Most patients who present with painful ophthalmoplegia will not have Tolosa-Hunt syndrome. The syndrome of painful ophthalmoplegia may be caused by any process exerting a mass effect on the cavernous sinus. These

(click for larger image) Table 1: Causes of Painful Ophthalmoplegia

include a primary intracranial tumor, lymphoma, other local or distant metastatic tumors, aneurysm, carotid-cavernous fistula, carotid dissection, cavernous sinus thrombosis, infection, vasculitis, and sarcoidosis (see Table 1). Of these conditions, tumors and vascular conditions are the most common. In addition to these structural, compressive lesions, painful ophthalmoplegia can also be caused by ophthalmoplegic migraine, giant cell arteritis, or a diabetic cranial nerve palsy.

The diagnosis of Tolosa-Hunt syndrome is based upon the clinical presentation in conjunction with neuroimaging results and a clinical response to corticosteroids. Laboratory tests and lumbar puncture are also recommended. The specific diagnostic criteria recommended by the International Headache Society are:²

• Unilateral headache
• Granulomatous inflammation of the cavernous sinus, superior orbital fissure, or orbit, demonstrated by MRI or biopsy
• Paresis of one or more of the ipsilateral third, fourth, and/or sixth cranial nerves
• Evidence of causation demonstrated by both:
  • Headache has preceded oculomotor paresis by <2 weeks or developed with it.
  • Headache is localized around the ipsilateral brow and eye
• Symptoms not accounted for by an alternative diagnosis

Glucocorticoid administration has diagnostic as well as therapeutic utility.3 Rapid resolution of pain, within 24 to 72 hours, helps to confirm suspected Tolosa-Hunt syndrome. Improvement of cranial nerve deficits and regression of MRI abnormalities over the subsequent two to eight weeks provide further confirmation of the diagnosis.⁶ A suggested regimen is prednisone 80 to 100 mg daily for three days. If pain has resolved, then taper prednisone to 60 mg, then to 40 mg, 20 mg, and 10 mg in two-week intervals. A small group of patients will require other immunosuppressive medications either to limit the complications of corticosteroid use or to keep the disorder in remission. Typically, such patients will require biopsy confirmation of the diagnosis.⁷

The prognosis for most patients is favorable. However, some patients follow a relapsing-remitting course requiring prolonged corticosteroid or other immunosuppressive therapy, and a few have permanent cranial nerve deficits.

Dr. Sterk is associate professor of emergency medicine at Loyola University Chicago–Stritch School of Medicine..

References

1. Iaconetta G, Stella L, Esposito M, et al. Tolosa-Hunt syndrome extending in the cerebello-pontine angle. Cephalalgia. 2005;25(9):746-750.
2. Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia. 2013;33(9):629-808.
3. Colnaghi S, Versino M, Marchioni E, et al. ICHD-II diagnostic criteria for Tolosa-Hunt syndrome in idiopathic inflammatory syndromes of the orbit and/or the cavernous sinus. Cephalalgia. 2008;28(6):577-584.
4. Kline LB, Hoyt WF. The Tolosa-Hunt syndrome. J Neurol Neurosurg Psychiatry. 2001;71(5):577-582.
5. Zhang X, Zhou Z, Steiner TJ, et al. Validation of ICHD-3 beta diagnostic criteria for 13.7 Tolosa-Hunt syndrome: analysis of 77 cases of painful ophthalmoplegia. Cephalalgia. 2014;34(8):624-632.
6. Cakirer S. MRI findings in Tolosa-Hunt syndrome before and after systemic corticosteroid therapy. Eur J Radiol. 2003;45(2):83-90.
7. Shindler KS. Tolosa-Hunt syndrome. UpToDate websitet. Accessed Nov.9, 2018.