Steroid responsive cavernous sinus syndrome due to Rosai-Dorfman disease: beyond Tolosa-Hunt syndrome – a case report

• Main Authors: Paulo Ribeiro Nóbrega, Pedro Gustavo Barros Rodrigues, Isabelle de Sousa Pereira, Carolina de Figueiredo Santos, Gunter Gerson, José Arnaldo Motta de Arruda, José Wagner Leonel Tavares Júnior, Pablo Picasso de Araújo Coimbra, Pedro Braga-Neto
• Format: Article
• Language: English
• Published: BMC 2021-07-01
• Series: BMC Neurology
• Subjects: Rosai-Dorfman Disease; Histiocytosis; Tolosa-Hunt Syndrome; Cavernous Sinus; Neuroimmunology; Case report
• Online Access: https://doi.org/10.1186/s12883-021-02255-z

Abstract Background The term “Tolosa-Hunt syndrome” (THS) has been used to refer to painful ophthalmoplegia associated with nonspecific inflammation of the cavernous sinus and many processes can result in a similar clinical picture, including infectious, inflammatory and neoplastic diseases. Rosai-Dorfman disease (RDD) is a lymphoproliferative disorder that rarely affects the central nervous system. We report a case of isolated CNS Rosai-Dorfman disease involving the cavernous sinus and presenting as “Tolosa-Hunt syndrome”. Case presentation Our patient presented with horizontal diplopia due to impairment of cranial nerves III, IV and VI and a stabbing/throbbing headache predominantly in the left temporal and periorbitary regions. There was a nonspecific enlargement of the left cavernous sinus on MRI and the patient had a dramatic response to steroids. Biopsy of a frontal meningeal lesion was compatible with RDD. Conclusions We highlight the importance of including Rosai-Dorfman disease as a differential diagnosis in cavernous sinus syndrome and demonstrate a satisfactory long-term response to steroid treatment in this disease.