The Tolosa-Hunt syndrome is a rare autoimmune with an estimated annual incidence of one case per million per year. It is characterized by painful ophthalmoplegia (weakness of the eye muscles) and is caused by an idiopathic granulomatous inflammation of the cavernous sinus. While considered a benign condition, permanent neurologic deficits can occur, and relapses are common, often requiring prolonged immunosuppressive therapy. Tolosa-Hunt syndrome must be carefully differentiated from more malignant diagnoses, a mandate challenged by the lack of a specific diagnostic test abnormality.
The Tolosa-Hunt syndrome is caused by an inflammatory process of unknown etiology. On histopathology, there is a nonspecific inflammation of the septa and wall …show more content…
Laboratory tests and lumbar puncture are also recommended. Direct tissue biopsy is rarely performed due to the technically difficult and potentially harmful approach to the cavernous sinus.
The specific diagnostic criteria recommended by the International Headache Society are summarized:
●Unilateral headache
●Granulomatous inflammation of the cavernous sinus, superior orbital fissure or orbit, demonstrated by MRI or biopsy
●Paresis of one or more of the ipsilateral third, fourth, and/or sixth cranial nerves
●Evidence of causation demonstrated by both:
•Headache has preceded oculomotor paresis by <2 weeks or developed with …show more content…
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.
Administration of IV glucocorticoids is often recommended, but oral prednisone is also effective.
The Tolosa-Hunt syndrome is caused by an inflammation in the cavernous sinus or superior orbital fissure of unknown etiology. lose 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. We recommend treatment with glucocorticoids for those who meet clinical and diagnostic criteria for Tolosa-Hunt syndrome We use prednisone 80 to 100 mg daily for three days. 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