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- Title
Steroid responsive cavernous sinus syndrome due to Rosai-Dorfman disease: beyond Tolosa-Hunt syndrome - a case report.
- Authors
Nóbrega, Paulo Ribeiro; Rodrigues, Pedro Gustavo Barros; de Sousa Pereira, Isabelle; de Figueiredo Santos, Carolina; Gerson, Gunter; de Arruda, José Arnaldo Motta; Júnior, José Wagner Leonel Tavares; de Araújo Coimbra, Pablo Picasso; Braga-Neto, Pedro
- Abstract
<bold>Background: </bold>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".<bold>Case Presentation: </bold>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.<bold>Conclusions: </bold>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.
- Subjects
CAVERNOUS sinus; NON-langerhans-cell histiocytosis; CASTLEMAN'S disease; CENTRAL nervous system; SYNDROMES; LYMPHOPROLIFERATIVE disorders
- Publication
BMC Neurology, 2021, Vol 21, Issue 1, p1
- ISSN
1471-2377
- Publication type
journal article
- DOI
10.1186/s12883-021-02255-z