TOLOSA HUNT SYNDROME

Question 1

TOLOSA HUNT SYNDROME

Answer 1

Granulomatous, but not cancerous, needs steroids:
* Painful ophthalmoplegia in the fifth decade with unknown aetiology located in SOF and anterior CSs.
* The cause of pain is granulomatous inflammation due to the infiltration of the walls of SOF or CS by lymphocytes and macrophages.
* If the pain reduces with steroid treatment within 72 hours it is diagnostic
* Inflammatory

This syndrome is caused by a nonspecific granulomatous inflammation (pseudotumour) in the anterior part of the cavernous sinus. Tolosa (1954) reported narrowing of the carotid artery in the sinus, a finding subsequently confirmed by Hunt et al. (1961).

Question 2

TOLOSA HUNT SYNDROME signs

Answer 2

The condition described by the international headache classification is characterised by:

One or more episodes of unilateral orbital pain, persisting for weeks if untreated.
Paresis of one or more of the third, fourth and/or sixth cranial nerves and/or demonstration of granuloma by magnetic resonance imaging or biopsy.
Paresis coincides with the onset of pain or follows it within 2 weeks.
Pain and paresis resolve within 72 hours when treated adequately with corticosteroids.
Other causes excluded by appropriate investigations.
Other features include the loss of sensation over the area of distribution of the first division of the trigeminal nerve, Horner’s syndrome and visual loss if the optic nerve is involved.

Question 3

TOLOSA HUNT SYNDROME mx

Answer 3

The syndrome responds to treatment with systemic steroids but can recur. Tumours at the orbital apex can mimic the Tolosa–Hunt syndrome and may show an initial response to steroids due to a reduction of the surrounding oedema. The appearance of the syndrome on magnetic resonance imaging (MRI), and in particular the disappearance of the lesion with steroid treatment, helps differentiate it from mass lesions at this site (Thomas et al. 1988).