Unusual clinical course in pediatric Tolosa-Hunt syndrome.

We report on a 7-year-old immunocompetent boy initially presenting with right-sided frontal cephalalgia, painful ophthalmoplegia, and ptosis for 1 month. Initial cerebrospinal fluid analysis produced normal results. Magnetic resonance imaging revealed an inflammatory pseudotumor of the right cavernous sinus after intravenous gadolinium administration, indicating a rare idiopathic inflammatory disorder of the cavernous sinus, i.e., Tolosa-Hunt syndrome. Ptosis and cephalalgia resolved after steroid treatment, although right-sided ophthalmoplegia remained. Breakthrough headache, associated with signs of meningeal irritation, developed 6 weeks later. Follow-up contrast-enhanced computed tomography revealed no enhancing cavernous soft tissue mass. A further lumbar puncture disclosed central nervous system infection with Staphylococcus saprophyticus. After 6 weeks of vancomycin, the headache resolved completely, and neuroimaging produced normal results. A diagnosis of Tolosa-Hunt syndrome should be rendered cautiously, because the etiology may involve a rare but not "idiopathic" infection. Moreover, if clinical signs are not fully responsive to steroid treatment, the underlying problems should receive careful investigation.

Tolosa-Hunt syndrome due to actinomycosis of the cavernous sinus: the infectious hypothesis revisited.

BACKGROUND: The 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 DESCRIPTION: A 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. RESULTS: Histopathological 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. CONCLUSIONS: The 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.