Table 1.
Diagnostic criteria for Tolosa-Hunt Syndrome and Recurrent Painful and Ophthalmologic Neuropathy according to ICHD-3
| Tolosa-Hunt syndrome | Recurrent painful ophthalmoplegic neuropathy | |
|---|---|---|
| DESCRIPTION | Unilateral orbital or periorbital pain associated with paresis of one or more between the 3rd, 4th and/or 6th cranial nerves caused by a granulomatous inflammation in the cavernous sinus, superior orbital fissure or orbit | Repeated attacks of paresis of one or more ocular cranial nerves (commonly the 3rd), with ipsilateral headache |
| DIAGNOSTIC CRITERIA | A. Unilateral orbital or periorbital headache fulfilling criterion C | A. At least two attacks fulfilling criterion B |
| B. Both of the following: | ||
| B. Both of the following: | ||
| 1. granulomatous inflammation of the cavernous sinus, superior orbital fissure or orbit, demonstrated by MRI or biopsy | 1. unilateral headache | |
| 2. ipsilateral paresis of one, two or all three ocular motor nerves | ||
| 2. paresis of one or more of the ipsilateral the 3rd, 4th and/or 6th cranial nerves | ||
| C. Orbital, parasellar or posterior fossa lesion has been excluded by appropriate investigation | ||
| C. Evidence of causation demonstrated by both of the following: | D. Not better accounted for by another ICHD-3 diagnosis | |
| 1. headache is ipsilateral to the granulomatous inflammation | ||
| 2. headache has preceded paresis of the the 3rd, 4th and/or 6th nerves by ≤ 2 weeks, or developed with it | ||
| D. Not better accounted for by another ICHD-3 diagnosis | ||
| COMMENTS | Some reported cases of Tolosa-Hunt syndrome had additional involvement of the 5th nerve (commonly the first division) or optic, 7th or 8th nerves. Sympathetic innervation of the pupil is occasionally affected | Some data suggest that headache can develop up to 14 days prior to ocular motor paresis |
| Gadolinium enhancement or nerve thickening can be demonstrated using MRI | ||
| Careful follow-up is required to exclude other causes of painful ophthalmoplegia such as tumours, vasculitis, basal meningitis, sarcoid or diabetes mellitus | ||
| Pain and paresis of Tolosa-Hunt syndrome resolve when adequately treated with corticosteroids |