Bing-Neel syndrome: a case report and systematic review of clinical manifestations, diagnosis, and treatment options.
BACKGROUND
Bing-Neel syndrome is an extremely rare neurologic complication of Waldenström macroglobulinemia (WM) that was first described in 1936. It is associated with central nervous system infiltration by neoplastic lymphoplasmacytoid and plasma cells with or without cerebrospinal fluid (CSF) hyperglobulinemia.


METHODS
We report a case of a 69-year-old white man with a 10-year history of WM. He was diagnosed with Bing-Neel syndrome based on magnetic resonance imaging and pathology studies of CSF. In addition, a comprehensive review of the reported cases of Bing-Neel syndrome in the up-to-date English-language literature was performed.


RESULTS
Our patient underwent successful treatment with cranial radiation and intrathecal chemotherapy. He has been in clinical and pathologic remission for 3 years following the completion of his treatment. Based on our literature review, we also summarize and discuss clinical manifestations, diagnosis, and treatment options for Bing-Neel syndrome.


CONCLUSIONS
Bing-Neel syndrome is a rare and potentially treatable complication of WM. Patients with a history of WM presenting with neurologic symptoms should be evaluated for possible Bing-Neel syndrome. Cranial radiation therapy alone or in combination with intrathecal chemotherapy is more likely to achieve sustainable remission than intrathecal chemotherapy alone.
Grewal
Jaspreet S
JS
Department of Internal Medicine, St. Joseph Mercy Hospital, Reichert Health Center, 5333 McAuley Dr, Ste 3009, Ann Arbor, MI 48106-0995, USA. jaspreet_grewal@hotmail.com
Brar
Preetkanwal K
PK
Sahijdak
Walter M
WM
Tworek
Joseph A
JA
Chottiner
Elaine G
EG
D000368:Aged; D006801:Humans; D008297:Male; D001921:Brain / Q000473:pathology; D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D016371:Cranial Irradiation; D010950:Plasma Cells / Q000473:pathology; D013577:Syndrome; D008258:Waldenstrom Macroglobulinemia / Q000134:cerebrospinal fluid / Q000150:complications