Tolosa-Hunt Syndrome: Prospective Clinical and Neuroimaging Studies

Objective: To demonstrate the diagnostic abilities of various neuroimaging methods in Tolosa-Hunt syndrome. Materials and methods: The present report describes a 35-year-old man with a history of intermittent pain in the left retrobulbar area lasting approximately six months. The disease debuted with a general infectious syndrome, left-sided exophthalmos with transient visual disturbances and local inflammation of the conjunctiva. The condition was considered initially as conjunctivitis, and later - as Tolosa-Hunt syndrome, which was successfully treated with eye drops, nonsteroidal anti-inflammatory drugs (NSAIDs), antibiotics and corticosteroids. For the final diagnosis prospective clinical and neuroimaging investigations (2D/3D/4D multimodal neurosonology, CT and MRI of the head) were performed. Results: The ophthalmic status at the beginning of the disease showed left-sided retrobulbar pain with mild exophthalmos, edema of the left eyelid, conjunctival injection and restricted horizontal movement of the left eye. Head CT showed a pseudo-tumor formation in the left medial retrobulbar space with slight swelling of the left optic nerve. Four months later the local neuro-ophthalmic status established mild left exophthalmos, ptosis of the left eyelid, conjunctival hyporeflexia of the left eye and hyposmia associated with CT data for left ethmoid sinusitis. There were found periods of accelerated erythrocyte sedimentation rate (ESR), normalized after treatment with corticosteroids. Thyroid hormones and tumor markers (carcinoembryonic antigen and prostate specific antigen) were within reference ranges. The multimodal ultrasound neuroimaging showed left optic disc drusen, slightly increased diameters of the left optic nerve/sheath complex and thickened intima-media of the left internal carotid artery. The control MRT/MRA studies performed 6 months after the onset of the disease, established normal brain parenchyma, intracranial vascular system, orbits and retrobulbar spaces. The diagnosis of Tolosa-Hunt syndrome was based on the criteria of the Intrenational Hedache Society. Discussion: Multimodal ultrasound methods help to diagnose and follow patients with Tolosa-Hunt syndrome.