Tolosa-Hunt syndrome: is it really necessary to show granuloma? - The report of eight cases

Introduction: Tolosa-Hunt syndrome (THS) is a rare entity, characterized by unilateral orbital pain associated with paresis of one or more of the oculomotor cranial nerves and caused by a granulomatous inflammation in the cavernous sinus, superior orbital fissure or orbit. The low prevalence of THS with broad spectrum of other disorders that could cause painful ophtalmoplegia resulted in stricter diagnostic criteria of THS in the latest edition of the ICHD. Current criteria require demonstration of granuloma by magnetic resonance imaging (MRI) or biopsy. The diagnosis could be difficult and the initiation of treatment delayed due to high variablity of clinical presentation of TSH. Reducing the number of patients that, based on clinical presentation could be THS, but not fullfil all diagnostic criteria further complicates establishing of correct diagnosis. Methods: Hereby we present eight patients diagnosed and treated as THS. Inspite the exclusion of other causes of painful ophtalmoplegia, granuloma could not been demonstrated in half of patients. Results: Clinical presentation of THS in patients with and without shown granuloma, did not significantly differ concerning headache characteristics (localization, intensity, quality, duration preceding cranial nerve palsy, response to steroids), affected cranial nerve, disease course and response to treatment, as well as types of diagnostic procedures that were performed in ruling out other diseases from extensive differential diagnosis of painful ophthalmoplegia. Conclusion of this observation is that there is no significant difference between the patients with and without demonstrated granuloma.