Tolosa Hunt Syndrome: An Atypical Presentation of a Rare Condition

Abstract

Abstract Purpose: Tolosa-Hunt Syndrome (THS) is a rare disorder with signs and symptoms that mimic various other diseases. Diagnosis of THS, especially in atypical cases, can be challenging due to the need to exclude other diseases. Observations: A 59-year-old-male presented with acute vision loss of his left eye preceded by acute worsening of chronic headaches. Additionally, he had near complete ophthalmoplegia of this eye. He underwent extensive workup including numerous hematologic studies, cerebrospinal fluid studies, and neuro-radiological imaging studies. All tests had normal results except for an elevated ESR of 92 mm/hour and an abnormal MRI of the brain and orbits. The MRI revealed the left orbit as abnormal with proptosis and intraconal inflammatory change. It also showed abnormal enhancement of the left orbital apex and adjacent lateral aspect of the left sphenoid sinus. He was diagnosed with THS and rapidly responded to corticosteroid treatment. Conclusions and Importance: THS can be challenging to diagnose because it is a diagnosis of exclusion. An added difficulty with THS is the need to quickly recognize and diagnose it since early treatment with corticosteroids can produce a rapid treatment response and decreases the chance of severe residual symptoms. THS does not usually affect the optic nerve, but optic nerve damage can occur and involvement of the optic nerve further increases the urgency of diagnosing it and promptly initiating treatment.