Orbital Pain and Double Vision: Diagnosis and Discussion

The patient presented in the case “Orbital Pain and Double Vision” (JW Neurol Jun 29 2010) had Tolosa-Hunt syndrome. Given the presenting signs and symptoms, the diagnosing clinician would also have to consider idiopathic hypertrophic pachymeningitis, sarcoidosis, tuberculosis-like infections, primary central nervous system lymphoma, Wegener granulomatosis, and Erdheim-Chester disease.

The vignette described a middle-aged, healthy woman with right orbital pain who developed right abducens palsy. The brain magnetic resonance image, with frequency-selective fat-saturation pulse sequences and gadolinium contrast, showed enhancement of the bilateral cavernous sinus on the right more than on the left. Enhancement extended toward the posterior aspect of the tentorium cerebelli from the right cavernous sinus. Note some involvement of the pituitary fossa but sparing of the orbits. Given this information, Tolosa-Hunt syndrome is the most likely diagnosis. Of respondents, 35% came to a similar conclusion. Incorrect answers included pituitary apoplexy, optic neuritis, cavernous sinus thrombosis, and granular cell neoplasm of infundibulum. The most interesting incorrect answer was Vogt-Koyanagi-Harada syndrome; however, our patient didn't have bilateral panuveitis, auditory symptoms, skin lesions, or other relevant symptoms.

This case highlights the importance of clinicoradiological correlation. Tolosa-Hunt syndrome is a granulomatous inflammation of the cavernous sinus that presents with headache and cranial neuropathies. A detailed history and physical exam will help eliminate many systemic etiologies. Diagnostic work-up should, at a minimum, include magnetic resonance imaging of the brain with contrast (before prescribing corticosteroids) and cerebrospinal fluid examination. Pain immediately responds to steroids, and remission usually follows in 3 to 12 months. For resistant cases, a biopsy may be pursued. For long-term nonresponders, corticosteroid-sparing drugs (e.g., azathioprine, methotrexate, cyclophosphamide, mycophenolate mofetil) and radiation therapy have been tried.