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 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.