Tolosa-Hunt syndrome: is it really necessary to show granuloma? - The report of eight cases
2017
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.
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