Novel Presentation to a Rare Disease: Refractory Seizures Caused by Rosai–Dorfman Disease
Rosai–Dorfman disease (RDD) is a non-Langerhans lymphoproliferative disorder that is defined by sinus histiocytosis with massive lymphadenopathy. It can present with a wide spectrum of clinical presentations depending on the organ systems affected. Definitive diagnosis of RDD can be established only...
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| Format: | Article |
| Language: | English |
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American College of Physicians
2024-12-01
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| Series: | Annals of Internal Medicine: Clinical Cases |
| Online Access: | https://www.acpjournals.org/doi/10.7326/aimcc.2024.0356 |
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| author | Shoon L. Oo Myint B. Thu Su S. Win Amro Ilaiwy |
| author_facet | Shoon L. Oo Myint B. Thu Su S. Win Amro Ilaiwy |
| author_sort | Shoon L. Oo |
| collection | DOAJ |
| description | Rosai–Dorfman disease (RDD) is a non-Langerhans lymphoproliferative disorder that is defined by sinus histiocytosis with massive lymphadenopathy. It can present with a wide spectrum of clinical presentations depending on the organ systems affected. Definitive diagnosis of RDD can be established only by tissue biopsy. Surgical excision is the definitive treatment of localized RDD. We present a case of uncontrolled seizures caused by RDD. Despite surgical excision and multiple antiepileptic agents, the seizure disorder remained uncontrolled. Subsequently, the refractory seizures responded to systemic chemotherapy with rituximab, which has not yet been approved for the treatment of RDD. |
| format | Article |
| id | doaj-art-269ef4fa49ce4a79a9c1904acd09276b |
| institution | OA Journals |
| issn | 2767-7664 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | American College of Physicians |
| record_format | Article |
| series | Annals of Internal Medicine: Clinical Cases |
| spelling | doaj-art-269ef4fa49ce4a79a9c1904acd09276b2025-08-20T02:27:45ZengAmerican College of PhysiciansAnnals of Internal Medicine: Clinical Cases2767-76642024-12-0131210.7326/aimcc.2024.0356Novel Presentation to a Rare Disease: Refractory Seizures Caused by Rosai–Dorfman DiseaseShoon L. Oo0Myint B. Thu1Su S. Win2Amro Ilaiwy31Cape Fear Valley Health Internal Medicine Residency Program, Fayetteville, North Carolina2Harlem Hospital Center Internal Medicine Residency Program, New York, New York1Cape Fear Valley Health Internal Medicine Residency Program, Fayetteville, North Carolina1Cape Fear Valley Health Internal Medicine Residency Program, Fayetteville, North CarolinaRosai–Dorfman disease (RDD) is a non-Langerhans lymphoproliferative disorder that is defined by sinus histiocytosis with massive lymphadenopathy. It can present with a wide spectrum of clinical presentations depending on the organ systems affected. Definitive diagnosis of RDD can be established only by tissue biopsy. Surgical excision is the definitive treatment of localized RDD. We present a case of uncontrolled seizures caused by RDD. Despite surgical excision and multiple antiepileptic agents, the seizure disorder remained uncontrolled. Subsequently, the refractory seizures responded to systemic chemotherapy with rituximab, which has not yet been approved for the treatment of RDD.https://www.acpjournals.org/doi/10.7326/aimcc.2024.0356 |
| spellingShingle | Shoon L. Oo Myint B. Thu Su S. Win Amro Ilaiwy Novel Presentation to a Rare Disease: Refractory Seizures Caused by Rosai–Dorfman Disease Annals of Internal Medicine: Clinical Cases |
| title | Novel Presentation to a Rare Disease: Refractory Seizures Caused by Rosai–Dorfman Disease |
| title_full | Novel Presentation to a Rare Disease: Refractory Seizures Caused by Rosai–Dorfman Disease |
| title_fullStr | Novel Presentation to a Rare Disease: Refractory Seizures Caused by Rosai–Dorfman Disease |
| title_full_unstemmed | Novel Presentation to a Rare Disease: Refractory Seizures Caused by Rosai–Dorfman Disease |
| title_short | Novel Presentation to a Rare Disease: Refractory Seizures Caused by Rosai–Dorfman Disease |
| title_sort | novel presentation to a rare disease refractory seizures caused by rosai dorfman disease |
| url | https://www.acpjournals.org/doi/10.7326/aimcc.2024.0356 |
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