This syndrome is noticeable as periorbital or hemicranial pain, ophthamoplegia and sensory loss. Several arrangements of lesions of the cranial nerve have been depicted in the syndrome but all localize the site of the lesion to be the “cavernous sinus” or “superior orbital fissure”. It is a condition that reacts well to therapy with steroids but which can reoccur months or even years after the first attack in up to 50% of the cases. The etiology is not known although it shares histopathogical features with idiopathic orbital pseudotumor.
While reports of intracranial extension of the inflammation exist [ 1 ], 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 [ 5 ]. 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 and microscopic polyangiitis" .)