Remittent painful ophthalmoplegia: the Tolosa-Hunt syndrome? A report of seven cases and review of the literature.

Seven patients with remittent painful ophthalmoplegia for which no specific local cause was found were seen during a period of five years. One had coincidental rheumatoid arthritis, and another had actinomycosis of the ipsilateral middle ear and contralateral parotid gland. The other five had no evidence of generalised autoimmune disease nor any other systemic disorder, two having separate episodes affecting each side. A history of relapsing and remitting painful ophthalmoplegia is suggestive of the Tolosa-Hunt syndrome, but it is rarely possible to confirm that the lesion in the cavernous sinus is the result of non-specific granulomatous infiltration so that the diagnosis remains one of exclusion. Carotid arteriography may show narrowing of the intracavernous part of the internal carotid artery. Orbital venography may also be helpful, particularly when the carotid arteriogram is normal. We believe that the Tolosa-Hunt syndrome is more common in England than is generally realised, but that its clinical features do not necessarily indicate a single pathological entity. Its recognition is important since the response to steroids, although not specific, is rapid in most patients, and the prognosis for complete recovery is relatively good.