- Kenneth S Shindler, MD, PhD
Kenneth S Shindler, MD, PhD
- Assistant Professor, Department of Ophthalmology
- University of Pennsylvania
The Tolosa-Hunt syndrome is a rare syndrome 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. This syndrome was first described in 1954, and its exquisite responsiveness to glucocorticoid treatment was recognized a few years later [2-4].
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 of the cavernous sinus, with a lymphocyte and plasma cell infiltration, giant cell granulomas, and 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 , 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 . 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".)
The Headache Classification Subcommittee of the International Headache Society describes the course and features of Tolosa-Hunt syndrome as "episodic orbital pain associated with paralysis of one or more of the third, fourth, and/or sixth cranial nerves which usually resolves spontaneously but tends to relapse and remit" .
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- CLINICAL FEATURES
- DIFFERENTIAL DIAGNOSIS
- DIAGNOSTIC EVALUATION
- Diagnostic criteria
- Magnetic resonance imaging
- Blood and CSF evaluation
- Response to glucocorticoids and further investigation
- Second-line treatments
- SUMMARY AND RECOMMENDATIONS