Tolosa-Hunt Syndrome

Excerpt

Tolosa-Hunt syndrome is a rare, unilateral idiopathic granulomatous inflammatory disease affecting the cavernous sinus, superior orbital fissure, or orbit. The condition presents with severe ocular pain and ophthalmoplegia due to paresis of cranial nerves III, IV, or VI. The syndrome falls within the spectrum of idiopathic orbital inflammatory diseases, a group that also includes orbital pseudotumor. As Tolosa-Hunt syndrome is idiopathic, other causes of painful ophthalmoplegia, such as vasculitis, meningitis, neoplasm, sarcoidosis, and orbital pseudotumor, must be excluded.

Tolosa-Hunt syndrome was first described in 1954 by Dr Eduardo Tolosa, a Spanish neurosurgeon. Similar cases were reported by Hunt et al in 1961. The term Tolosa-Hunt syndrome was first introduced by Smith and Taxdal in 1966. Smith and Taxdal described a dramatic response to steroid therapy. This steroid responsiveness is used as a diagnostic marker, yet it also becomes a common cause for misdiagnosis.

Tolosa-Hunt syndrome is one of the rare disorders recognized by the National Organisation for Rare Disorders. The International Classification of Headache Disorders (ICHD-3 beta) defines Tolosa-Hunt syndrome as unilateral orbital pain accompanied by paresis of one or more of the third, fourth, and sixth cranial nerves, attributed to granulomatous inflammation confirmed by magnetic resonance imaging (MRI) or biopsy. Subsequent case series using the ICHD-3 beta diagnostic criteria demonstrated a high percentage of false-negative and false-positive cases, making the diagnostic criteria less helpful. With other underlying causes of painful ophthalmoplegia being identified, such as lymphoma, infections, vasculitis, and other noninfectious orbital inflammatory disorders, including granulomatosis with polyangiitis, polyarteritis nodosa, sarcoidosis, and IgG-4-related disease, fewer cases of Tolosa-Hunt syndrome are diagnosed. A thorough diagnostic evaluation remains essential to ensure accurate diagnosis and appropriate management.