What is Tolosa-Hunt Syndrome?

Tolosa-Hunt syndrome is a very rare condition that can cause severe headaches, eye pain, and vision disturbances on one side of the face. The disorder appears to be related to acute inflammation of the sinus cavity that sits just behind and below the eyes. It is usually unclear what causes inflammation and resulting Tolosa-Hunt syndrome, which makes it difficult for physicians to accurately diagnose the disorder. Almost half of cases spontaneously get better on their own within a few days, and treatment with anti-inflammatory drugs can usually correct more persistent instances.

Very little is known about the causes of Tolosa-Hunt syndrome, partly because it is such an uncommon finding. It is relatively well understood what happens after sinus inflammation begins. Swollen and irritated tissue puts pressure on the eye socket. Inflammation typically spreads upward from the sinus and engulfs the superior orbital fissure, which serves as a passageway for important facial nerves. In many cases, symptoms develop very quickly over the course of just a few hours.

Constant pressure on the eye and nearby nerves can result in a range of symptoms. Dull aches in the eye socket and forehead are common, and some people experience shooting pains in their eyes. Ophthalmoplegia, or partial eye paralysis, can occur when nerves are compressed. Vision can become blurry or doubled, which may result in additional symptoms of nausea, headache, and vomiting. It is very important to visit an emergency room at the first sign of sudden eye pain and vision changes so prompt care can be provided.

A diagnosis of Tolosa-Hunt syndrome is typically made after several other, more common causes of symptoms are ruled out. A doctor collects blood samples, performs vision tests, and asks about medical history. A specialist may take x-rays and magnetic resonance imaging scans of the skull and brain to look for signs of physical abnormalities, such as congenital eye defects or tumors in the sinuses. In addition, a cerebrospinal fluid sample is often collected to check for infections.

Hospital treatment for Tolosa-Hunt syndrome typically includes large doses of an anti-inflammatory corticosteroid. Medication may be injected directly into the sinus or provided through an intravenous line. With regular treatment, swelling tends to ease within one to two days and symptoms begin to resolve. Doctors generally encourage patients to continue taking oral corticosteroids at home and return for a follow-up visit in about one week. Most cases are fully resolved after a round of treatment, but it is possible for a patient to have reoccurring eye problems.