• Jessica Mandrioli, Giorgio Frank, Patrizia Sola, Maria Ernesta Leone, Giovanni Guaraldi, Pietro Guaraldi, Guido Collina, Federico Roncaroli, and Pietro Cortelli.
• Department of Neuroscience, University of Modena and Reggio Emilia, Italy.
• Headache. 2004 Sep 1;44(8):806-11.

BackgroundThe Tolosa-Hunt syndrome is characterized by ophthalmoplegia with unilateral severe retro-orbital pain associated to a granulomatous inflammatory process occupying the cavernous sinus or the superior orbital fissure. The etiology is unknown and diagnosis is based upon a clinical response to steroid treatment and exclusion of neoplasm, trauma, aneurysms, infectious, and inflammatory diseases.Case DescriptionA 43-year-old man was admitted because of a 1-week history of acute onset left-sided retro-orbital pain, followed by left sixth cranial nerve palsy. Magnetic resonance imaging was normal and Tolosa-Hunt syndrome was suspected. Steroid treatment controlled pain with recovery of ophthalmoplegia. Four months later, when a good response to treatment was still present, brain magnetic resonance imaging revealed a lesion enlarging the left cavernous sinus, isointense with the gray matter on T1-weighted sequences, hypointense on T2-weighted images, and with homogeneous enhancement after gadolinium injection. Two months later, ocular pain and sixth cranial nerve palsy recurred and new brain magnetic resonance imaging showed an extension of the tissue occupying the left cavernous sinus, over the sella, to the right cavernous sinus, making possible an endoscopic transphenoidal biopsy.ResultsHistopathological study revealed a granulomatous aspecific inflammation containing actinomycetes colonies. The patient was treated with intravenous penicillin G followed by amoxicillin per os, with improvement of pain and ophthalmoplegia. A control magnetic resonance imaging 1 month after therapy showed a consistent reduction of the enlarged cavernous sinus, and 3 months later neurological examination and brain magnetic resonance imaging were completely normal.ConclusionsThe present case suggests that the International Classification of Headache Disorders (2nd edition) definition of Tolosa-Hunt syndrome does not reflect the complexity of the syndrome and that some cases of secondary painful ophthalmoplegias can fit the criteria for the primary form. Since the biopsy can only rarely be performed, we agree with other authors that clinical and radiological follow-up should be performed for at least 2 years. Moreover, we propose that in patients with painful ophthalmoplegia having transient response to steroid therapy, a trial with antibiotic therapy should be taken into account.

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