocorticoid therapy or develop untoward side effects, you might try other immunosuppressive treatments while gradually tapering the steroids. Chlorambucil (0.1 to 0.2 mg/kg daily) or methotrexate (7.5 to 15 mg weekly) could be tried with the understanding that these treatments are not proven for THS but are empiric. Another alternative is azathioprine as reported by Hannerz.
Medscape Neurology. 2000;2(1) © 2000 Medscape
Cite this: Rohit Bakshi. What Is the Best Immunosuppressant Regimen for Tolosa-Hunt Syndrome - Medscape - May 02, 2000.