Painful ophthalmoplegia is also known as or subsumes Orbital inflammatory pseudotumor, Orbital myositis, and Tolosa-Hunt syndrome. -ed.
Orbital inflammatory pseudotumor is a condition that presents with orbit pain and variable degrees of proptosis, conjunctival injection, diplopia, and vision loss from optic nerve involvement. It is generally responsive to oral corticosteroids, which form the mainstay of treatment, but occasionally the disease is refractory and additional immunosuppressive agents or orbit radiation may be indicated. In this clinical article, Dr. James Goodwin of the University of Illinois cites evidence that in some patients with orbital inflammatory disease, it is a localized manifestation of a systemic sclerosing IgG4-related disease, and CT may show orbital bone erosion that initially suggests neoplasia.
Orbital inflammatory pseudotumor refers to idiopathic inflammation in the orbit that may extend through the superior orbital fissure into the cavernous sinus, or may occur primarily in the cavernous sinus (Tolosa-Hunt syndrome) and extend into the orbit secondarily.
If any specific infectious etiology is discovered, the process is not called orbital inflammatory pseudotumor but is named for the specific infection, eg, tuberculosis or fungi.
Most cases respond promptly to high-dose (60 to 100 mg/day) prednisone, which should be continued for several weeks at high dose until ocular motility returns toward normal and then tapered to seek the lowest steroid dose that will maintain clinical remission.
If the process responds to corticosteroids initially but breaks through repeatedly as the dose is tapered, consider the possibility of a lymphoma in the orbit and obtain tissue for pathologic examination, including cytology and other markers for neoplasm.