Rosai Dorfman disease: case with extensive dural involvement and cerebrospinal fluid pleocytosis.
We report a young adult man who presented with chronic raised intracranial tension features and unusually progressive bilateral visual and hearing impairment of 18 months duration. MR imaging showed extensive dural involvement and contiguous orbital and spinal disease. Cerebrospinal fluid demonstrated persistent high lymphocytic pleocytosis. Dural biopsy obtained from posterior cervical approach with C1 arch excision and meningeal biopsy revealed features of classical of Rosai-Dorfman disease. Histiocytes were strongly positive for CD-68 and S-100 proteins. The illness relentlessly progressed with patient developing total deafness and near total blindness at last follow-up.
Department of Neurology, National Institute of Mental Health and Neurosciences, Bangalore, India. atchayaramnalini@yahoo.co.in
Atchayaram
A
Nalini
Saini
S
Jitender
Gudipati
G
Anantaram
Vani
V
Santosh
D000328:Adult; D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D001706:Biopsy; D018450:Disease Progression; D004388:Dura Mater / Q000473:pathology*; D006311:Hearing Disorders / Q000209:etiology; D006644:Histiocytes / Q000378:metabolism; D015618:Histiocytosis, Sinus / Q000134:cerebrospinal fluid* / Q000473:pathology*; D006801:Humans; D007958:Leukocyte Count; D007964:Leukocytosis / Q000134:cerebrospinal fluid*; D008279:Magnetic Resonance Imaging; D008297:Male; D008578:Meninges / Q000473:pathology; D009915:Orbit / Q000473:pathology; D009418:S100 Proteins / Q000378:metabolism; D013122:Spinal Diseases / Q000209:etiology / Q000473:pathology; D014786:Vision Disorders / Q000209:etiology; D000097382:CD68 Molecule
D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D009418:S100 Proteins; C067980:CD68 antigen, human; D000097382:CD68 Molecule