• 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, de Araújo CoimbraPablo PicassoPPUniclinic Diagnóstico por Imagem - UDI Fortaleza, Fortaleza, Brazil., and Pedro Braga-Neto.
• Division of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Rua Capitão Francisco Pedro, 1290, 60430-370, Fortaleza, Brazil. paulo_r_med@yahoo.com.br.
• Bmc Neurol. 2021 Jul 5; 21 (1): 264.

BackgroundThe 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 PresentationOur 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.ConclusionsWe 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.

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