13.7 Tolosa-Hunt syndrome

Description:
Unilateral orbital 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.

Diagnostic criteria:
A. Unilateral headache fulfilling criterion C
B. Both of the following:

    1. granulomatous inflammation of the cavernous sinus, superior orbital fissure or orbit, demonstrated by MRI or biopsy
    2. paresis of one or more of the ipsilateral IIIrd, IVth and/or VIth cranial nerves

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

    1. headache has preceded paresis of the IIIrd, IVth and/or VIth nerves by ≤2 weeks, or developed with it
    2. headache is localized around the ipsilateral brow and eye

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

Comments:
Some reported cases of 13.7 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. The syndrome has been caused by granulomatous material in the cavernous sinus, superior orbital fissure or orbit in some biopsied cases.

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 13.7 Tolosa-Hunt syndrome resolve when it is treated adequately with corticosteroids.