Bing-Neel Syndrome revisited.
Since the 1936 seminal description of neurologic difficulties in patients with hyperglobulinemia, the "Bing-Neel Syndrome" has been applied to a range of neurologic symptoms. To clarify the central nervous system (CNS) manifestations of Waldenström macroglobulinemia (WM), we performed a literature search (years 1936 to 2008) of reports of "Bing-Neel Syndrome" or "WM affecting the CNS" (WM-CNS). We excluded cases of hyperviscosity, malignant transformation, vasculitis, and purely ophthalmologic manifestations. After analysis of symptoms, cerebrospinal fluid (CSF), imaging, and histopathology, we separate WM-CNS into (1) lymphoplasmacytoid cells infiltrating the CNS, and (2) a non-cellular form, in which other mechanisms, such as IgM deposition, might produce the neurologic symptoms.
Department of Radiology, Massachusetts General Hospital, Boston MA 02114, USA.
Florian
F
Fintelmann
Reza
R
Forghani
Pamela W
PW
Schaefer
Ephraim P
EP
Hochberg
Fred H
FH
Hochberg
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002493:Central Nervous System Diseases / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D013577:Syndrome; D008258:Waldenstrom Macroglobulinemia / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000473:pathology