Table 1 ICHD-II classification part three

13.16 Tolosa-Hunt syndrome

Description:

Episodic orbital pain associated with paralysis of one or more of the third, fourth and/or sixth cranial nerves which usually resolves spontaneously but tends to replase and remit.

Diagnostic criteria:

A. One or more episodes of unilateral orbital pain persisting for weeks if untreated

B. Paresis of one or more of the third, fourth and/or sixth cranial nerves and/or demonstration of granulomas by MRI or biopsy

C. Paresis coincides with the onset of pain or follows it within 2 weeks

D. Pain and paresis resolve within 72 h when treated adequately with corticosteroids

E. Other causes have been excluded by appropriate investigations¹

Note:

1. Other causes of painful ophthalmoplegia include tumours, vasculitis, basal meningitis, sarcoid, diabetes mellitus a ophthalmoplegic 'migraine'.

Comments:

Some reported cases of Tolosa-Hunt syndrome had additional involvement of the trigeminal nerve (commonly the first; division) or optic, facial or acoustic 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 possible causes of painful ophthalmoplegia.