Issue: Vojnosanit Pregl 2017; Vol. 74 (No. 3)

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

Authors:
Ana Podgorac, Jasna Zidverc-Trajković, Zagorka Jovanović, Aleksandar Ristić, Aleksandra Radojičić, Aleksandra Pavlović, Milija Mijajlović, Tamara Švabić-Medjedović, Nadežda Čovičković Šternić

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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 a broad spectrum of other disorders
that could cause painful ophtalmoplegia resulted in a stricter
diagnostic criteria of THS in the latest edition of the International
Classification of Headache Disorders. Current criteria
require demonstration of granuloma by magnetic resonance
imaging or biopsy. The diagnosis could be difficult and the
initiation of treatment delayed due to a high variablity of clinical
presentation of TSH. Reducing the number of patients
that, based on clinical presentation, could be classified as having
THS, but do not fullfil all diagnostic criteria further
complicates establishing of correct diagnosis. Case report.
Hereby we presented eight patients diagnosed with and treated
for THS. Inspite the exclusion of other causes of painful
ophtalmoplegia, granuloma could not be demonstrated in a
half of patients. 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), the affected cranial nerve, disease course and response
to the treatment, as well as types of diagnostic procedures
that were performed in ruling out other diseases from
the extensive differential diagnosis of painful ophthalmoplegia.
Conclusion. There is no significant difference between
the THS patients with and without demonstrated granuloma.