Table 1 International Classification of Headache Disorders (ICHD-3) criteria for Tolosa-Hunt syndrome

Unilateral orbital and periorbital pain associated with paresis of one or more of the third, fourth or sixth cranial nerves caused by a granulomatous inflammation in the cavernous sinus, superior orbital fissure or orbit.

DIAGNOSTIC CRITERIA

A. Unilateral orbital or periorbital headache fulfilling criterion C.

B. Both of the following:

1. Granulomatous inflammation of the cavernous sinus, superior orbital fissure or orbit, demonstrated by MR scan or biopsy.

2. Paresis of one or more of the ipsilateral third, fourth and/or sixth cranial nerves.

C. Evidence of causation demonstrated by both of the following:

1. Headache is ipsilateral to the granulomatous lesion.

2. Headache has preceded paresis of the third, fourth and/or sixth nerves by ≤2 weeks or developed with it.

D. Not better accounted for by another ICHD-3 diagnosis.

Comments:

► Some reported cases of 13.8 Tolosa-Hunt syndrome had additional involvement of the fifth nerve (commonly the first division) or optic, seventh or eighth nerves. Sympathetic innervation of the pupil is occasionally affected.

► Careful follow-up is required to exclude other causes of painful ophthalmoplegia such as tumours, vasculitis, basal meningitis, sarcoidosis or diabetes mellitus.

► Pain and paresis of 13.8 Tolosa-Hunt syndrome resolve when it is treated adequately with corticosteroids.