Neuro-Ophthalmology & Neuro-Otology
Transient visual loss
Sep. 25, 2024
MedLink®, LLC
3525 Del Mar Heights Rd, Ste 304
San Diego, CA 92130-2122
Toll Free (U.S. + Canada): 800-452-2400
US Number: +1-619-640-4660
Support: service@medlink.com
Editor: editor@medlink.com
ISSN: 2831-9125
Toll Free (U.S. + Canada): 800-452-2400
US Number: +1-619-640-4660
Support: service@medlink.com
Editor: editor@medlink.com
ISSN: 2831-9125
Worddefinition
At vero eos et accusamus et iusto odio dignissimos ducimus qui blanditiis praesentium voluptatum deleniti atque corrupti quos dolores et quas.
Painful ophthalmoplegia is the term applied to unilateral periocular or cranial pain together with ipsilateral single or multiple ocular motor palsies. Also present sometimes are hypesthesia in the ophthalmic and sometimes maxillary divisions of the trigeminal nerve, Horner syndrome, and optic neuropathy. The offending lesion is usually in the cavernous sinus or superior orbital fissure. There are many causes, including idiopathic pachymeningitis (formerly known as Tolosa-Hunt syndrome), intracavernous carotid aneurysm, arteriovenous fistula, malignancy, vasculitis, and bacterial or fungal infection. An important distinction must be drawn between these entities and the self-limited ischemic cranial mononeuropathy in adults with diabetes or essential hypertension, in which case imaging will not reveal any pertinent abnormalities. In children and younger adults, recurrent single unilateral ocular motor neuropathy with periocular pain has been labeled “recurrent painful ophthalmoplegic neuropathy,” an idiopathic condition in which brain imaging may show extra-axial inflammation of the affected ocular motor nerve. Other mimickers are inflammations and tumors of the orbit, which should be evident on imaging. Imaging is critical to localize the process, but an exact diagnosis often depends on biopsy or discovery of other relevant factors. Idiopathic pachymeningitis generally improves on treatment with corticosteroids, but so do the many other causes of this clinical syndrome.
• Painful ophthalmoplegia is characterized by unilateral periocular or cranial pain; ipsilateral single or multiple ocular motor palsies; and sometimes first or second trigeminal hypesthesia, Horner syndrome, and optic neuropathy. | |
• The process affects the cavernous sinus, superior orbital fissure, and/or orbit. | |
• A common cause is idiopathic pachymeningitis (formerly known as Tolosa-Hunt syndrome), but other causes include cavernous aneurysms, arteriovenous fistulas, malignancies, vasculitis, infections, and ischemia, as well as orbital inflammation and tumors. | |
• Localization and pathologic diagnosis depend on imaging, biopsy, and other contributory factors. | |
• When only a single ocular motor palsy is present in older adults with diabetes or hypertension, an important consideration is a self-limited ischemic process affecting the extra-axial nerve; brain imaging typically shows no pertinent abnormalities. | |
• When only a single ocular motor palsy occurs repeatedly without contributory factors, a diagnosis of “recurrent painful ophthalmoplegic neuropathy” has been assigned; imaging may show enhancement of the affected cranial nerve. | |
• Idiopathic pachymeningitis often improves clinically and radiologically with corticosteroid treatment. |
In 1858, Hirschfeld first described the “syndrome of the superior orbital fissure” in a patient with a head injury (13). At autopsy, a hematoma was demonstrated near that structure. In 1955, Jackson described four cases of ocular motor palsy associated with severe headache in patients with diabetes (17). In 1890, the term “ophthalmoplegic migraine” was first used by Charcot to describe cases of headache with ocular motor nerve palsy (06).
In 1954, Tolosa described a 47-year-old man with recurrent periocular pain and dysfunction of the third, fourth, fifth, and sixth cranial nerves (32). There had been a 3-year spontaneous remission between symptomatic episodes. The patient died a few days after an intracranial exploratory operation that had yielded no definite pathology. The autopsy, however, later showed inflammation in the intracavernous portion of the internal carotid artery and adjacent cranial nerves.
In 1961, Hunt and colleagues reported six patients with periocular and brow pain along with various combinations of third, fourth, and sixth cranial nerve dysfunction on the same side (15). They reviewed Tolosa's cases and judged that their patients had the same condition based on rapid and dramatic relief of pain and ophthalmoplegia produced by systemic corticosteroid administration.
Lakke described clinically similar cases and presented pathologic material from a 47-year-old male patient with severe right periocular pain followed by progressive ipsilateral ophthalmoplegia and Horner syndrome (22). In 1966, Smith and Taxdal used the term “Tolosa-Hunt syndrome” for the first time and described an excellent response to corticosteroids (29). However, many other cavernous sinus conditions, including aneurysms, arteriovenous fistulas, malignancies, and fungal infections, have a similar clinical presentation and may improve with corticosteroid treatment. Imaging often cannot easily distinguish among them. Consequently, many observers have urged that the eponym Tolosa-Hunt be discarded. In fact, the terms “idiopathic pachymeningitis,” “idiopathic hypertrophic pachymeningitis,” or “idiopathic granulomatous pachymeningitis” are now preferred.
The idea that orbital inflammation could also cause periocular pain and ipsilaterally diminished eye movements was long ago recognized by Birch-Hirschfeld, who coined the term "pseudotumor" to distinguish orbital inflammation from orbital tumor (05). In the early 1940s, a distinction was drawn between inflammation primarily involving the extraocular muscles and inflammation primarily involving orbital fat or other tissues (08). The term "orbital myositis" was coined to describe cases with primarily extraocular muscle involvement. The term “idiopathic orbital inflammation” has been applied when no underlying cause is identified.
Nearly 3,000 illustrations, including video clips of neurologic disorders.
Every article is reviewed by our esteemed Editorial Board for accuracy and currency.
Full spectrum of neurology in 1,200 comprehensive articles.
Listen to MedLink on the go with Audio versions of each article.
MedLink®, LLC
3525 Del Mar Heights Rd, Ste 304
San Diego, CA 92130-2122
Toll Free (U.S. + Canada): 800-452-2400
US Number: +1-619-640-4660
Support: service@medlink.com
Editor: editor@medlink.com
ISSN: 2831-9125