Bing-Neel Syndrome Mimicking Behavioral Variant Frontotemporal Dementia. (7th June 2022) - Donix, Markus
Abstract : An 80-year-old man presented with a 2-year history of cognitive deficits, apathy, and verbal impulsivity, suggestive of behavioral variant frontotemporal dementia. Medical history included idiopathic facial nerve palsy and Waldenstrom macroglobulinemia, a lymphoplasmacytic lymphoma. Brain MRI showed paraventricular, leptomeningeal, and cranial nerve enhancement (Figure) and enlarged ventricles. Immunocytologic analysis of CSF (58 MPt/L, protein 2.69 g/L, immunoglobulin M 173.25 mg/L) revealed neoplastic B cells. Brain biopsy and immunohistology confirmed Bing-Neel syndrome, a rare diffuse or tumorous 1 central nervous manifestation of Waldenstrom macroglobulinemia. Clinical features include sensory or motor deficits, behavioral and cognitive changes, as well as facial or oculomotor nerve palsy. 2