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

• Autor(a) principal: Nóbrega, Paulo Ribeiro
• Data de Publicação: 2021
• Outros Autores: Rodrigues, Pedro Gustavo Barros, Pereira, Isabelle de Sousa, Santos, Carolina de Figueiredo, Gerson, Gunter, Arruda, José Arnaldo Motta de, Tavares Júnior, José Wagner Leonel, Coimbra, Pablo Picasso de Araújo, Braga-Neto, Pedro
• Tipo de documento: Artigo
• Idioma: eng
• Título da fonte: Repositório Institucional da Universidade Federal do Ceará (UFC)
• Texto Completo: http://www.repositorio.ufc.br/handle/riufc/63149
• topic: Histiocytosis, Sinus; Histiocitose Sinusal; Histiocytosis; Histiocitose; Tolosa-Hunt Syndrome; Síndrome de Tolosa-Hunt; Cavernous Sinus; Seio Cavernoso

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.