13.8 Tolosa-Hunt syndrome

Description:

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.

Diagnostic criteria:
    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 IIIrd, IVth 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 IIIrd, IVth and/or VIth 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 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.