Painful ophthalmoplegia: Overview with a focus on tolosa-hunt syndrome

Abstract

Painful ophthalmoplegia is an important presenting complaint to emergency departments, ophthalmologists, and neurologists. The etiological differential diagnosis of painful ophthalmoplegia is extensive and consists of numerous sinister etiologies including vascular (eg, aneurysm, carotid dissection, carotid-cavernous fistula), neoplasms (eg, primary intracranial tumors, local or distant metastases), inflammatory conditions (eg, orbital pseudotumor, sarcoidosis, Tolosa-Hunt syndrome), infectious etiologies (eg, fungal, mycobacterial), and other conditions (eg, microvascular infarcts secondary to diabetes, ophthalmoplegic migraine, giant cell arteritis). A systematic approach to the evaluation of painful ophthalmoplegia can lead to prompt recognition of serious disorders that if left untreated, can be associated with significant morbidity or mortality. Inflammatory conditions such as Tolosa-Hunt syndrome and orbital pseudotumor are highly responsive to corticosteroids, but should be diagnoses of exclusion.

Actinomycosis Mimicking Tolosa-Hunt Syndrome in a 6-Year-Old Boy: Case Report

“…Tolosa-Hunt syndrome is a rare idiopathic disorder characterized by painful unilateral ophthalmoplegia with associated paralysis of one or more of the third, fourth, and sixth cranial nerves caused by chronic granulomatous inflammation of the cavernous sinus or superior orbital fissure [1,2]. The differential diagnosis of ophthalmoplegia is broad and includes vascular, neoplastic, inflammatory, and infectious etiologies.…”

“…In the diagnosis of Tolosa-Hunt syndrome, MRI is the neuroimaging modality of choice to exclude alternative disease entities. Treatment is systemic corticosteroid therapy, which dually serves to further solidify the diagnosis, as dramatic responsiveness of pain to steroids is universally described [2,3]. …”

Cavernous sinus syndrome: need for early diagnosis

“…As seen in our patient, the most common cause of CSS is a neoplastic lesion, which can be secondary, result from local spread, or be primary in origin 5–7. Other causes of CSS include: thrombophlebitis, aspergillosis, Tolosa-Hunt syndrome, inflammatory pseudotumour, aneurysm of the internal carotid artery, carotid–cavernous fistula and dural arteriovenous shunt 1 2 5 8. Also, there are many conditions that can produce a painful ophthalmoplegia and mimic a CSS such as vasculitis, basal meningitis, diabetes mellitus and ophthalmoplegic migraine 4 8…”

A case of posterior scleritis: differential diagnosis of ocular pain

“…Among diseases affecting the eye, orbital pseudotumour and tumours (primary or secondary) should be considered. In these cases ocular signs, such as conjunctival injection, proptosis, eyelid oedema and chemosis are usually more evident and ophthalmoplegia can be associated [8]. Vascular diseases may also cause ocular pain.…”

“…Carotid artery dissection may present with facial or ocular pain frequently, but not necessarily, in association with Claude-Bernard-Horner syndrome. Neoplastic, infectious and inflammatory diseases as well as Tolosa Hunt syndrome, which affect the cavernous sinus, may present with painful ophthalmoplegia, even though at onset ocular movements may be normal [8]. Trigeminal neuralgia is another important cause of facial pain, stabbing in character, which can involve only the first trigeminal branch and therefore present with periocular pain, although this localisation is the least frequent (5%) [9].…”