Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rosai-Dorfman disease (RDD) predominantly affects cervical lymph nodes and presents with fever and pancytopenia. Central nervous system involvement though uncommon is often reported. Hydrocephalus and paraparesis as a consequence of RDD is an extremely rare entity. We present a 58-year-old male, diagnosed and treated for RDD with cervical lymphadenopathy, who now presented with spastic paraparesis and on evaluation was found to have communicating hydrocephalus that resolved after ventriculoperitoneal shunt surgery.
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PMID:Spastic Quadriparesis and Communicating Hydrocephalus as Late Sequel of Rosai-Dorfman Disease: A Case Report and Review of Literature. 2968 50

A 46-year-old male experienced progressive neurocognitive decline, weight loss, intermittent headaches, and weakness over 6 months. Magnetic resonance imaging of the brain revealed hydrocephalus and the spinal cord imaging showed diffuse leptomeningeal enhancement with prominent nerve root involvement. Intradural biopsy of lumbar arachnoid tissue found mixed inflammatory infiltrate consisting predominantly of histiocytes, S100 and CD68 positivity, and lymphocytophagocytosis (emperipolesis) consistent with extranodal Rosai-Dorfman disease. Rosai-Dorfman disease, a non-Langerhans cell histocytic disorder, can mimic the appearance of neurosarcoidosis and leptomeningeal carcinomatosis and should remain on the differential of a patient presenting with diffuse leptomeningeal enhancement, a common occurrence on a neurohospitalist service.
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PMID:Rosai-Dorfman Disease: A Less Common Cause of Leptomeningeal and Nerve Root Enhancement. 3298 53