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Unilateral orbital or periorbital pain associated with paresis of one or more of the IIIrd, IVth and/or VIth cranial nerves caused by a granulomatous inflammation in the cavernous sinus, superior orbital fissure or orbit.
- Unilateral orbital or periorbital headache fulfilling criterion C
- Both of the following:
- granulomatous inflammation of the cavernous sinus, superior orbital fissure or orbit, demonstrated by MRI or biopsy
- paresis of one or more of the ipsilateral IIIrd, IVth and/or VIth cranial nerves
- Evidence of causation demonstrated by both of the following:
- headache is ipsilateral to the granulomatous inflammation
- headache has preceded paresis of the IIIrd, IVth and/or VIth nerves by ≤2 weeks, or developed with it
- Not better accounted for by another ICHD-3 diagnosis.
Some reported cases of 13.8 Tolosa-Hunt syndrome had additional involvement of the Vth nerve (commonly the first division) or optic, VIIth or VIIIth 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, sarcoid or diabetes mellitus.
Pain and paresis of 13.8 Tolosa-Hunt syndrome resolve when it is treated adequately with corticosteroids.