[Long-term risk of relapses in Tolosa-Hunt syndrome]

The reviewed diagnostic criteria of Tolosa- Hunt síndrome (THS) by the International Classification of Headache Disorders (ICHD-II, 2004) includes demonstration of inflammatory changes by magnetic resonance imaging (MRI) in the cavernous sinus region as an alternative to biopsy. Careful follow-up is required to exclude other possible causes of painful ophthalmoplegia. It remains unclear for how long such close observation should be extended to assure the diagnosis nor whether THS represents a self-limited condition in the long-term.

An observational study along 13 years of a patient fulfilling current ICHD-II criteria for THS to clarify the natural history of the disorder.

We witnessed three episodes, the presenting one causing severe left orbital pain, ipsilateral abducens palsy , and hypoesthesia in the territory of the ophthalmic branch of the trigeminal nerve. Gadolinium MRI showed enhancement of tissue infiltrating the lateral wall of the carotid sinus. A relapse 21 months later caused involvement of left oculomotor and abducens nerves in addition to orbital pain which responded rapidly to steroids. Ensuing remission maintained for up to 11 years concluded in a new relapse again characterized by severe ipsilateral orbital pain and frontal numbness, but no ophthalmoplegia. A gadolinium-MRI did not show enhancement on this occasion despite prompt response to steroids.

The risk of relapses in THS and its response to steroids may be maintained for as long as 13 years.