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

BMC Neurology

Gunter Gerson¹  Isabelle de Sousa Pereira²  José Wagner Leonel Tavares Júnior²  Paulo Ribeiro Nóbrega²  Pedro Gustavo Barros Rodrigues²  Pedro Braga-Neto³  José Arnaldo Motta de Arruda⁴  Carolina de Figueiredo Santos⁵  Pablo Picasso de Araújo Coimbra⁶ 

[1] Department of Pathology, Universidade Federal do Ceará, Fortaleza, Brazil;Division of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Rua Capitão Francisco Pedro, 1290, 60430-370, Fortaleza, Brazil;Division of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Rua Capitão Francisco Pedro, 1290, 60430-370, Fortaleza, Brazil;Center of Health Sciences, Universidade Estadual do Ceará, Fortaleza, Brazil;Division of Neurosurgery, Department of Surgery, Universidade Federal do Ceará, Fortaleza, Brazil;Hospital Infantil Albert Sabin, Fortaleza, Brazil;Uniclinic Diagnóstico por Imagem - UDI Fortaleza, Fortaleza, Brazil;

关键词: Rosai-Dorfman Disease;    Histiocytosis;    Tolosa-Hunt Syndrome;    Cavernous Sinus;    Neuroimmunology;    Case report;   

DOI  :  10.1186/s12883-021-02255-z

来源: Springer

【 摘 要 】

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.

【 授权许可】

CC BY