13.8 Tolosa-Hunt syndromeHartmut Gobel2018-02-06T13:08:25+00:00

Description:

Unilateral orbital or periorbital pain associated with paresis of one or more of the III^rd, IV^th and/or VIth cranial nerves caused by a granulomatous inflammation in the cavernous sinus, superior orbital fissure or orbit.

Diagnostic criteria:

1. 1. Unilateral orbital or periorbital headache fulfilling criterion C
   2. 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 III^rd, IV^th and/or VIth cranial nerves
   3. Evidence of causation demonstrated by both of the following:
      1. headache is ipsilateral to the granulomatous inflammation
      2. headache has preceded paresis of the III^rd, IV^th and/or VI^th nerves by ≤2 weeks, or developed with it
   4. Not better accounted for by another ICHD-3 diagnosis.

   Comments:

   Some reported cases of 13.8 Tolosa-Hunt syndrome had additional involvement of the V^th nerve (commonly the first division) or optic, VII^th or VIII^th 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.