English

Abstract

Tolosa-Hunt syndrome (THS) is a painful ophthalmoplegia caused by nonspecific inflammation of the cavernous sinus or superior orbital fissure. The syndrome consists of periorbital or hemicranial pain, combined with ipsilateral ocular motor nerve palsies, oculosympathetic paralysis, and sensory loss in the distribution of the ophthalmic and occasionally the maxillary division of the trigeminal nerve. Although they have relapsing and remitting course, they respond promptly to systemic corticosteroid therapy. The diagnostic eponym Tolosa-Hunt syndrome has been applied to these patients and it is this entity which forms the basis of this review. CASE REPORT: A 42 year old man presented with painful diminution of vision since one month and diplopia in the left eye since 7 days. Tingling sensation was noted over left half of upper face. On clinical examination –V/A was 6/12 (B/E) improving to 6/6 with pinhole, IOP and fundus was normal both eyes, extra ocular movement was completely restricted in left eye with painful movements. Left III, IV and VI Cranial nerve palsies were noted with decreased sensation over the area supplied by V Cranial Nerve. The patient was sent for an MR examination. MR imaging was performed on a 1.5 Tesla MR scanner (GE). 10ml Gadolinium was injected in the left antecubital vein to obtain post contrast T1WI.Study revealed moderately enhancing soft tissue in the region of left cavernous sinus and orbital apex. It was isointense to muscle on T1WI and hypo intense to fat on T2WI. MANAGEMENT: On the basis of history and imaging findings, a diagnosis of THS was made and the patient was started on corticosteroid therapy: Injection Methyl-Prednisolone (1gm.I.V for 3 days).Dramatic improvement in diplopia and pain was noted within 48 hours of starting IV steroid therapy. Oral Prednisolone 40mg daily was started after 3 days and continued for 6 weeks. At the end of therapy the patient's ophthalmoplegia also recovered. DISCUSSION: Tolosa–Hunt syndrome (THS) is a rare disorder characterized by severe and unilateral headaches with extra ocular palsies, usually involving the third, fourth, fifth, and sixth cranial nerves, and pain around the sides and back of the eye, along with weakness and paralysis (ophthalmoplegia) of certain eye muscles1. The syndrome of painful ophthalmoplegia consists of periorbital or hemicranial pain, combined with ipsilateral ocular motor nerve palsies, oculosympathetic paralysis, and sensory loss in the distribution of the ophthalmic and occasionally the maxillary division of the