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...

Full description

Saved in:
Bibliographic Details
Main Authors: Shoon L. Oo, Myint B. Thu, Su S. Win, Amro Ilaiwy
Format: Article
Language:English
Published: American College of Physicians 2024-12-01
Series:Annals of Internal Medicine: Clinical Cases
Online Access:https://www.acpjournals.org/doi/10.7326/aimcc.2024.0356
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1850146744169398272
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
work_keys_str_mv AT shoonloo novelpresentationtoararediseaserefractoryseizurescausedbyrosaidorfmandisease
AT myintbthu novelpresentationtoararediseaserefractoryseizurescausedbyrosaidorfmandisease
AT suswin novelpresentationtoararediseaserefractoryseizurescausedbyrosaidorfmandisease
AT amroilaiwy novelpresentationtoararediseaserefractoryseizurescausedbyrosaidorfmandisease