Objective: Central nervous system involvement by malignant cells is a rare complication of Waldenstrom macroglobulinemia (WM), known as Big-Neel syndrome (BNS). BNS is even rarer than WM and involves central nervous system (CNS) infiltration by neoplastic lymphoplasmacytoid and plasma cells with or without cerebrospinal fluid (CSF) hyperglobulinemia. Due to the rarity of this diagnosis, few descriptions of neuropsychological performance exist in the literature. This case study highlights the neurobehavioral characteristics WM with confirmed BNS. We aim to expand understanding of cognitive effects of this rare diagnosis. Method: A 71 year old Caucasian male physician underwent brain MRI, lumbar puncture (LP), and serial neuropsychological testing. Initial symptoms included disorientation, anomia, motor difficulties, and short-term memory disturbance. Due to initial neuropsychological test results in the context of WM, BNS was suspected and confirmed by LP. Results: Initial neuropsychological testing showed severe problems with executive functioning and mild deficits in dexterity, as well...

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