The inflammatory changes of Tolosa-Hunt syndrome are limited to the superior orbital fissure and can sometimes be detected by MRI; coronal views taken after gadolinium infusion show the lesion to best advantage. However, sarcoidosis, lymphomatous infiltration, and a small meningioma may produce similar radiographic findings and granulomatous (temporal) arteritis rarely causes ophthalmoplegia. The sedimentation rate in our patients with orbital pseudotumor or Tolosa-Hunt syndrome has varied but can be elevated, sometimes accompanied by a leukocytosis at the onset of symptoms. Sarcoidosis also can infiltrate the posterior orbit or cavernous sinus and cause a single or multiple unilateral nerve ophthalmoparesis as discussed in Chaps. 12 and 44.
Both orbital pseudotumor and Tolosa-Hunt syndrome are treated with corticosteroids. A marked response with reduction in pain and improved ophthalmoplegia in 1 or 2 days is typical; however, as pointed out in the review by Kline and Hoyt, tumors of the parasellar region that cause ophthalmoplegia may also respond, albeit not to the same extent. In both diseases, we have generally given prednisone 60 mg and tapered the medication slowly; although there are no data to guide the proper treatment, corticosteroids should be continued for several weeks or longer. The absence of a response to steroids should cause reconsideration of the diagnosis of Tolosa-Hunt syndrome.
When a total or nearly complete loss of eye movements of both eyes evolves within a day or days, it raises a limited number of diagnostic possibilities. Keane, who analyzed 60 such cases, found the responsible lesion to lie within the brainstem in 18 (usually infarction and less often Wernicke disease), in the cranial nerves in 26 (Guillain-Barré syndrome or tuberculous meningitis), within the cavernous sinus in 8 (tumors or infection), and at the myoneural junction in 8 (myasthenia gravis and botulism). Our experience has tended toward the Miller Fisher variant of Guillain-Barré syndrome, as did Keane’s later series (2007), and myasthenia. The ophthalmoplegic form of Guillain-Barré syndrome is very frequently associated with circulating antibodies to GQ1b ganglioside (see Chap. 44). There may be an accompanying paralysis of the dilator and constrictor of the pupil (“internal ophthalmoplegia”) that is not seen in myasthenia.
Unilateral complete ophthalmoplegia has an even more limited list of causes, largely related to local disease in the orbit and cavernous sinus, mainly infectious, neoplastic, or thrombotic.