Paulo Ribeiro Nóbrega1, Pedro Gustavo Barros Rodrigues2, Isabelle de Sousa Pereira2, Carolina de Figueiredo Santos3, Gunter Gerson4, José Arnaldo Motta de Arruda5, José Wagner Leonel Tavares Júnior2, Pablo Picasso de Araújo Coimbra6, Pedro Braga-Neto2,7. 1. 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. 2. Division of Neurology, Department of Clinical Medicine, Universidade Federal do Ceará, Rua Capitão Francisco Pedro, 1290, 60430-370, Fortaleza, Brazil. 3. Hospital Infantil Albert Sabin, Fortaleza, Brazil. 4. Department of Pathology, Universidade Federal do Ceará, Fortaleza, Brazil. 5. Division of Neurosurgery, Department of Surgery, Universidade Federal do Ceará, Fortaleza, Brazil. 6. Uniclinic Diagnóstico por Imagem - UDI Fortaleza, Fortaleza, Brazil. 7. Center of Health Sciences, Universidade Estadual do Ceará, Fortaleza, Brazil.
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.
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.
Authors: Jean-François Emile; Oussama Abla; Sylvie Fraitag; Annacarin Horne; Julien Haroche; Jean Donadieu; Luis Requena-Caballero; Michael B Jordan; Omar Abdel-Wahab; Carl E Allen; Frédéric Charlotte; Eli L Diamond; R Maarten Egeler; Alain Fischer; Juana Gil Herrera; Jan-Inge Henter; Filip Janku; Miriam Merad; Jennifer Picarsic; Carlos Rodriguez-Galindo; Barret J Rollins; Abdellatif Tazi; Robert Vassallo; Lawrence M Weiss Journal: Blood Date: 2016-03-10 Impact factor: 22.113
Authors: Oussama Abla; Eric Jacobsen; Jennifer Picarsic; Zdenka Krenova; Ronald Jaffe; Jean-Francois Emile; Benjamin H Durham; Jorge Braier; Frédéric Charlotte; Jean Donadieu; Fleur Cohen-Aubart; Carlos Rodriguez-Galindo; Carl Allen; James A Whitlock; Sheila Weitzman; Kenneth L McClain; Julien Haroche; Eli L Diamond Journal: Blood Date: 2018-05-02 Impact factor: 22.113