title: Novel diagnostic approaches in Bing-Neel syndrome.
abstract: The central nervous system (CNS) manifestations of Waldenström's macroglobulinemia (WM) are known as the Bing-Neel syndrome (BNS). Patients with BNS can be classified into Group A and Group B based on the presence of lymphoplasmacytoid (LMP) cells within the brain parenchyma, leptomeninges, dura, and/or cerebrospinal fluid (CSF). To identify characteristic imaging findings for both Group A and Group B patients, we reviewed all 36 cases (26 referenced, 10 unreported) of proven WM with CNS symptoms, CSF analysis and/or biopsy, and magnetic resonance imaging (MRI) of the brain and/or spinal cord. Enhancement on MRI suggests invasion of the central neuraxis by LMP cells, and can help distinguish between Group A and Group B patients. In addition to differentiating true WM lesions in the CNS from ischemia, hyperviscosity events, and demyelinating lesions, evaluation of brain and spinal cord with gadolinium-enhanced MRI has the potential to guide management.
authors.affiliation: Massachusetts General Hospital Cancer Center, Boston, USA.
authors.forename: K Ina
authors.initials: KI
authors.lastname: Ly
authors.forename: Florian
authors.initials: F
authors.lastname: Fintelmann
authors.forename: Reza
authors.initials: R
authors.lastname: Forghani
authors.forename: Pamela W
authors.initials: PW
authors.lastname: Schaefer
authors.forename: Ephraim P
authors.initials: EP
authors.lastname: Hochberg
authors.forename: Fred H
authors.initials: FH
authors.lastname: Hochberg
mesh_terms: D000368:Aged; D001706:Biopsy; D001921:Brain / Q000473:pathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D013116:Spinal Cord / Q000473:pathology; D008258:Waldenstrom Macroglobulinemia / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000473:pathology