Painful ophthalmoplegia due to inflammation of the cavernous sinus in a patient with familial Mediterranean fever

Abstract

We report a 32-year-old patient with familial Mediterranean fever (FMV) who presented with painful ophthalmoplegia due to unilateral cavernous sinus inflammation. At age 32, she was admitted to our hospital due to the development of headache, retroorbital pain in the right eye and diplopia. Brain magnetic resonance imaging showed soft tissue thickening with homogenous enhancement at the right cavernous sinus. Hence, a diagnosis of Tolosa–Hunt syndrome was made, and intravenous dexamethasone administration was begun. To date, several inflammatory disorders have been reported to develop in FMF patients, but clinical manifestations in the central nervous system (CNS) other than headache have been rarely seen in FMF patients. Our observations also indicate that Tolosa–Hunt syndrome may be an important CNS complication in FMF patients.

Keywords:

• Familial Mediterranean fever
• inflammation of cavernous sinus
• painful ophthalmoplegia
• Tolosa–Hunt syndrome

Acknowledgements

We thank Prof. Hiroaki Ida, Kurume University School of Medicine, Kurume, Japan, for DNA analysis of the TNFRSF1A gene. This study was supported by a Grant-in-Aid for Scientific Research in Japan (DK).

Patient’s consent

Written informed consent was obtained from the patient for submission of her facial photographs and MRI.

Conflict of interest

None.

Additional information

Funding

This study was supported by a Grant-in-Aid for Scientific Research, Ministry of Education, Culture, Sports, Science and Technology in Japan (26860477 to DK).