Bing-Neel Syndrome at Diagnosis of Waldenstrom: An Extremely Rare Case Report

• Published in: Blood 2015-12, Vol.126 (23), p.5065-5065
• Main Authors: Stoumbos, Dionisios, Patsias, Ioannis, Asmanis, Evaggelos, Christaki, Evangelia Eleni, Vailiki, Katsiva, Tasidou, Anna, Kyrtsonis, Marie-Christine, Repousis, Panagiotis, Kotsopoulou, Maria
• Format: Article
• Language: English
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood.V126.23.5065.5065

BACKBGROUND: Bing-Neel syndrome (BNS) is an extremely rare neurologic complication of Waldenström macroglobulinemia (WM) that was first described in 1936 by Jens Bing and Axel Valdemar Neel. It is associated with central nervous system infiltration by neoplastic lymphoplasmacytoid and plasma cells with or without cerebrospinal fluid (CSF) hyperglobulinemia. Herein we report one case of a BNS, presented with central nervous system infiltration at diagnosis. We discuss its epidemiology, presentation, diagnosis and treatment. CASE PERESENTATION A 65-year-old man, was admitted to our hospital with no previous medical history presenting episodes of confusion, slurred speech, headache, fatigue, ataxia and memory problems. Neurological examination revealed left hemiparesis and abnormal pupillary response; normal cranial nerve III, IV, VI examination; Bell's palsy present characterized by left-sided facial droop, pathological Barre Test of the left upper and lower limb and pathological Babinski's sign left; the assessment of the sensory neuron responses did not reveal any findings; the muscular mass was hypotonic and hypotrophic. Initial investigations revealed a normal CBC; biochemical investigations were within normal ranges; thyroid investigation was normal; immunology investigations: IgG 3,02 g/L, IgA