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

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

doi:10.2298/VSP150703180P

Ana Podgorac Faculty of Medicine, University of Belgrade, Belgrade, Serbia; Institute of Mental Health, Belgrade, Serbia
Jasna Zidverc-Trajković Faculty of Medicine, University of Belgrade, Belgrade, Serbia; Neurology Clinic, Clinical Center of Serbia, Belgrade, Serbia
Zagorka Jovanović Faculty of Medicine, University of Belgrade, Belgrade, Serbia; Neurology Clinic, Clinical Center of Serbia, Belgrade, Serbia
Aleksandar Ristić Faculty of Medicine, University of Belgrade, Belgrade, Serbia; Neurology Clinic, Clinical Center of Serbia, Belgrade, Serbia
Aleksandra Radojičić Faculty of Medicine, University of Belgrade, Belgrade, Serbia; Neurology Clinic, Clinical Center of Serbia, Belgrade, Serbia
Aleksandra Pavlović Faculty of Medicine, University of Belgrade, Belgrade, Serbia; Neurology Clinic, Clinical Center of Serbia, Belgrade, Serbia
Milija Mijajlović Faculty of Medicine, University of Belgrade, Belgrade, Serbia; Neurology Clinic, Clinical Center of Serbia, Belgrade, Serbia
Tamara Švabić-Medjedović Neurology Clinic, Clinical Center of Serbia, Belgrade, Serbia
Nadežda Čovičković-Šternić Faculty of Medicine, University of Belgrade, Belgrade, Serbia; Neurology Clinic, Clinical Center of Serbia, Belgrade, Serbia

DOI: https://doi.org/10.2298/VSP150703180P

Keywords: tolosa-hunt syndrome, brain, diagnosis, differential, magnetic resonance imaging,

Abstract

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

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