A Rare Neuromyelitis Optica Mimic: Primary CNS Histiocytic Sarcoma. (5th December 2022)

• Record Type: Journal Article
• Authors: Rogawski, David; Nirschl, Jeffrey; McDonald, Jamie; Nie, Esther; Schwartz, Nicholas; Vogel, Hannes; Scott, Brian; Gold, Carl; Kipp, Lucas
• Is Part Of: Neurology. Volume 99:Number 23(2022)Supplement 2
• Page Start: S32
• Page End: S33
• Publication Date: 2022-12-05
• Subjects: Neurology -- Periodicals; Neurology -- Periodicals; Neurologie -- Périodiques; 616.8
• DOI: 10.1212/01.wnl.0000903280.55740.ae
• Languages: English
• ISSNs: 0028-3878

Abstract

Abstract : Objective: NA. Background: NA. Design/Methods: NA. Results: Primary CNS histiocytic sarcoma is a rare hematolymphoid malignancy with features of mature histiocytes and carries a poor prognosis. We describe a unique case in which a 50-year-old woman presented with recurrent acute brainstem syndrome, area postrema syndrome, and myelitis with corresponding MRI lesions meeting diagnostic criteria for seronegative NMOSD. Despite initial improvement with steroids and plasma exchange, she experienced recurrent symptoms over ten months referable to new and persistently enhancing lesions. At autopsy, neuropathology revealed a diffusely infiltrative primary CNS histiocytic sarcoma. This case represents a rare clinicoradiologic mimic of NMOSD, underscoring the importance of evaluation for infiltrative diseases in cases of atypical seronegative NMOSD. Conclusions: NA.