Bing-Neel Syndrome Mimicking Behavioral Variant Frontotemporal Dementia. (7th June 2022)

• Record Type: Journal Article
• Authors: Donix, Markus; Haussmann, Robert; Brandt, Moritz D.; Linn, Jennifer
• Is Part Of: Neurology. Volume 98:Number 23(2022)
• Page Start: 980
• Page End: 981
• Publication Date: 2022-06-07
• Subjects: Neurology -- Periodicals; Neurology -- Periodicals; Neurologie -- Périodiques; 616.8
• DOI: 10.1212/WNL.0000000000200668
• Languages: English
• ISSNs: 0028-3878

Abstract

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